Topic: Will those with ACA presentation please step forward?
Melanie Reber
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(please see my questions at the end of this post)
Acrodermatitis Chronica Atrophicans
Background Acrodermatitis chronica atrophicans (ACA) is the third or late stage of European Lyme borreliosis (LB). This unusual, progressive, fibrosing skin process is due to the effect of continuing active infection with Borrelia afzelii. Buchwald first delineated it in 1883; Herxheimer and Hartmann described it in 1902 as a tissue paper-like cutaneous atrophy. It is evident on the extremities, particularly on the extensor surfaces, beginning with an inflammatory stage with bluish red discoloration and cutaneous swelling and concluding several months or years later with an atrophic phase. Sclerotic skin plaques may also develop. Physicians should use serologic and histologic examination to confirm this diagnosis.
Pathophysiology B afzelii is the predominant, but may not be the exclusive, etiologic agent of acrodermatitis chronica atrophicans. Another genospecies of the Borrelia burgdorferi sensu lato complex, Borrelia garinii, has also been detected.
Acrodermatitis chronica atrophicans is the only form of Lyme borreliosis in which no spontaneous remission occurs. Its pathophysiology is not yet fully understood. Acrodermatitis chronica atrophicans appears to be associated with long-term persistence of Borrelia organisms in the skin; several nonspecific reactions together with a specific immune response may contribute to its manifestations.
The persistence of the spirochetes despite a marked cutaneous T-cell infiltration and high serum antibody titers may be connected with resistance of the pathogen to the complement system; the ability to escape to immunologically protected sites (eg, endothelial cells, fibroblasts); and the ability to change antigens, which may lead to an inappropriate immune response. Lack of protective antibodies, with a narrow antibody spectrum and a weak cellular response with down-regulation of major histocompatibility system class II molecules on Langerhans cells, has been observed in patients with Lyme borreliosis.
A restricted pattern of cytokine expression in acrodermatitis chronica atrophicans, including the lack of interferon-gamma, may contribute to its chronicity. Cross-reactive antibody responses could take part in autoimmune damage, but whether autoimmune reactions play any role in the pathogenesis of the disease is unclear. The pathogenic mechanism of atrophic skin changes has also not been clarified. Perhaps periarticular regions are favorite sites because of reduced acral skin temperatures or reduced oxygen pressure.
History Because of its late onset, patients with acrodermatitis chronica atrophicans rarely remember a tick bite. Instead, they recall having been in the woods or grassy areas a few months or years previously, especially in a geographically endemic region. A history of EM is recalled by about 20% of patients. Acrodermatitis chronica atrophicans can develop directly from EM or after 6-36 months, often involving the same region of the body. Sometimes, the disease may be preceded by a latent phase (lasting up to several years) or by other manifestations of Lyme borreliosis; the latter can also develop simultaneously.
* The patient notices localized cutaneous swelling on the distal extremity or on only one of the digits and sometimes discovers that one foot is larger than the other when buying shoes. Acrodermatitis chronica atrophicans is most often unilateral, although bilateral acrodermatitis chronica atrophicans is also common.
* Progressive allodynia, the exaggerated reaction to pain, is a characteristic symptom and, thus, may be a clue to the diagnosis of acrodermatitis chronica atrophicans.
* Patients commonly complain of spontaneous acral pain and paresthesia or dysesthesia or cognitive dysfunction.
* Acrodermatitis chronica atrophicans starts with an inflammatory phase, characterized by few to several soft, erythematous, slowly enlarging cutaneous swellings or flat infiltrations of various sizes or with diffuse bluish red discoloration and edema of the skin.
o Acrodermatitis chronica atrophicans usually appears on the distal part of at least one extremity, predominantly on the extensor surfaces on the bony prominences.
o Common sites are the foot, the lower leg or the hand, the forearm, and the olecranon area; however, they uncommonly appear proximally on the upper arm and the shoulder or the thigh and the buttock.
o Sometimes, the erythema is slight and swelling may dominate, or the signs are very subtle and may be overlooked by the patient or the physician.
o Lymphadenopathy may be noticed.
* Only one part of an extremity may be affected for many months or years. With time, the skin lesions may extend on one extremity or appear on additional ones and also involve other parts of the body.
* Fibrotic nodules (often multiple, localized linearly in the vicinity of joints) are typical. They can precede acrodermatitis chronica atrophicans or develop simultaneously. The most common sites of these nodules are the elbows and the knees.
* Acrodermatitis chronica atrophicans does not heal spontaneously; gradual conversion into its atrophic phase may occur during many years of infection.
o The skin becomes thin, atrophic, wrinkled, dry, and translucent.
o The hair is lost; the number of sebaceous and sweat glands are decreased.
o Even minor trauma may produce large, slow-to-heal ulcerations of the affected skin.
* About 5-10% of patients with acrodermatitis chronica atrophicans develop sclerodermalike plaques. Anetodermalike skin lesions can be seen concomitant with acrodermatitis chronica atrophicans.
* Acrodermatitis chronica atrophicans is accompanied often by peripheral neuropathy, musculoskeletal pains, and joint damage underneath the cutaneous plaques. Involvement of the small joints of the hands and the feet by the fibrotic reaction is often seen.
Physical The clinical recognition of acrodermatitis chronica atrophicans may be difficult, even in typical cases. A detailed history, including epidemiologic data, is helpful. Physicians should confirm the clinical diagnosis by histopathologic examination and serologic test results.
* The early, inflammatory phase of acrodermatitis chronica atrophicans is marked by soft, painless, poorly demarcated, bluish reddish plaques tending to coalescence or by diffuse erythema and edema localized on the distal extremities that spread proximally.
o In the authors' experience, not only the distal extremities but also the proximal parts, the trunk, and the face may be involved in the early stage. Others have also observed skin-colored facial edema as an initial manifestation of acrodermatitis chronica atrophicans.
o Skin changes are often associated with regional or generalized lymphadenopathy.
* The later, atrophic phase of acrodermatitis chronica atrophicans is more characteristic clinically. The affected skin has a dark red or brownish red discoloration; focal hyperpigmentation; telangiectasias; and a thin, wrinkled, cigarette paper-like, translucent appearance.
o Because of the loss of subcutaneous fat, the skin vessels become prominent.
o Atrophy of the epidermis and lack of hairs, sebaceous glands, and often sweat glands make the skin poorly protected and vulnerable.
o Large ulcerations can be observed, and malignant lesions may also occur.
o The atrophic poikilodermic changes are often bilateral and most noticeable over the knees, the elbows, and the dorsal surfaces of the hands and the feet. They may also involve the trunk (particularly the chest) and the face.
* Sclerodermalike changes may appear in patients with acrodermatitis chronica atrophicans in both the inflammatory phase and the atrophic phase.
o These changes are usually limited to the legs and the feet, but they occasionally occur on the trunk.
o The lesions, similar to morphea and lichen sclerosus and atrophicus, may appear in regions where no acrodermatitis chronica atrophicans is present.
* Single or multiple fibrotic nodules or bands may be seen on the extensor surfaces of the elbows and the knees or adjacent to other joints. They are generally firm; bluish-red, yellowish, or skin-colored; and 0.5 to 2-3 cm in diameter.
* Acrodermatitis chronica atrophicans has been described in association with localized amyloidosis, eczema, psoriasis, lupus erythematosus, leprosy, and Hodgkin disease. These associations may be coincidental. One of the authors' patients with histologically and serologically confirmed acrodermatitis chronica atrophicans was a 68-year-old woman first seen with prominent livedo racemosa on the leg where typical acrodermatitis chronica atrophicans inflammatory phase patches developed. Others also observed the same phenomenon, so perhaps this may be more than a chance linkage.
* Detailed clinical and neurophysiologic examinations in patients with acrodermatitis chronica atrophicans-associated polyneuropathy often show a sensory polyneuropathy.
o Neuropathy symptoms, most often pain and/or paresthesia, are evident in one half of patients with acrodermatitis chronica atrophicans.
o One of the authors' patients had paresis of the brachial plexus.
o Marked abnormality of the vibratory threshold is a common finding.
o Patients with localized or asymmetric neuropathy seem to have changes more often found in the extremities with, rather than without, visible acrodermatitis chronica atrophicans lesions.
o Abnormalities in cerebrospinal fluid seldom have been found in patients with acrodermatitis chronica atrophicans.
* Acrodermatitis chronica atrophicans can produce deformities of the fingers and the toes if it is not treated promptly. Persistent reducible deformities of the fingers may be consistent with Jaccoud arthropathy.
Causes Lack of adequate or appropriate treatment of early Lyme borreliosis facilitates acrodermatitis chronica atrophicans development.
I have a few questions for those with ACA presentation:
Have you been diagnosed clinically or through serology? Has your LLMD offered treatment options, and if so, what are they? Have you been able to halt the progression with treatment? Do you know for certain where you were infected? Have you ever traveled to Europe or to other places outside of the states? Do you have photographic evidence of your condition? How long were you ill before this presented? What other diseases besides Lyme have you been diagnosed with? What are your main symptoms? Do you know of others with ACA?
Thank you so very much in advance for any information you can provide, Melanie
SForsgren
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I think it is rare to see this in the US b/c it is from the European strains and an advanced sign. I did see it once on a teenager that had it but doesn't seem to be common here
-------------------- Be well, Scott Posts: 4617 | From San Jose, CA | Registered: Jul 2005
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Melanie Reber
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Thank you Scott, How are you BTW?
I know that the current thought by many is that it is uncommon in the states, however, new research is proving that it is transferred by Borellia ssp. that is yet to be coded. I will try to locate that finding and post it.
I have since heard from many who are presenting with the exact images that I now have...and they have never traveled out of the country.
Acrodermatitis chronica atrophicans is a dermatological condition that takes a chronically progressive course and finally leads to a widespread atrophy of the skin. Involvement of the peripheral nervous system is frequently observed, predominantly a sensory polyneuropathy. Acrodermatitis chronica atrophicans follows a peculiar geographical distribution forming clusters of high prevalence in certain regions.
Acrodermatitis chronica atrophicans is a clinical manifestation of borreliosis, an infectious disease transmitted by ticks. Clinical manifestations of borreliosis are multiple. They most often affect the skin, nervous system, joints and heart. Cutaneous pseudolymphomas, erythema chronicum migrans of Afzelius, acrodermatitis chronica atrophicans of Pick-Herxheimer, meningo-radiculitis and various arthropathies are the most commonly encountered diseases.
Melanie Reber
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Cutaneous manifestations of Lyme borreliosis Elisabeth Aberer1 and Herta Klade1 Infection Volume 19, Number 4 / July, 1991
Summary
The dermatological symptoms of Lyme borreliosis present with a typical clinical pattern and characteristic time of appearance. In contrast to other manifestations of Lyme borreliosis they are easily recognizable in most of the cases. In the first stage, erythema migrans arises at the tick bite site. With this symptom the diagnosis of Lyme borreliosis can be established. During all manifestations of Lyme borreliosis the history of erythema migrans is an important parameter to verify the diagnosis. In the early stage of disease a lymphocytic proliferation can appear at the tick bite site, at the ear lobe, or at the mamilla.
Borrelia lymphocytoma can be diagnosed when antibodies againstBorrelia burgdorferi are positive. Years after infection, acrodermatitis chronica atrophicans arises at distal body sites causing livid swelling and gradually skin atrophy. Skin lesions can be accompanied by neuropathies, mostly of the lower legs, which in contrast to the skin lesions, do not respond well to antibiotic therapy. There is evidence that some cases of Shulman syndrome, morphea and lichen sclerosus et atrophicus might be related to a borrelia infection as indicated by cultivation ofB. burgdorferi from skin biopsies of morphea and response to antibiotic treatment in some cases.
The classical dermatological symptoms of Lyme borreliosis, erythema migrans, borrelia lymphocytoma and acrodermatitis chronica atrophicans respond to oral antibiotic treatment. In acrodermatitis chronica atrophicans parenteral antibiotic therapy is sometimes necessary.
Melanie Reber
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Lyme disease: the evolution of erythema chronicum migrans into acrodermatitis chronica atrophicans. Patmas MA. Community Medical Center, Toms River, New Jersey. Cutis. 1993 Sep;52(3):169-70.
Erythema chronicum migrans and acrodermatitis chronica atrophicans are both recognized to be lesions associated with Lyme disease, although they are thought to be distinct entities. In this paper, the clear evolution of erythema chronicum migrans into acrodermatitis chronica atrophicans is demonstrated.
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Melanie Reber
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Is Serological Follow-Up Useful for Patients with Cutaneous Lyme Borreliosis? [JOURNAL ARTICLE] Mullegger RR, Glatz M Curr Probl Dermatol 2009.:178-182.
Serologic follow-up examinations are frequently performed in patients with erythema migrans, borrelial lymphocytoma, and acrodermatitis chronica atrophicans (the 3 dermatoborrelioses) to evaluate treatment efficacy. There is, however, substantial proof in the literature that antibody titer development after therapy is unpredictable and variable, and moreover it is largely uncorrelated with the clinical course and mode of antibiotic treatment.
For example, persistent positive IgG and/ or IgM antibody titers do not indicate treatment failure. Thus, repeated serologic testing is of very limited value for assessing therapy efficacy, and therefore not recommended in the follow-up of dermatoborrelioses patients. Since cultivation of the etiologic agent, Borrelia burgdorferi sensu lato, and polymerase chain reaction are also inadequate for this purpose, the assessment of patients with cutaneous manifestations of Lyme borreliosis in the follow-up rests primarily on the clinical picture.
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Melanie Reber
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The expanding spectrum of cutaneous borreliosis. [Journal Article] Eisendle K, Zelger B G Ital Dermatol Venereol 2009 Apr; 144(2):157-71.
The known spectrum of skin manifestations in cutaneous Lyme disease is continuously expanding and can not be regarded as completed. Besides the classical manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially morphea, lichen sclerosus and cases of cutaneous B-cell lymphoma are causally related to infections with Borrelia.
Also granuloma annulare and interstitial granulomatous dermatitis might be partly caused by Borrelia burgdorferi or similar strains. There are also single reports of other skin manifestations to be associated with borrelial infections like cutaneous sarcoidosis, necrobiosis lipoidica and necrobiotic xanthogranuloma. In addition, as the modern chameleon of dermatology, cutaneous borreliosis, especially borrelial lymphocytoma, mimics other skin conditions, as has been shown for erythema anulare centrifugum or lymphocytic infiltration (Jessner Kanof) of the skin.
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Melanie Reber
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Dermatological aspects of Lyme borreliosis. [JOURNAL ARTICLE] Lipsker D Med Mal Infect 2007 Mar 26.
Lyme borreliosis is a tick-borne zoonosis due to bacterial infection by Borrelia (B.) burgdorferi sensu lato The disease presents differently in Europe or North America and may be called European borreliosis when acquired in Europe. Lyme borreliosis evolves in 3 stages. The main manifestations include cutaneous, neurological, and joint involvement.
Erythema migrans (EM) is the most specific and most frequent finding in patients with Lyme borreliosis. It is the hallmark of early-localized borreliosis. EM is a slowly expanding red macula that occurs in about 60-80% of patients contracting Lyme borreliosis. Central clearing of the red patch can occur. It appears at the site of the tick bite, 7 to 20 days after the bite.
Borrelial lymphocytoma (BL) occur rarely in patients with the early-disseminated stage of the disease. BL is a red or brown nodule or plaque located on the nipple, the earlobe, the scrotum, or the face. It should not be confused with cutaneous B-cell lymphoma.
Acrodermatitis chronica atrophicans (ACA) is the cutaneous manifestation of late borreliosis. It starts as a violaceous patch, usually located on the extensor surface of a limb. Periarticular nodules and cords can also be present. Without treatment, it will evolve over weeks or months to the typical atrophic stage with extensive dermo-epidermal atrophy and visibility of superficial veins. Only these 3 manifestations are clearly related to an infection with B. burgdorferi. The relationship between infection with B. burgdorferi and other dermatoses, especially morphea, lichen sclerosus, and interstitial granulomatous dermatitis is still debated.
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Melanie Reber
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Course of Borrelia burgdorferi DNA Shedding in Urine after Treatment. [Journal Article] Aberer E, Bergmann AR, Derler AM, Schmidt B Acta Derm Venereol 2007; 87(1):39-42.
Diagnosis of Lyme borreliosis by urine polymerase chain reaction (PCR) has been recognized as having better diagnostic sensitivity in patients with erythema migrans than serological methods. We made serial tests with 192 urine specimens from 70 patients with erythema migrans and 60 urine specimens from 21 patients with acrodermatitis chronica atrophicans to evaluate the course of positive urine PCR after antibiotic treatment.
Before treatment, urine samples from patients with erythema migrans showed a positive PCR in 27/34 samples (79%), and those from patients with acrodermatitis chronica atrophicans in 7/11 (63%). The specificity of bands was proven by hybridization with GEN-ETI-KTM-DEIA kit in 40/41 samples. Borrelia DNA in urine decreased gradually within the observation period of one year in both patients with erythema migrans and acrodermatitis chronica atrophicans, and persisted without clinical symptoms in 4/45 patients with erythema migrans (8%) after 12 months. Urine PCR can serve as a diagnostic method in early Lyme borreliosis and also in seropositive patients with unclear clinical symptoms.
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Melanie Reber
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Focus floating microscopy: "gold standard" for cutaneous borreliosis? [Journal Article] Eisendle K, Grabner T, Zelger B Am J Clin Pathol 2007 Feb; 127(2):213-22.
Borrelia burgdorferi is difficult to detect in routine biopsy material from patients with skin lesions of borreliosis. In this study, a new immunohistochemical method, focus floating microscopy (FFM), was developed to detect B burgdorferi in tissue sections and was compared with polymerase chain reaction (PCR).
By using standard histologic equipment, tissue sections stained with a polyclonal B burgdorferi antibody were simultaneously scanned through 2 planes: horizontally in serpentines and vertically by focusing through the thickness of the section. Borrelia were detected in 47 of 71 ticks, 34 of 66 tick bites, 30 of 32 erythema chronicum migrans cases, 41 of 43 borrelial lymphocytomas, and 50 of 51 acrodermatitis chronica atrophicans cases.
FFM proved to be more sensitive than PCR (96.0% vs 45.2%) and nearly equally specific (99.4% vs 100%). All 169 control cases, except 1 false-positive case of secondary syphilis, were negative with FFM. FFM is an easy, quick, and inexpensive method to reliably detect Borrelia in cutaneous tissue sections.
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Marnie
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I wonder if the skin could be treated with light like they do for psoriasis?
Although psoriasis can be linked to H.Pylori.
Or...if you chose abx, go here to see what was Rx'd, how much and for how long:
glm1111
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I have/had this Melanie.
As I get rid of the infections...parasites/worms/bb etc that I believe are inhabiting the small intestine my hands are coming back to normal.
It seems like the infections in the small intestine were spilling over and desseminated into the hands/head/organs etc. I hope this makes sense,
Gael
-------------------- PARASITES/WORMS ARE NOW RECOGNIZED AS THE NUMBER 1 CO-INFECTION IN LYME DISEASE BY ILADS* Posts: 6418 | From philadelphia pa | Registered: Jul 2008
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Marnie
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P.S. Are we using the wrong class of abx. to combat OspB?
Melanie Reber
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An American Case of Acrodermatite Chronica Atrophicans Ang�lique Gagn�-Henley; Dominique Hanna; Bruno Maynard, Centre hospitalier universitaire de Sherbrooke CHUS, Sherbrooke, QC
Introduction: Lyme disease is due to infection with a spirochete, Borrelia, and is transmitted by Ixodes ticks. Borrelia species in North America (Borrelia burgdorferi sensu stricto) are different from the European species (B. afzelii, B. garinii), which explains a different presentation of the disease. Acrodermatitis chronica atrophicans is well described following infection by the European species of borrelia (almost always B. afzelii) but is extremely rare in North America. It appears months to years after the infection, as a slowly progressive bluish-red patch that becomes atrophic.
Methods: We describe the case of a 36-year-old woman with a several year history of lesions on her knees and ankles. The lesions were asymptomatic, but more prominent since her pregnancy. She spent her summers, as a child, on the coasts of New England.
Results: Complete blood work was normal. The skin biopsy was compatible with acrodermatitis chronica atrophicans. Finally, serology by ELISA and Western blot were positive for Borrelia burgdorferi and confirmed Lyme disease.
Treatment: The treatment is beneficial for all stages of disease and if treated early, it is completely curable. Our patient received a sixty day treatment of Amocixicyllin 500 mg per os QID. Surprisingly, with this treatment, the lesions healed, leaving a discrete atrophic patch.
Conclusion: Acrodermatitis chronica atrophicans caused by American species of Borrelia, although very rare, has been described in a few case reports. We present a patient with chronic lesions, skin biopsy and serology compatible with Acrodermatitis chronica atrophicans.
BEAUTIFUL Miss Marnie!!! Thank you!!!Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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"P.S. Are we using the wrong class of abx. to combat OspB?"
From what I understand, Penicillin is the preferred treatment method for ACA...either old simple oral or IM Bicillin.
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sammy
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Interesting, thank you for sharing with your research with us Melanie:)
To answer your questions, yes I have been diagnosed with ACA.
Have you been diagnosed clinically or through serology? Pos. western blot
Has your LLMD offered treatment options, and if so, what are they? Not specifically for ACA, treating Lyme in general. You mentioned Bicillin, tried that for about 6mo. Switched to various orals. Have been on IV Rocephin for about 8wks now.
Have you been able to halt the progression with treatment? Hopefully yes, I have not noticed any new or worsening areas recently.
Do you know for certain where you were infected? Yes, about 5yrs ago, went hiking in near my home in OH, developed EM and symptoms shortly after.
Have you ever traveled to Europe or to other places outside of the states? Yes. London, city only, about a week in the winter. Asia, South and Latin America for longer periods of time.
Do you have photographic evidence of your condition? No but I guess I could try to take pictures if needed.
How long were you ill before this presented? 3-4years. Diagnosed with Lyme about a year ago. Dermatologist diagnosed ACA a couple months after Lyme dx.
What other diseases besides Lyme have you been diagnosed with? Babesia, Cpn, Mycoplasma P, secondary Addisons. Also have Lichen sclerosis.
What are your main symptoms? Too many. Fatigue, cognitive difficulties, neuropathy (peripheral and Vagal/GI), vision problems, these are probably the worse right now.
Do you know of others with ACA? Nope.
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Melanie Reber
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"Have you been diagnosed clinically or through serology? Pos. western blot"
Thank you Sammy. Was your WB for Bb or one of the other strains?
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sammy
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Yes, it was Bb.
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TO LIFE
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Dear Melanie,
I hope you don't have it.
My Lyme MD has always seened the Euro strain in me, which we have talked about ACA some. Let me explain my SX.
1.Inflammation with elevated sed. rate. Cardio MD wanted Vasculitis ruled out. My skin had a purple look to it. I had so much inflammation in the Brachial Plexus, I had to have my subclavicle artery released.
2. I get a blood blister rash come and go about every 3 weeks, just when I think their gone new ones appear. Their getting better, but still get some.
3. My skin on my arms and hands are thin. I used to do hairdressing so I thought it was from that.Arms are getting alot better.
4. I have a prior DX of RSD, which is very rough burning nerve pain with allodynia. I will look for a couple articles relating RSD to Lyme if anyone is interested.
5.Some left Vertricle heart damage. which I believe I am healing from.
6. I was bedridden for nearly 2 years.
7. Weak and fatigued.
8. Swollen lymph nodes.
9. Broken capullaries are in my skin.
10. Hair loss on my legs and arms, but not my head.
I have had to have very aggresive therapy. My Lyme MD has never given up on me and is very postive for my recovery. I have been on antibiotic treatment for 2 years. 7 months of various IV's.
I am doing 50% to 70% better. I believe I will have my life back, and be an asset to society again.
Jill E.
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Yes, my mother has it - she is American, not European. My LLMD confirmed it when doing a visual exam on my mother and saw ACA in several places, and I had already suspected it with my mom.
My mother has really bad lipomas, some are covered with ACA, but she also has ACA on non-lipoma areas of her body.
Jill
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Melanie Reber
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Thanks to each of you Jill, Life Sammy and Gael,
Do any of you have photos you would like to share with me? I am trying to compile visual evidence. I would keep the patient information strictly anonymous. Thank you for considering helping others in this manner. M
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Melanie Reber
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Lyme borreliosis (LB) is a multisystemic infectious disease involving the skin, joints, nervous system, heart, and eyes. Today at least three subtypes pathogenic for humans have been identified: Borrelia burgdorferi sensu stricto, Borrelia garini, Borrelia afzelii. Different genospecies strains of Borrelia have been associated with different clinical manifestations. LB is classically described as having three clinical stages or, similarly to syphilis, an early phase and a late one. The early infection corresponds to the first stage, the late infection includes the second and the third stages. LB skin manifestations could be divided into five classes. Erythema migrans, lymphadenosis benigna cutis, and acrodermatitis chronica atrophicans are proven skin manifestations of LB.
Lichen sclerosus et athrophicus, morphea, scleroderma, scleredema Buschke, atrophodermia of Pierini and Pasini, Parry-Romberg progressive facial hemiatrophy, and Shulman fasciitis are controversial LB manifestations. Granuloma annulare, atypical persistent pityriasis rosea, and pityriasis lichenoides are skin lesions occasionally related to LB. Urticaria, erythema nodosum, and papular acrodermatitis (Giannotti Crosti disease) are reactive LB skin manifestations. Nodular panniculitis (Pfeifer-Weber-Christian) (I think this is what I have growing inside of me!!!), B-cell cutaneous lymphoma, and juvenile chronic myeloid leukemia are exceptional skin manifestations of LB.
In the last years, there have been numerous and important advances of many aspects of Lyme borreliosis (LB). However, it appears that many questions concerning this disease remain unanswered. It is not clear, how spirochetes behave when they enter into the human body. They can produce pathognomonic lesions, skin manifestations mimicking other diseases, or clinical pictures that can be induced also by other etiologic agents.
We became aware of the complexity of this disease since genetic studies can identify different species of Borrelia burgdorferi (Bb) sensu lato responsible for human infections: Bb sensu stricto, B. garinii and B. afzelii. In Japan a new species was recently described, B. japonica, which does not appear to be a human pathogen. In future, additional species of Borrelia will probably be identified. The recurrent fever also is caused by several species of Borrelia, and the vectors are different ticks (B. recurrentis transmitted by Pediculus sp. in epidemic relapsing fever, B. caucasica, B.crocidurae, B. duttonii, B.hermisii, B. hispanica, B. mazzottii, B. parkeri, B. persica, B. turicatae, B. venezuelensis transmitted by Ornithodoros sp. in endemic relapsing fever). Diagnosis of LB is certain, when the infection is transmitted by a hard tick of genus Ixodes, and erythema chronicum migrans (ECM) appears.
Different Bb species and strains can have different organ tropisms and can induce different clinical manifestations. B. afzelii has been found in patients with acrodermatitis chronica atrophicans (ACA), while Bb sensu stricto in patients with arthritis and B. garinii in patients with neuroborreliosis. A possible Bb follicular hair tropism can explain the ECM with hair loss.
Atypical LB skin manifestations could be ordered in the following classes:
1. Controversial LB skin manifestations 2. Skin manifestations occasionally related to LB 3. Reactive LB skin manifestations 4. Exceptional skin manifestations during LB.
Skin lesions that appear immediately after tick-bite should also be mentioned: they can be mild and transient reactions, or, sometimes, edematous papular dermatitis of some centimeters of diameter, exceptionally with tissue necrosis.
Bb infection requires certain preconditions:
* Infected tick * Attachment of the tick to the skin * Appearance of enlarging erythema after an incubation period of at least 4-5 days
During the visit the tick can be observed on the skin, or sometimes the patient shows a detached tick in a little box. In such cases the tick bite is certain, while in all other cases the tick bite may be only supposed.
1. Controversial manifestations of LB
Essentially the following atrophic and sclerosing (sclerodermatous) disorders can be included:
Lichen sclerosus et atrophicus (LSA) has been related to Borrelia infection by Asbrink who noticed the frequent association between LSA and ACA. Aberer demonstrated the presence of Borrelia in LSA. It is characterized by sclerotic atrophic patches, sometimes confluent, and often located on genitals. In 3 children affected by LSA living in endemic areas, borrelial DNA was found in the involved skin, by PCR (2). No specific DNA was found in 4 cases affected by LSA living in non endemic area. The same authors confirmed the relation of morphea to Borrelia infection, while others maintain that there is no such correlation. Probably morphea can be caused sometimes by Borrelia afzelii (1).
The possible relationship between LB and LSA or morphea is suggested by the following evidence:
* Clinical and histological similarities between morphea, LSA and ACA. * The presence of antibodies against Borrelia burgdorferi in some patients with LSA and/or morphea. * Identification of borrelial organisms in histological sections. * The coexistence of ACA, LSA and/or morphea in the same patient. * A response to antimicrobial therapy in many cases of LSA and morphea.
2. Skin manifestations occasionally related to LB
Granuloma annulare (GA) has been described in association with LB. In some cases the author was able to find positive serological tests or spirochetal bodies in the affected skin by silver stain. In his experience the GA is very seldom related to LB. In 3 patients he detected Borrelia in the affected skin by PCR, but in these cases clinical evolution has been unusual and the treatment by nimesulide hasn't been effective.
Atypical persistent pityriasis rosea, lasting longer than 4-5 months, could be suspected of being related to LB.
The author is studying some children who developed a papular dermatitis with perifolliculitis, mimicking pityriasis lichenoides (2). In one case he was able to isolate Borrelia sp. in BSK from the involved skin. Further studies are necessary to confirm the relationship with Lyme disease (3).
3. Reactive skin manifestations of LB
Such manifestations can be observed also in other infectious diseases:
* urticaria, * erythema nodosum and * papular acrodermatitis
Two varieties of urticaria can be distinguished:
* diffuse * localized
The first form appears usually in early LB, whereas the localized form is more frequent in late LB. The localized form often involves the skin adjacent to the affected joints.
Erythema nodosum has also been observed during active LB. Recently, the author has reported two children who have developed papular acrodermatitis (Gianotti-Crosti disease) after borrelial infection.
4. Exceptional skin manifestations described during LB
Hassler (4) has reported an association between LB and involvement of subcutaneous tissue (Pfeifer-Weber Christian disease) and he has been able to demonstrate the presence of Borrelia in the affected skin even after several antibiotic treatments. There is also the problem of a possible correlation between LB and cutaneous B-cell (or T-cell) lymphomas. Evolution of borrelial lymphadenosis benigna cutis (LABC) towards malignancy has been supposed, but this hypothesis needs further investigations.
.......
I know there are a lot of big and confusing medical terms in this article, so if you just highlight what you, or anyone else, doesn't understand and open a new window to look it up, you will be able to make your way through this more clearly...at least that is what I had to do! Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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From the 2006 IDSA Guidelines...(I know, I know...but at least it is acknowledged) read treatment guidelines with this in mind.
Background and Diagnosis of Acrodermatitis Chronica Atrophicans
Acrodermatitis chronica atrophicans is a late skin manifestation of Lyme disease that develops insidiously several years after initial infection (range, 0.5-8 years) [109, 242]. Approximately 20% of patients have a history of a preceding erythema migrans lesion, usually of the same extremity [242]. Acrodermatitis chronica atrophicans is diagnosed most frequently in women greater than 40 years of age. Although any of the species of Lyme Borrelia may cause the lesion, by far the most common etiologic agent is B. afzelii. Therefore, this manifestation is much more common in Europe than in the United States [243-246].
Acrodermatitis chronica atrophicans occurs most often on the extensor surfaces of the hands and feet, and early lesions are characterized by a slight bluish-red discoloration and doughy swelling. Initially unilateral, the lesion may later become bilateral. The lesion enlarges slowly over months to years, in association with resolution of the edema and development of skin atrophy (figure 4) (sometimes referred to as ``cigarette paper skin''). Nodules may develop over bony prominences, such as the elbow or patella [197, 242, 247]. In some patients, sclerosing lesions develop. Because of atrophy of the skin, the veins become prominent, which may lead to a misdiagnosis of venous insufficiency [109, 197, 242]. Approximately two-thirds of patients have an associated peripheral neuropathy, typically involving the affected extremity, manifested primarily as local sensory loss [248, 249].
The diagnosis of acrodermatitis chronica atrophicans is based on appropriate epidemiology, clinical characteristics, histological findings, and IgG seropositivity. Histopathology shows a pronounced lymphoplasmacellular infiltration of the skin and sometimes also of the subcutis, with or without atrophy [195].
Evidence to support treatment recommendations.
Acrodermatitis chronica atrophicans does not appear to resolve spontaneously. There are no prospective, randomized studies on treatment. Oral or parenteral antimicrobial therapy (table 2) given for 3 weeks (range, 2-4 weeks) has resulted in improvement in pain and swelling, diminution in fibrous nodules, and gradual fading of the lesion within 2-6 months [250-252].
Atrophic areas often persist, and little objective improvement can be demonstrated in the neuropathy in uncontrolled studies, regardless of whether antibiotics are administered parenterally. However, progression of neurologic involvement is halted, and the neuropathic symptoms of pain and paresthesia are improved [251, 252]. In the United States, treatment of Lyme disease-associated peripheral neuropathy with intravenous ceftriaxone usually results in improvement. The reasons for the differences in the experience with this manifestation of the disease in the United States and Europe are not clear.
Recommendations
1. Available data indicate that acrodermatitis chronica atrophicans may be treated with a 21-day course of the same antibiotics (doxycycline [B-II], amoxicillin [B-II], or cefuroxime axetil [B-III]) used to treat patients with erythema migrans (tables 2 and 3). A controlled study is warranted to compare oral with parenteral antibiotic therapy for the treatment of acrodermatitis chronica atrophicans.
Jill E.
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Hi Melanie,
I don't think I can ask my mom to take photos - she is really resistant to going through Lyme/co treatment at this late stage of her life. She has seen me, and then my dad, suffer so much with this illness that I can't bring it up at the moment. But if things change, I'll let you know.
Jill
-------------------- If laughter is the best medicine, why hasn't stand-up comedy cured me? Posts: 1773 | From San Diego | Registered: Apr 2006
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Melanie Reber
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As I get rid of the infections... parasites/worms/bb etc that I believe are inhabiting the small intestine my hands are coming back to normal.
It seems like the infections in the small intestine were spilling over and desseminated into the hands/head/organs etc.
Hello Gael, I actually ran across something the other day that DID speak to fillial (sp) worms. Gosh my memory is so bad right now...but I will try to find it again and post. Thank you!
I completely understand Jill, not to worry.
THANK YOU Sammy!!!
Roz, I'm so happy you have a new comp. I would be more than happy to help you with any photo questions, just ask, OK? I am not real sure exactly WHAT I have right now...still trying to lean as much as I can and put the mystery together. But, I AM making progress! Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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CLINICAL AND LABORATORY INVESTIGATIONS
Morphoea: a manifestation of infection with Borrelia species? K. Eisendle, T. Grabner and B. Zelger Department of Dermatology and Venereology, Innsbruck Medical University, Anichstra�e 35, 6020 Innsbruck, Austria
Background Morphoea or localized scleroderma is a cutaneous inflammatory disease with still unknown aetiology. Borrelia burgdorferi as causative agent has been discussed controversially.
Objectives To assess the evidence for infection with B. burgdorferi in patients with morphoea by focus-floating microscopy (FFM).
Methods Using standard histological equipment, tissue sections stained with a polyclonal B. burgdorferi antibody were simultaneously scanned through in two planes: horizontally as in routine cytology, and vertically by focusing through the thickness of the section, i.e. FFM. Part of the material was also investigated with a Borrelia-specific polymerase chain reaction (PCR).
Results One hundred and twenty-two cases of morphoea and 68 controls (58 negative and 10 positive by PCR) were investigated for the presence of Borrelia within tissue specimens. Using FFM Borrelia was detected in 84 cases (68�9%) of morphoea and in all positive controls, but was absent in all negative controls.
Borrelia was significantly more frequent in early inflammatory-rich (75%) than late inflammatory-poor (53%) cases (P = 0�018). What seemed to be vital microorganisms were mostly found close to the active border, while degenerated forms were more common in fibrosclerotic parts. The presence of B lymphocytes determined by CD20 staining proved to be a good positive predictor of the microorganism (correlation 0�85, P < 0�001). Borrelia-specific DNA was detected in only one of 30 cases of morphoea analysed by PCR.
Conclusions FFM is a reliable and highly sensitive method to detect Borrelia in tissue sections. The frequent detection of this microorganism in morphoea points to a specific involvement of B. burgdorferi or other similar strains in the development of or as a trigger of this disease.
Health
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Hi,
I would say I have ACA, and it started to progress even while IN lyme treatment, and now I am treating Babesia and it is getting better I would say, my facve almost looks normal,
I was starting to get it on my face, and that is very upsetting. I am on Mepron and Azithromycin for about 10 months now, and had to stop this treatment because it was too strong, but now have been back on it for some time now.
I feel because my Babesia in the past had not been treated long enough, I have the strain Babesia Duncani, and this strain my LLMD says takes 10 or more months of treatment,
as well I had untreated lyme for about 15 years, and untreated Babesia for about 18 years.
I will post on here occasionaly to put in my comments, I hope that I dont have the ACA permanent.
If Babesia is not treated, for me anyways, the Lyme got worse, because immune system worse.
I have also had the hands ITCH like heck from this Mepron and Azithromyin where the ACA is, the ACA that is on me is not as bad as the pictures, and I hope that treating,
the Babesia and then the Lyme again, and possibly Bartonella? I will no longer have any ACA signs.
If you have ACA treat Babesia if you have not.
I also got sick in Europe and I live in Canada.
So maybe got it over there.
Hope this helps.
Will post another time, if I get worse or better.
I even have the ACA starting to show on eye lids, and had the eye lids itch like heck from herxing, now I look pretty good almost normal.
My face is getting better, through treating babesia.
Trish
Posts: 1250 | From Canada | Registered: Aug 2004
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hshbmom
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Hi Melanie,
My hands look very dry and leathery regardless of the time of year. My kids say I have "Grandma hands." I'm not THAT old. Ha ha.
Some day I'll take some photos and send them to you.
I never remember to ask my good doctor about them.
My family member has the swelling all over that you described recently. Diuretics don't help. It looks like they take massive doses of steroids. I think it's vasculitis. The fluid & protein leaks out of the blood vessels, then more fluid follows to dilute the protein.
Your skin coloring looks like mine, how is your circulation?
I am doing much better. I believe 100% you can go into remmision with ACA as well. I am 65% percent better and have been in treatment for 2 years.
How are you doing as far as detoxing goes?
My treatment right now is red clover therapy, it has several herbs in it and is supposed to cleanse the blood. My thinking is, if the blood is clean no disease can live.
I am also taking just a couple humaworm tablets a day.
I just bought this real cheap tent sauna for 129.-maybe it will help as well.
I am still trying to trace down the people that took photo's of my arm before treatment.
Melanie Reber
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Livedo racemosa: an unusual late manifestation of borreliosis? Baumann M, Tebbe B, Arnold M, Krengel S, Goerdt S, Orfanos CE. Hautarzt. 2000 Aug;51(8):593-6.
Klinik und Poliklinik f�r Dermatologie, Universit�tsklinikum Benjamin Franklin, Freie Universit�t Berlin.
Classic variants of cutaneous borreliosis are erythema chronicum migrans (ECM), lymphadenosis benigna cutis (LBC) and acrodermatitis chronica atrophicans (ACA). Other dermatoses have been reported in the literature as possibly linked to borreliosis.
A 59-year old female patient was seen in the late phases of cutaneous borreliosis with histologically confirmed ACA. In addition, prominent livedo racemosa was seen on the legs, also showing tissue changes similar to those of ACA.
Borrelia burgdorferi infection was serologically confirmed by the presence of anti-IgM and anti-IgG antibodies. The clinical spectrum of late cutaneous borreliosis should be enlarged to include livedo racemosa.Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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Acrodermatitis chronica atrophicans in a 15-year-old girl misdiagnosed as venous insufficiency for 6 years. Zalaudek I, Leinweber B, Kerl H, M�llegger RR. Department of Dermatology, Medical University of Graz, Graz, Austria. J Am Acad Dermatol. 2005 Jun;52(6):1091-4.
Acrodermatitis chronica atrophicans, the characteristic cutaneous manifestation of late Lyme borreliosis, typically occurs in elderly women. To our knowledge, only 4 cases of acrodermatitis chronica atrophicans in children have been described. Prompt diagnosis and treatment are important to prevent progression of disease and extracutaneous complications.
We describe a 15-year-old girl with acrodermatitis chronica atrophicans of the left leg that had been misdiagnosed as chronic venous insufficiency for 6 years. Because of the long-standing disease course, skin changes expanded and progressed to marked atrophy. The correct diagnosis was finally based on clinical, histopathologic, and serologic findings. The girl was treated with oral doxycycline for 6 weeks, but her skin changes did not fully normalize.
This case illustrates the possibility of acrodermatitis chronica atrophicans appearing in childhood and the difficulties in differentiating vascular disorders from acrodermatitis chronica atrophicans on the basis of the clinical appearance alone.
Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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Well, it is official... the reason I am a wrinkled smurf is because I have ACA! I LOVE my LLMD!
So, we are going to anti-smurf me soon. Up for more possible ACA people?
Melanie Reber
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Yes... it is sort of odd that after 6 years (how many 30 day treatments would that make? Ummm 73 rounds of curative treatment) of MASSIVE ABX...
I still am presenting with an acute Borrelia infection.
Hmmmm, someone has to be misinformed here somewhere. I wonder who that could be?
Shoes would be lovely, but only if they go with my new pink toenails. Posts: 7052 | From Colorado | Registered: Mar 2003
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Pinelady
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Great Work. Are the bottoms the same and is the
toe flaring signs of neuro involvement?
I would love to
see um whats his names face at the IDSA review
when and if they get to see that hot pink!
-------------------- Suspected Lyme 07 Test neg One band migrating in IgG region unable to identify.Igenex Jan.09IFA titer 1:40 IND IgM neg pos 31 +++ 34 IND 39 IND 41 IND 83-93 + DX:Neuroborreliosis Posts: 5850 | From Kentucky | Registered: Dec 2008
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Melanie Reber
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Good morning Lady of the pines,
Are you asking if the bottoms of my feet are smurfing too? If so, actually no, they remain either yellow or white or red, I am almost patriotic, huh?
However, the inside of my palms under the thumbs... and both of my forearms are turning blue too now.
Mostly things change pretty rapidly, so I am very happy that I have kept up with the photos to chronicle this weirdness, as I don't think anyone would believe me otherwise.
Having said that... while in my LLMD apt yesterday morning; I showed the docs my purple (red AND blue) freezing feet and my blue palms and forearms. While at the same time, my yellow and blue hands were burning hot. So, at least they were witness to this weirdness.
Yes, I do think the toe flaring is something weird as well. This is new for me. But I am also experiencing severe cramping... so who knows what the actual cause is. Could be multiple reasons.
Although, one sign of ACA IS an outward flaring of the extremity digits... ~sigh~
Hope YOU are doing well? M
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Melanie Reber
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IDSA 'Recommendations' from above guidelines:
1. Available data indicate that acrodermatitis chronica atrophicans may be treated with a 21-day course of the same antibiotics (doxycycline [B-II], amoxicillin [B-II], or cefuroxime axetil (Ceftin) [B-III]) used to treat patients with erythema migrans (tables 2 and 3). A controlled study is warranted to compare oral with parenteral (IM or IV) antibiotic therapy for the treatment of acrodermatitis chronica atrophicans.
*21 day course... OK, my mistake... so that means I have actually done 104 curative courses.
*doxycycline... Ah, I guess that first 2 weeks of Doxy didn't do it after all? Nor did the other few months of Doxy, well into treatment?
*amoxicillin... And the 3 months of Amoxy up to 6 grams, that followed the Doxy, wasn't enough either?
*cefuroxime axetil (Ceftin)... OK, so now I see where I went wrong... I have never taken Ceftin!
*compare oral with parenteral (IM or IV) antibiotic therapy... Hmmm, the 15 months of IV and subsequent 4 months of IM as compared to the continuous 6+ years of oral therapy really HAS been worth studying. ...
What is it that guy with the mustache says? GIVE ME A BREAK!
(Can you tell I am a bit peeved? )
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Pinelady
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OK thanks for asking. I haven't hit the ER so I guess I'm good. Bump up to Eric555 about 5inches up. He's got Ceftin. Hang on to that humor and you will get through this.
-------------------- Suspected Lyme 07 Test neg One band migrating in IgG region unable to identify.Igenex Jan.09IFA titer 1:40 IND IgM neg pos 31 +++ 34 IND 39 IND 41 IND 83-93 + DX:Neuroborreliosis Posts: 5850 | From Kentucky | Registered: Dec 2008
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Tincup
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I thought the upward toes are how it is suppose to be when you CA dudes are surfing on those boards?
Truthfinder
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Melanie, I probably have some kind of ACA presentation, but I'm stumbling over all the medical terminology that describes what ACA is or resembles. I've looked at the photos; some look similar but the resolution on my screen isn't the best.
Like TO LIFE, two different punch biopsies on my back came back as Lichen Simplex Chronicus. That was several years ago. The LSC starting kicking in about the same time as other Lyme symptoms began to emerge (didn't know it at the time, of course).
But I have two different, new things going on the last 2-3 years, too, though one manifestation is similar to the LS in how it changes and damages my skin.
First, I get all these strange color changes in my skin when I shower. Redish blotches on my arms and other areas. My hands or fingers can change color. It eventually fades and returns to normal. When I look down at my feet, I've noticed this dusky color where my leg meets my foot - that wasn't there until about a year ago. I've noted no changes in skin texture or appearance with this. But I have noticed that my ankles are much thicker than they ever were, and I have pooling `edema' below my ankle bone.
But there's something else, too, similar to the LS. The Lichen Simplex stuff starts off with a tiny itch - like one single pore in my skin is irritated. So, I scratch it. If I continue to scratch it, it becomes a rash with several pores irritated. Then the texture of the skin changes, it gets a reddish color, kind of hard and inflexible, often with little scales, and I have a full-blown patch of Lichen Simplex.
I was told at Mayo Clinic to treat them with a prescription steroid cream, which does work. But the skin is often `wrinkled, dry, and translucent' when it's all over.
These latest `patches' look similar - slightly darker than normal skin or reddish, and can either be scaly or not. But there is never any itch associated with them - they just appear. I've had one on my L knee, and one on my right thigh and now the knuckles of both hands are affected. They last for several months. I don't know if steroid cream would work as I haven't tried it. But nothing else works that I've tried.
Eventually, the `rash' fades and leaves the skin `wrinkled, dry, and translucent'. Or in the case of my knuckles, it's almost like scar tissue or like when you get a sunburn - like kind of hard and not as flexible. It's almost like dead skin - it isn't anything like the skin I had before. Maybe that would be termed `sclerotic'. And none of these areas has ever returned to normal.
BTW, I've never been to Europe.
So, I'm not sure what I have, really. I do know that we have B. bissettii and B. parkeri here in Northern Colorado and in this county (found that in an old NIH document), which could be implicated.
B. bissettii is a common Borreliosis culprit in parts of Europe (like Slovenia). Here in Northern Colorado, reservoirs for B. bissettii appear to include chipmunks and ground-squirrels.
As it turns out, I had both a wild chipmunk and a wild ground-squirrel as pets between the ages of about 9 and 15. I don't know if ACA is common in B. bissettii infections in Europe, though, so I'm not sure if there is a connection. I handled both animals a lot and don't recall any fleas or ticks on them, nor was I ever bitten that I remember.
I suspect I'm in the ACA group, but just not sure.
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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Good morning Tracy,
Thank you so much for stepping forward! Yes, the medical terminology is/was very difficult for me as well. I have/had to keep looking up every other word in every abstract or article I read... so that slows things way down.
By now, though, I am beginning to get a tad more familiar with most terms.
I get all these strange color changes in my skin when I shower. Redish blotches on my arms and other areas.
Yes! I could NOT have described this better myself. It is as if the skin looks burned from the warm shower water! But, it is patchy in unexpected places, but mostly for me, on my hands, feet and certain areas of my legs.
I don't know WHAT this is, but feel it has to be connected somehow. My theory is... we have very poor circulation that goes along with ACA, most times, my extremities are freezing cold- thus the bluish hues. So, when exposed to warm water, the circulation increases to those cold areas, turning them red.
It is almost the same phenomena as frostbite. The frozen skin changes color to a bluish hue, and when re-exposed to warmth, if it is not too late, it turns bright red.
But I have noticed that my ankles are much thicker than they ever were, and I have pooling `edema' below my ankle bone.
My exact experience. And the photos I posted verify this. I need to take recent pictures, as the edema has subsided, but still there remains a blob right behind the ankle bone.
I was told at Mayo Clinic to treat them with a prescription steroid cream, which does work. But the skin is often `wrinkled, dry, and translucent' when it's all over.
So sorry... but I shudder to even hear that name. I'm sure you are aware by now as to why? As you know, the `wrinkled, dry, and translucent' skin IS the atrophy process associated with ACA.
Goodness, knowing you are in CO and have never been to Europe really intrigues me. I lived there (Durango and then Denver) for 23 years and was bitten by numerous ticks the whole time. CO is known to have some very interesting ticks that carry very interesting diseases. One just needs to dig a bit deeper to find the proof, and I am so thankful that you have done just that!
No, I never saw ONE flea while in CO either. But, when I moved here to CA, my cat became immediately infested. I didn't even know what one was supposed to do for fleas! But we figured it out. Unfortunately, by that time, the damage had been done... the cat became very ill and I relapsed terribly.
When I can make the time and find the energy to research your theory of B. bissettii... I will be certain to post it.
Meanwhile... I am not a physician, obviously, but what you are describing, at least to me, most certainly puts you in the ACA/ Scleroderma category. I'm so terribly sorry, but also so pleased that you have shared such valuable information. I do hope you will seek the help you need to take care of this before it progresses further.
We are currently collecting stories like yours and photos for educational use. If you would be willing to send some photos my way, it would be more than appreciated!
Melanie Reber
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up... for others?
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Melanie Reber
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Solitary borrelial lymphocytoma in adult patients. [Journal Article] Maraspin V, Cimperman J, Lotric-Furlan S, Ruzić-Sabljić E, Jurca T, Picken RN, Strle F Wien Klin Wochenschr 2002 Jul 31; 114(13-14):515-23.
During the period from 1986 to 2000, 85 adult patients with solitary borrelial lymphocytoma were diagnosed at the Department of Infectious Diseases, University Medical Centre Ljubljana, Slovenia. There were 36 (42.4%) females and 49 (57.6%) males with a median age of 49 (15-74) years.
Borrelial lymphocytoma was located on the breast (nipple--areola mammae region) in 68 (80%) patients, on the ear lobe in eight (9.4%), and in other locations in nine (10.6%). A concomitant erythema migrans enabling clinical diagnosis of Lyme borreliosis was registered or reported in 67 (78.8%) patients. Fifteen (17.6%) patients had no accompanying symptoms, 34 (40%) reported local and constitutional symptoms, 23 (27.1%) recounted only local symptoms, and 13 (15.3%) patients had solely constitutional symptoms.
Clinical findings indicating early disseminated borrelial infection were observed at the first visit in 12 (14.1%) patients: six (7.1%) had multiple erythema migrans, one had meningitis, one meningoradiculitis and arthritis, one radiculoneuritis and arthritis, one peripheral facial palsy and concomitant meningitis, and two arthritis. In addition, one of the patients with borrelial lymphocytoma on the breast had acrodermatitis chronica atrophicans.
A seropositive response to borrelial antigens was found in 30 (35.3%) patients at the initial examination. In 11/46 (23.9%) patients, infection with Borrelia burgdorferi sensu lato was confirmed by isolation of the agent from lymphocytoma tissue. Eight out of nine (88.9%) typed borrelial strains were found to be B. afzelii, and one (11.1%) B. bissettii.
Patients were treated with doxycycline, azithromycin, amoxycillin, cefuroxime-axetil, phenoxymethylpenicillin, or ceftriaxone. Median time to complete disappearance of lymphocytoma was 28 days (range 7-270 days) after the institution of antibiotic treatment; disappearance took longer in patients with prolonged duration of the skin lesion prior to treatment.
Treatment failure was registered in 11 (12.9%) patients who were later re-treated. The outcome of borrelial infection assessed at the end of a follow-up period of one year was favourable.
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Melanie Reber
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Borreliosis--increasing clinical problem [Journal Article] Dybowska D Wiad Lek 2006; 59(1-2):23-6.
Lyme disease (LD) is due to infection with Borrelia burgdorferi (B. burgdorferi). We analysed some aspects of epidemiology and clinical manifestation of borreliosis. We tried to estimate the efficiency of diagnostic methods and treatment.
We analyzed medical documentation of 300 patients with LD treated in our department between 1993-2001. The diagnosis was made according to Lyme Disease Foundation's criteria. Patients suffering from LD were divided into 3 groups according to stages of the disease.
The most frequent manifestation of LD was erythema migrans (EM). The number of LD cases had increased during the observation time. The exposition to tick-bites was greater during summer and early autumn. The great number of patients with EM was observed at the same time.
Cases of Lyme arthritis (LA), disseminated EM, Lyme carditis (LC) and neuroborreliosis represented the group number 2.
LA, uveitis and acrodermatitis chronica atrophicans (ACA) were diagnosed in the third group of patients.
Serological markers of B. burgdorferi infection were found in about 50% of cases of EM and in each patient in group 2 and 3. Complete recovery after antibiotic therapy was observed in every case in early LD and partial one in the late stage.
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Truthfinder
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Melanie,
Interesting that you found a case where B. Bissettii may be implicated in ACA.....
I think B. bissettii is a `new name' for what were once classified as European DN127 type strains..... it gets confusing. I do have a small collection of excerpts and links regarding Colorado strains if you want me to send those to you. It's been awhile since I did any research on it.
Not much attention is given to B. bissettii here in the USA, I think mostly because of articles that say stuff like this (vintage 2004 - Allen Steere was involved in this one, no surprise):
quote:Similarly, in Colorado, wood rats and I. spinipalpis ticks may be infected with Borrelia bissettii, one of the nonpathogenic species, in a cycle that is not known to cause human infection (13). http://www.jci.org/articles/view/21681/version/1
Well, that's just not true in Europe, so the above statement is extremely misleading.
As for photos, I've had a digital camera for 3 years and still haven't taken the time to figure out how to download the software and get photos transferred onto my computer, lol. This computer has been such a snarled up mess since I got it that it sort of takes the fun out of using it.
Anyway, I've been checking my body the past few days for picture-worthy `examples' of possible manifestations..... very disappointing! I haven't had a single after-shower reddish splotch show up, except a couple of very small, vaguely-pink spots that won't show up on camera. I'm sure they will be back, but for now they are quiet.
I should get someone else to take pics of the odd skin on my knuckles. And perhaps the thick ankles and pooling edema below my ankle bones. Those are the only things that MIGHT show up decently on film.
Oh, and I forgot to mention that I, too, have the `droopy eyelid' thing, only it's different on each eye. It isn't really the eye `lids' themselves, so much; it's that skin below my eyebrow (like in your photo). AND, I developed a puffiness below my eyes - mostly one side - in that `dark circle' area, too. That happened about a year and a half ago. The puffiness comes and goes, but never completely leaves.
Also forgot to mention that before I posted last time, I had some kind of skin eruption on my scalp - like 2 pimples or bug bites - right near the hairline on the back of my neck. I'd never had that before, but saw something similar in your photos.
I think you are probably right about the circulation issues. My feet are often very cold, but they will still sweat, which just makes things worse. So, I have clammy, cold feet in both winter and summer.
Last winter, I finally broke down and bought several pairs of Smartwool socks (washer-friendly), and it DID make a difference. For some reason, my feet either don't perspire as much, or the moisture gets wicked away. I even bought 3 summer-weight pairs recently but don't know how they will work out yet. I usually itch terribly from wool but I haven't had a problem with the Smartwool. (I did wash them a couple of times before wearing.)
I also have periods when my feet are hot and burning. Though I don't have the color changes you mentioned, it always reminded me of frostbite - first freezing cold, then when they get warm, they go to the extreme.
This appears to be related to what I eat and how much I eat more than anything. And if I don't eat enough, or try to stick with mostly vegetables, I can't get warm. Even in bed with lots of blankets and comforters. I'm just not generating enough heat (circulation again?). It's just so crazy.
Re fleas: I feel so lucky that I just haven't had a problem. Fleas around here can carry the Plague, among other things. Cats are particularly susceptible since they hunt birds and rodents.
And with the crazy deer sleeping in my back yard several days a week, squirrels in my trees (that someone imported from somewhere 10 years ago), Mourning Doves that appear to be nesting in my darn chimney (!) right here in town, you'd think I'd have lice, fleas, and ticks to deal with in my cats and dog. But I haven't yet. The only time I ever had fleas here was when I boarded my dog for few days and he came home with fleas. What a mess.
I realized that I never answered the questions you posted .... well, I won't be much help there but here are the answers:
"Have you been diagnosed clinically or through serology?" Neither, really. Had an ultimate high-positive Bowen test in 2005 and low CD57 test last year.
"Has your LLMD offered treatment options, and if so, what are they?" I don't have an LLMD, or a Lyme doc of any kind.
"Have you been able to halt the progression with treatment?" Haven't been on any specific medical protocol.
"Do you know for certain where you were infected?" Nope. Probably had this since I was a kid and I grew up here. Spent countless hours out in the brush with livestock, horses, pets, and critters of every kind, had attached ticks regularly in summer. There were some early symptoms but nothing overt until mid-1980s.
"Have you ever traveled to Europe or to other places outside of the states?" Nope. Spent time in both Alaska and Hawaii, traveled through Canada and some into Mexico, but never have been overseas.
"Do you have photographic evidence of your condition?" Not yet, but if I get some, I'll send it to you.
"How long were you ill before this presented?" Hmmm. If we consider the `Lichen Simplex' issue, then I've had this going on since about 1992.
"What other diseases besides Lyme have you been diagnosed with?" Actual diseases? Not many. If we include conditions and syndromes, then the list is pretty long. I'd have to think about it, but if you want all that, I can gather the info.
"What are your main symptoms?" Musculo-skeletal problems (bulging disks, degeneration), fatigue and sleep issues, cognitive issues, and digestive/elimination problems. Those are my top 4 at this moment.
"Do you know of others with ACA?" No.
Gee, sorry this is so long!
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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Good morning Tracy,
yes I would be very interested in seeing what you have re: the strains in CO. As you may or may not know; I am trying to keep track of each state's known ticks and diseases that they carry on the Lyme Memorial site, and would really like to add to the CO page with more info that you can provide.
Yes, things do get confusing in the naming of these buggers, as they change so quickly as more are discovered. It takes a lot of research to try and keep things straight.
For example, if you read the abstract below, you will see that new strains of Borrelia have been found just recently in the Carolinas. B Bissettii was also implicated as 'a diverse group' found in the wildlife there.
I can completely relate to trying to learn new tech things when ill. I had a printer for over 2 years before I finally was well enough to figure out how to hook the darn thing up. (still have not tackled the fax capabilities)
As far as transferring photos from your camera... you may want to try what I have done with the borrowed camera I am using.
Get a cable that hooks into your camera on one end and into your comp USB port on the other. Then, you can turn on the camera while in the replay pictures mode, and open up a new window of 'My Computer'. Now you will see the camera listed, simply double click on the camera icon, and your PC should walk you through an instant download wizard w/out any additional software.
Hope that was clear enough? It is certainly worth a try, and if it works for you, you will not have to do anything else to download any images from now on.
I find it really interesting that you have found circulation and body temp issues are related to what you eat. I too go through periods, almost daily, when I sort of crash mid-afternoon. I just have to lay down or fall down. So I make my way to the sofa and cover up with down. Even pull it over my head.
It matters not what temperature is is inside.. I begin to shiver uncontrollably until I fall asleep. usually to awaken in a sweat.
I think most of that has to do with my endocrine system being pretty messed up. But my doc keeps harping on my eating habits, which are basically nil. She tells me that if I would just eat consistently throughout the day, this crazy pattern would begin to regulate itself.
I'm still smiling about the flea comments. I lived at 7,200 elevation there, and NEVER saw a flea.
You don't have a Lyme doc? At all? Who is treating you then? Sorry to pry, I am just concerned.
And please do not apologize for the length... you are providing wonderful information, and I really do appreciate it! M
Melanie Reber
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Borrelia carolinensis sp.nov. - a new (14th) member of Borrelia burgdorferi sensu lato complex from the southeastern United States. [JOURNAL ARTICLE] Rudenko N, Golovchenko M, Grubhoffer L, Oliver JH J Clin Microbiol 2008 Nov 19.
Approximately 118 Borrelia isolates were cultured from a variety of rodents, birds and ticks collected in the southern USA. In addition to a highly diverse group of Borrelia bissettii strains, and a homogenous group of Borrelia burgdorferi sensu stricto strains, a group of 16 isolates with unusual characteristics was found.
Isolates were cultured from ear biopsies of the rodents Peromyscus gossypinus and Neotoma floridana trapped at 5 localities in South Carolina. Multilocus sequence analysis of rrf-rrl intergenic spacer, 16S rRNA, flagellin, ospA and p66 genes were used to clarify the taxonomic status of the new group of B. burgdorferi sensu lato isolates. Thirteen species of B. burgdorferi sensu lato complex were used as controls. Unique RFLP patterns of rrf-rrl intergenic spacer region and flagellin gene were recognized. Unique signature nucleotides were also found in the 16S rRNA gene. Phylogenetic analysis shows that the 16 new isolates cluster together but separately from the other species in the B. burgdorferi sensu lato complex.
Our data strongly support the recognition of the 16 isolates as a new B. burgdorferi sensu lato species. We propose to name this genospecies Borrelia carolinensis with respect to the place of its currently known geographic location.Posts: 7052 | From Colorado | Registered: Mar 2003
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Previous work described Borrelia burgdorferi sensu lato group DN127 as a new genospecies, Borrelia bissettii, and prompted the present study to identify the Borrelia spp. that exist in northern Colorado.
To determine the genospecies present, we analyzed two specific intergenic spacer regions located between the 5S and 23S and the 16S and 23S ribosomal genes.
Phylogenetic analysis of the derived sequences clearly demonstrated that these isolates, originating from rodents captured in the foothills of northern Colorado, diverged from B. burgdorferi sensu stricto by 5 to 5.5% and were members of the new genospecies B. bissettii.
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Three multiplex assays for detection of Borrelia burgdorferi sensu lato and Borrelia miyamotoi sensu lato in field-collected Ixodes nymphs in North America. [Journal Article] Ullmann AJ, Gabitzsch ES, Schulze TL, Zeidner NS, Piesman J J Med Entomol 2005 Nov; 42(6):1057-62.
Two hundred fifty New Jersey field-collected Ixodes scapularis Say ticks and 17 Colorado Ixodes spinipalpis Hadwen & Nuttall ticks were tested using three separate multiplex real-time polymerase chain reaction (PCR) assays.
One assay targets the rrs-rrlA IGS region of Borrelia spp. to detect Borrelia burgdorferi sensu lato (s.l.) and Borrelia miyamotoi s.l. The second assay targets the ospA region of B. burgdorferi s.l. to detect B. burgdorferi sensu stricto (s.s.), Borrelia bissettii, and Borrelia andersonii. The final assay targets the glpQ region of B. miyamotoi s.l. to differentiate B. miyamotoi LB-2001 and Borrelia lonestari.
A testing scheme combining these tests yielded 18% of tested I. scapularis ticks surveyed from New Jersey positive for B. burgdorferi s.s., 3.2% I. scapularis ticks positive for B. miyamotoi LB-2001, and 41.2% I. spinipalpis ticks positive for B. bissettii surveyed from Colorado.
...........
41.2% is rather high considering... don't you think? Posts: 7052 | From Colorado | Registered: Mar 2003
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Truthfinder
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I'll write more later on, but.....
Great information about Borrelia carolinensis, Melanie. Borrelia b. may be just the tip of the iceberg.
Oh, I hadn't seen that 2005 article - wow! 41.2% of Borrelia bissettii in Colorado ticks seems pretty darn high to me, for sure! Nice work, Melanie.
In fact, isn't that a much higher incidence rate (in ticks) than we see in the Northeast for most Borrelia burgdorferi strains?
This could be very important information!
The first article you posted in one that I have, and then there's another one that is similar from 2002. I'll send you what I have, but it won't be until later. I have more info somewhere about B. bissetti and I can't seem to find it. Grrr. And I see that to my articles don't take you to the article itself..... grrr. Well, I've copied enough info from the articles that it can be found using a Google search, I guess.
Here's an excerpt that I thought was extremely interesting..... it confirms why there are so many negative serological results out there......
quote:......strains genetically similar to those of B. bissettii from New York, California, South Carolina, and Florida have been isolated from several humans in Slovenia . Those patients had clinical presentations ranging from relatively benign illness to some severe afflictions. Some of the patients had variable and unpredictable serologic responses, including a lack of antibody response despite disseminated disease (29). Interestingly, some of the Lyme disease patients in the southern United States also lacked a serologic response to antigens derived from B. burgdorferi sensu stricto (9).....
Gotta go finish another post and get busy here......
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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Hi Trish, I'm so sorry, I wasn't trying to ignore you, I just somehow missed your reply.
If you have ACA treat Babesia if you have not.
Yes, Babs has been a difficult one for me to treat. It took me way too long to admit that I even had it, and when I did treat, it was for about a year and a half.
Now, it has returned, and has apparently teamed up with a second strain of Duncani as well. So, yes, we have been going after it pretty aggressively.
I have also had the hands ITCH like heck from this Mepron and Azithromyin where the ACA is...
I did ask my LLMD about this insane itching that happens to me on my legs anytime I try to go for a walk. I was told it is a manifestation of neuropathy! Well, of course I have the numb and tingling PN, but had no clue that the itching was also PN.
This does make sense, because an early symptom of ACA IS PN of the extremities. So perhaps this will help to explain why your areas of ACA itch?
I'm very pleased to know that you are doing better with treatment!
Hey Roz, sorry I somehow missed your last reply as well.
Your skin coloring looks like mine, how is your circulation? I am doing much better. I believe 100% you can go into remmision with ACA as well. I am 65% percent better and have been in treatment for 2 years. How are you doing as far as detoxing goes?
My circulation stinks. I am so pleased you are doing so well! This is what I have been told as long as you get straight to treating effectively. My detox ability stinks too. Posts: 7052 | From Colorado | Registered: Mar 2003
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Melanie Reber
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Thanks for the additional info Tracy.
Ya know what... this really disturbs me. This is NOT rocket science! If WE can find the info we need to connect the dots after only looking for a few minutes... and only having access to very limited information...
WHY can't the 'powers that be' do the same?????
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Carol in PA
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quote:Originally posted by Melanie Reber:
During the period from 1986 to 2000, 85 adult patients with solitary borrelial lymphocytoma were diagnosed....
Borrelial lymphocytoma was located on the ear lobe in...9.4%...
I have had little "blind pimples" on my earlobes since my teen years. I have some right now. The LightWorks healed them in a few days, after more than six months, but there is still something there.
This is the best pic I could find:
Posts: 6947 | From Lancaster, PA | Registered: Feb 2004
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Melanie Reber
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Hey there Miss C, That is actually a great picture of Borrelial lymphocytoma. (and rated G as well, thanks)
I know we have spoken before about overlapping symptoms... I so wish we could get you on some ABX to see what would happen with you.
Love, M
PS...I know I owe you an email, actually a few. Just know that I appreciate them and you so much!
Posts: 7052 | From Colorado | Registered: Mar 2003
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Truthfinder
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Melanie, I just sent you some Colorado info via your e-mail addy at the Lyme Memorial Project. I hope it was okay to use e-mail...... it will be more readable than through the PM system here (which still appears to be a bit unreliable).
Well, I still don't have time to post much this morning - and we've got some thunder and lighting going on close by, so I'll probably have to shut down the computer pretty soon anyway.
I'll get back here eventually.......!
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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Thank you SO much Tracy,
I'm afraid I am really dragging on the newest meds, but will get back with you as soon as I can.
Truthfinder
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Thanks for the camera tip, Melanie. I actually printed that out. I actually bought some little USB cable thingie so that I wouldn't have to use the camera batteries to download..... and it is still in the package, lol. I just want to make sure that I can edit photos once they are off the camera. Somebody told me that they couldn't do that on the computer. That didn't sound right, but again, it's probably a software issue.
Yes, I think eating small meals through out the day would probably be the best. But like you, I just have trouble doing this.
My temperature fluctuations are not only annoying but because they vary so much, I never know how to dress for the weather if I go somewhere! Gee, will I be freezing today or not? Like you said, the actual temperature is only a starting point; it's my internal temp that dictates my comfort level. I've acquired so many clothes that a `normal' person doesn't need because I must wear at least 2 layers, even in summer.
Example of the madness: Just a couple of hours ago, I was wearing a winter watch cap here at the computer! Yep, it's true. I ate a small `vegetable' meal last night, had trouble getting warm in bed, and then, of course, when I got up this morning, it felt like an icy breeze was blowing on my head. But its 68 degrees here at the computer. Craziness!
No, I have no Lyme doc, even an alternative one. I'm just sort of winging it at the moment. If I treat with someone, it will be a homeopath. I don't tolerate drugs or herbs or supplements well so it's the best choice for me, really. Not only that, I think homeopathy is a terrific system of medicine. Thanks for your concern - I appreciate that.
One thing I thought of re the `source' of my Lyme and probable ACA.....
Though I've never been to Europe, the man I married (since divorced) was from Oklahoma and spent a couple of years at Ramstein Air Force Base in Germany. He did considerable traveling and camping throughout Europe. It was AFTER I began a serious relationship with him that my symptoms started to manifest.
That is only one factor of many things that all happened about the same time, though. Still, it's possible that he had been exposed to some European TBI strain that set my symptoms in motion. I hadn't thought of him as a possible factor.
(BTW, ex-hubby was a physical mess when I met him and had all kinds of musculoskeletal problems, mostly spinal - which is where my worst physical symptoms are. He'd been disabled for 2 years with back problems, had 2 cervical fusions, 5 knee surgeries plus later had both knees replaced, surgery on one wrist for compartment syndrome, and a couple of other surgeries that I can't recall.)
Also, I'm having peripheral neuropathy or something similar in my feet. I figure it was diabetes (no, don't have that yet) or circulation. Maybe another clue there.
So, I guess I can add that information to my list of things that make me go, ``Hmmmm''.
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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Hey there Tracy,
Your story just gets more interesting by the day!
About editing. Do you have PhotoShop loaded on your comp? That would make life a whole lot easier for you, but only if you know how to use it I suppose.
Most comps do have a built in editing program, you may have to search and experiment to find it though.
Another option would be to send the unedited pics to me and I can edit for you?
I'm SO behind already for the day and it isn't even 7am yet! So, I have to shut down windows so I can concentrate on work a bit. Just let me know IF you would like any help with the pics, OK? M
Posts: 7052 | From Colorado | Registered: Mar 2003
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Tracy, my husband has the exact same diagnosis and you describe his sores exactly. I asw similar loking pics in Schaller's bart book.
My husband has never been to CO, but has hiked quite a bit in the western states: alaska, montana mostly. I dont think he remembers a tick bite.. we know how that goes.
We are from the east coast.
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Truthfinder
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Thanks for the offer, Melanie. We'll see how it goes. No, I don't find Photoshop on here - just 2 other photo programs, one is a Microsoft product, the other is some Dell program.
R62, Bart, huh? Interestingly, I LIVED in interior Alaska for 10 years..... and it was there that the skin on my face started doing weird things.....
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Tracy, he doesnt have other bart symptoms, esp not neuro symptoms. His only symptoms are headaches and muscle pain in his thighs. And these sores that you described to a tee.
He has been to Alaska twice. Once in Denali with me and the other hiking the Brooks Range. He just told me that he had these before he went to Alaska...
???
He also has the fry mystery bug.
I have also wondered if they are gluten related.
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Truthfinder
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Well, sort of glad to know that Alaska probably didn't have anything to do with it.
Bart is certainly a possibility for me, though I don't know about the Fry bug or gluten.
-------------------- Tracy .... Prayers for the Lyme Community - every day at 6 p.m. Pacific Time and 9 p.m. Eastern Time � just take a few moments to say a prayer wherever you are�. Posts: 2966 | From Colorado | Registered: Dec 2005
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Melanie Reber
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The spirochetal etiology of acrodermatitis chronica atrophicans Herxheimer. [Journal Article] Asbrink E, Hovmark A, Hederstedt B Acta Derm Venereol 1984; 64(6):506-12.
Spirochetes were recovered from the skin lesion of 1 out of 10 acrodermatitis chronica atrophicans patients (ACA). Spirochetes from this skin isolate and from Ixodes (I.) ricinus and I. dammini spirochetes were used as antigens in indirect immunofluorescence tests. All sera from 17 ACA patients showed high antibody titers to the three antigens.
Seven of the 17 sera which had the highest titers had crossreactive antibodies to treponemal antigen detectable in the FTA-ABS test.
The results indicate that spirochetes are of importance for ACA and probably the causative agent of this disease. The connection between ACA and tick bites and the relationship to erythema chronicum migrans Afzelius (ECMA) and Lyme disease are discussed.
The results are consistent with the hypothesis that ECMA and ACA are different manifestations of the same spirochete, with ACA as a late manifestation.
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Melanie Reber
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Many times ACA, Scleroderma and Raynaud's Phenomenon go hand in hand. Here is an excellent overview of Scleroderma. I realize this is very long...but it contains some very valuable information. As with most overviews, please be cautious re: some suggestions, and talk everything over with your treating physician before any decisions are made re: your healthcare.
Symptoms * Because significant depression can affect more than 50% of people with scleroderma, researchers say it may be beneficial for scleroderma patients to get routine screening for depression.
Causes * Researchers have discovered a gene called connective-tissue growth factor (CTGF), which they say is more common in people with systemic scleroderma than in those without the disease.
Prognosis * The prognosis for patients with systemic scleroderma has improved since the 1970s. Ten-year survival rates are up, and deaths from kidney crises have dropped. However, deaths from pulmonary fibrosis have increased during this time period.
Treatment * High-dose immunosuppressant therapy with cyclophosphamide significantly improved skin and overall function in patients with scleroderma.
* Evidence shows that intravenous iloprost given in progressively increasing doses can reduce the duration and frequency of Raynaud's phenomenon attacks.
* A potential new therapy using PVAC, a substance derived from the bacterium, Mycobacterium vaccae, can improve skin symptoms without causing significant side effects.
Introduction The name scleroderma comes from the Greek words skleros, which means hard, and derma, which means skin. The disease is categorized as a rheumatologic disorder because it affects the connective tissues in the body.
Scleroderma is a rare disease marked by the following:
* Damage to the cells lining the walls of small arteries
* An abnormal buildup of tough scar-like tissue in the skin
Patients with scleroderma may develop either a localized or a systemic (body-wide) form of the disease.
Localized Scleroderma
Localized scleroderma usually affects only the skin on the hands and face. Its course is very slow, and it rarely, if ever, goes throughout the body (becomes systemic) or causes serious complications. There are two main forms of localized scleroderma: morphea and linear scleroderma.
Morphea Scleroderma. In morphea scleroderma, patches of hard skin form and can last for years. Eventually, however, they may improve or even disappear. There is less than a 1% chance that this disorder will progress to systemic scleroderma.
Linear Scleroderma. Linear scleroderma causes bands of hard skin across the face or on a single arm or leg. Linear scleroderma may also involve muscle or bone. Rarely, if this type of scleroderma affects children or young adults, it may interfere with growth and cause severe deformities in the arms and legs.
Systemic Scleroderma
Systemic scleroderma is also called systemic sclerosis. This form of the disease may affect the organs of the body, large areas of the skin, or both. This form of scleroderma has two main types: limited and diffuse scleroderma. Both forms are progressive, although most often the course of the disease in both types is slow.
Limited Scleroderma (also called CREST Syndrome). Limited scleroderma is a progressive disorder. It is classified as a systemic disease because its effects can be widespread throughout the body. It generally differs from diffuse scleroderma in the following ways:
* Most often the internal organs are not affected.
* Patients with scleroderma have a less serious course, unless they develop pulmonary hypertension (a particular danger with the CREST syndrome). Pulmonary hypertension is high blood pressure in the lungs (see the Lung Complications section).
Limited scleroderma is commonly referred to by the acronym CREST, whose letters are the first initials of characteristics that are usually found in this syndrome:
* Calcinosis. With this condition, mineral crystal deposits form under the skin, usually around the joints. Skin ulcers filled with a thick white substance may form over the deposits.
* Raynaud's phenomenon. In this syndrome, the fingers of both hands are very sensitive to cold, and they remain cold and blue-colored after exposure to low temperatures. This occurs in nearly all cases of scleroderma, both limited and diffuse. It is caused by abnormal changes in small blood vessels. These changes cause the vessels to narrow, and blood flow is temporarily interrupted, usually in the fingers.
* Esophageal motility dysfunction. The esophagus carries food from the mouth to the stomach. In esophageal motility dysfunction, the muscles in the esophagus become scarred by scleroderma and do not contract normally. This can cause severe heartburn and other symptoms of gastroesophageal reflux disorder (GERD).
* Sclerodactylia (also called acrosclerosis). This is the stiffness and tightening of the skin of the fingers, a classic symptom of scleroderma. Bone loss may occur in the fingers and toes.
* Telangiectasia. In this situation, widening of small blood vessels causes numerous flat red marks to form on the hands, face, and tongue.
In general, people with limited scleroderma develop Raynaud's phenomenon long before they develop any of the other symptoms. One or more of the CREST conditions can also occur in other forms of scleroderma.
Diffuse Scleroderma. Diffuse scleroderma, the other systemic sclerosis, has the following characteristics:
* It can affect wide areas of the skin, connective tissue, and other organs.
* It can have a very slow course, but it also may start quickly and be accompanied by swelling of the whole hand. If it gets worse quickly early on, the condition can affect internal organs and become very severe -- even life threatening.
* Diffuse scleroderma can overlap with other autoimmune diseases, including systemic lupus erythematosus and polymyositis. In such cases, the disorder is referred to as mixed connective disease.
Symptoms and Complications
Raynaud's Phenomenon
Raynaud's phenomenon is often the first sign of the scleroderma disease process. With this condition, small blood vessels narrow in the fingers, toes, ears, and even the nose.
Attacks of Raynaud's phenomenon can occur several times a day, and are often brought on or worsened by cold. Warmth relieves these attacks. In severe cases, attacks can develop regardless of the temperature. Severe cases may also cause open sores or damage to the skin and bones, if the circulation is cut off for too long. Stress also can trigger the syndrome.
Typically, the fingers go through three color changes:
* First, they become very pale.
* As the blood flow is cut off, they turn a bluish color, usually in the top two sections of the second and third fingers.
* Finally, when blood flow returns, the fingers become red.
Tingling and pain can occur in the affected regions.
Raynaud's is very common and occurs in 3 - 5% of the general population. It's important to note that more than 80% of patients with Raynaud's phenomenon do not have scleroderma, lupus, rheumatoid arthritis, or other more serious illnesses. Raynaud's is more likely to be a symptom of scleroderma or some other connective tissue disease if it develops after age 30, if it is severe, and if it is accompanied by other symptoms (such as skin changes and arthritis).
Skin Changes
Course of Typical Skin Changes. The primary symptoms of scleroderma occur in the skin. They often take the following course:
* Typically, pitted scars appear first on the hands. The skin begins to thicken and harden on the hands, feet, and face. The fingers may swell. This condition is called sclerodactylia or acrosclerosis. Patients with diffuse scleroderma may have swelling of the whole hand before the skin significantly thickens.
* Thickened or hardened patches may also develop on other areas of the body. (Their appearance on the trunk and near the elbows or knees tends to be a sign of a more severe condition.)
* For the first 2 or 3 years, the skin continues to thicken and feel puffy.
* This process then stops, and can even get better. The skin may soften.
* As the disease progresses further, however, the skin loses its ability to stretch, and becomes shiny as it tightens across the underlying bone, particularly in the fingers, toes, and around the mouth.
* Eventually, in severe cases, the fingers may lose the ability to move, and can be difficult to bend. The hands and feet may curl from the tightness of the skin. It may be difficult to open the mouth widely.
Other Skin Changes. The following skin symptoms may also occur:
* Flat red marks, known as telangiectasis, may appear in various locations, usually the face, palms, lips, or the inside of the mouth.
* In calcinosis, small white lumps form beneath the skin, sometimes oozing a white substance that looks like toothpaste. Calcinosis can lead to infections.
* Small blood vessels at the base of the fingernails may be lost in some places, and may widen in other places. This is an indication that internal organs might be involved.
* The entire surface of the skin may get darker over time, and contain patches of abnormally pale skin.
* Hair loss may occur.
* About 1% of patients have Sjogren syndrome, a group of symptoms that include dry eyes and dry mucus membranes (such as those in the mouth).
* Inside the mouth, scleroderma can also cause changes that impair gum healing.
Bone and Muscle Symptoms
Changes in bones, joints, and muscles can cause the following symptoms:
* Mild arthritis. The condition is usually distributed equally on both sides of the body.
* Bone loss in the fingers. The destruction is not as severe as it is in rheumatoid arthritis, although the fingers may shorten over time.
* Trouble bending the fingers, if the disease has affected the tendons and joints.
* Muscle weakness may occur, especially near the shoulder and hip.
Digestive Tract Symptoms and Complications
Complications in the Upper Digestive Tract.
* Esophageal motility disorder develops when scarring in the muscles of the esophagus causes them to lose the ability to contract normally, resulting in trouble swallowing, heartburn, and gastroesophageal reflux (also known as GERD). Some experts believe that patients with severe GERD may breathe in microscopic amounts of stomach acid, which in turn may be a major cause of lung scarring.
* About 80% of patients also experience impaired stomach activity. A delay in stomach emptying is very common.
* Some patients develop "watermelon stomach" (medically referred to as CAVE syndrome), in which the stomach develops red-streaked areas from widened blood vessels. This causes a slow bleeding that can lead to anemia (low red blood cell counts) over time.
* There may be a higher risk for stomach cancer.
* Problems with movement of the food through the intestines (motility) also develop. Patients may experience an increase in bacteria and have trouble absorbing nutrients from foods through the intestines.
Complications in the Lower Digestive Tract. Complications in the lower tract are uncommon. If they do occur, they can include the following:
* Scarring can cause blockages and constipation. In rare cases, constipation can become so severe that the bowel develops holes or tears, which can be life threatening.
* Scarring can also damage the absorption of fats in the intestines. This can lead to an increase in the number of bacteria, which causes watery diarrhea.
* Fecal incontinence (the inability to control bowel movements) may be more common than studies indicate, because patients are reluctant to report it.
Many patients, however, have few or even no lower gastrointestinal symptoms.
Lung Symptoms and Complications
In severe cases, the lungs may be affected, causing shortness of breath or difficulty in taking deep breaths. Shortness of breath may be a symptom of pulmonary hypertension, an uncommon but life-threatening complication of systemic scleroderma.
Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in scleroderma patients. Two major lung conditions associated with scleroderma, pulmonary fibrosis and pulmonary hypertension, can occur either together or independently.
Interstitial Pulmonary Fibrosis. Scleroderma involving the lung causes scarring (pulmonary fibrosis). Pulmonary fibrosis occurs in about 70% of scleroderma patients, although the progression is very slow and patients have a wide range of symptoms:
* Some patients may not experience any symptoms.
* When pulmonary fibrosis progresses, patients develop a dry cough, shortness of breath, and reduced ability to exercise.
* Severe pulmonary fibrosis occurs in about 16% of patients with diffuse scleroderma. About half of these patients experience the most profound changes within the first 3 years. In such cases, lung function worsens rapidly over that period, and then the progression slows down.
This condition also places the patient at higher risk for lung cancer. One study suggested that interstitial lung disease may be due to severe dysfunction in the esophagus, which causes patients to breathe in tiny amounts of stomach acid.
The most important indication of future worsening in the lungs appears to be inflammation in the small airways (alveolitis). Doctors detect alveolitis by using a lung test called bronchoalveolar lavage.
Pulmonary hypertension is the narrowing of the pulmonary arteries in the lung. The narrowing of the arteries creates resistance and increases the workload of the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is to control the symptoms, although the disease usually develops into congestive heart failure.
Pulmonary Hypertension. The primary symptom of pulmonary hypertension is shortness of breath, which becomes severe over time.
Pulmonary hypertension can develop in one of two ways:
* As a complication of pulmonary fibrosis
* As a direct outcome of the scleroderma process itself. In this case, it is most likely to develop in patients with limited scleroderma after many years.
Kidney Symptoms and Complications
Signs of kidney problems, such as increased levels of protein in the urine and mild high blood pressure (hypertension), are common in scleroderma. As with pulmonary hypertension, the degree of severity depends on whether the kidney problems are acute or chronic.
Slow Progression. The typical course of kidney involvement in scleroderma is a slow progression that may produce some damage but does not often lead to kidney failure.
Renal Crisis. The most serious kidney complication in scleroderma is renal crisis. It is a rare event that occurs in a small number of patients with diffuse scleroderma, most often early in the course of the disease. This syndrome includes a life-threatening condition called malignant hypertension, a sudden increase in blood pressure that can cause rapid kidney failure. This condition may be fatal. However, if the condition is successfully treated, it rarely recurs.
Until recently, renal crisis was the most common cause of death in scleroderma. Aggressive treatment with drugs that lower blood pressure, particularly those known as ACE inhibitors, is proving to be successful in reducing this risk.
Heart Symptoms and Complications
Many patients with even limited scleroderma have some sort of functional heart problem, although severe complications are uncommon and occur in only about 15% of patients with diffuse scleroderma. As with other serious organ complications, they are more likely to occur within 3 years after the disease begins.
Fibrosis of the Heart. The most direct effect of scleroderma on the heart is fibrosis (scarring). It may be very mild or it can cause pain, low blood pressure, or other complications. By damaging muscle tissue, the scarring increases the risk for heart rhythm problems, problems in electrical conduction, and heart failure. The membrane around the heart can become inflamed, causing a condition called pericarditis.
Pulmonary hypertension and hypertension associated with kidney problems in scleroderma can also affect the heart.
Other Symptoms and Complications
Other complications of scleroderma may include the following:
* Patients with CREST may be at increased risk for biliary cirrhosis, an inflammatory autoimmune disorder of the liver.
* Nerve damage may occur in the extremities (legs and feet, arms and fingers), causing numbness and pain. This damage can progressively worsen and lead to severe open sores (ulcerations), particularly in the hands. The feet are less often affected, but when they are, the disease tends to affect the joints and cause pain.
* Bone loss (osteoporosis) can occur because of impaired blood flow.
* About half of patients develop underactive thyroid gland (hypothyroidism).
* Impotence, usually due to scarring of the penis, may be one of the first complications of the disease in men.
* Some studies using imaging techniques have found changes in brain tissue, but because the brain has little connective tissue, scleroderma appears to have little effect on mental functioning, except possibly in the late stages of severe disease.
* Systemic scleroderma does not generally affect fertility in women. Pregnant women with scleroderma, however, have a slightly increased risk of premature birth and low-birth-weight babies. Although they can carry a baby to term, because complications such as kidney crisis can occur with the disease, pregnant women with scleroderma need to be monitored closely in a high-risk obstetric facility.
* More than half of scleroderma patients are likely to experience significant depression. Researchers say it may be beneficial for scleroderma patients to be routinely screened for depression.
Causes
Most likely this disease is caused by a number of inherited (genetic) abnormalities, which are triggered by environmental factors.
Researchers have found a gene, called connective-tissue growth factor (CTGF), which they say regulates the production of a protein that may be a key to systemic scleroderma. This gene is more common in scleroderma patients than in people without the condition. However, researchers say the gene is just one factor that affects the development of the disease.
Research published in 2005 also showed that the growth of new blood vessels is abnormal in people with scleroderma, particularly those whose disease affects the blood vessels in the lungs. Researchers now know that cells in the blood vessels and skin of scleroderma patients make too much of certain chemicals, and not enough of others. Studies revealed that the cause is an alteration in the hereditary material, DNA. These changes "turn off" some genes and "turn up" others. It is hoped that certain drugs, some of which are already used in cancer treatments, can some day be used to stop these DNA changes.
Inflammatory Response and Autoimmunity
The disease process leading to scleroderma appears to occur as an autoimmune response, in which an abnormal immune system attacks the body itself. In scleroderma, this response produces swelling (inflammation) and too much production of collagen. Collagen is the tough protein that helps build connective tissues such as tendons, bones, and ligaments. Collagen also helps scar tissue form. When normal tissue from skin, lungs, the esophagus, blood vessels, and other organs is replaced by this type of abnormal tissue, none of these body parts work as well, and many of the symptoms previously described occur.
Antigens are large molecules (usually proteins) on the surface of many cells -- both human cells, and cells of viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. When the immune system recognizes an antigen as being foreign (not part of the human body), it starts offensive and defensive actions against them by producing antibodies and other chemicals such as cytokines that destroy any cells in the area.
Much of this activity is directed by T cells, which are categorized as killer T cells or helper T cells (TH cells).
The actions of the helper T cells are of special interest in scleroderma. For some unknown reason, the T cells become overactive in scleroderma and mistake the body's own collagen as a foreign antigen. This triggers a series of immune responses to destroy the collagen. When the body creates antibodies against itself in this way, it is called an autoimmune response.
Cytokines and the Inflammatory Response. Helper T cells also release powerful immune factors called cytokines. In small amounts, cytokines are necessary for healing. If overproduced, however, they can cause serious damage, including inflammation and injury.
Neutrophils. Cytokines attract to the scene large numbers of other white blood cells known as neutrophils. Neutrophils help activate chemicals known as leukotrienes. Scleroderma patients have high levels of specific leukotrienes that may contribute specifically to lung disease in scleroderma.
Fetal Cell Theory and Microchimerism
A process called microchimerism has been proposed as a cause of scleroderma. The theory arose from the fact that scleroderma occurs mostly in women, and its symptoms resemble those of graft-versus-host disease (GVHD). GVHD occurs in bone marrow transplant patients who have received cells from another person. It happens when the transplanted donor immune cells launch an attack against the patient's cells.
Chimerism occurs when cells from two different individuals exist in the same body. When there is a low number of cells of one body in another, the condition is referred to as microchimerism.
However, if microchimerism plays a role, it most likely does so only in a subset of patients.
Triggering the Immune Response
It is still not clear why the immune system responds abnormally in people with scleroderma. Some experts believe that environmental factors, such as a virus or a chemical, may trigger the response in individuals with a genetic vulnerability.
Oxygen-Free Radicals and Abnormal Metal Accumulation. One focus for researchers investigating scleroderma involves an observation that, as blood vessels narrow and become inflamed, destructive particles known as oxygen-free radicals are produced. Oxygen-free radicals are made by natural processes in the body. They cause harm by setting off a chemical chain reaction, which can damage any type of cell in the body. Environmental toxins, infections, and other factors may cause very high amounts of these oxygen-free radicals to build up in the body.
Chemicals. Occupational exposure to certain chemicals can cause blood vessel constriction and attacks of Raynaud's phenomenon. Despite the fact that women are at higher overall risk for scleroderma, among people who are exposed to solvents at work, men face a higher risk for the disease. However, no specific work-related factors have been proven to cause the disorder.
It is nearly impossible to determine whether specific chemicals may actually cause systemic scleroderma, primarily because few people develop the disease, even though many people are exposed to such chemicals. In addition, research has been unable to consistently repeat studies that have reported links with chemicals.
Studies have found, however, that certain industrial toxins are significantly associated with severe lung problems in people with scleroderma. The toxins most likely to be associated with severe disease include epoxy resins, white spirit, solvents, and silica mixed with welding fumes.
Repetitive Stress Injuries. Raynaud's phenomenon and symptoms of scleroderma have been associated with jobs that require intense repetitive hand and arm movements, such as working jackhammers or other vibrating tools. However, many workers are involved in such occupations, yet scleroderma is still very rare, even in this group. If there is a link, the disease would most likely develop in individuals with genetic factors that make them susceptible to the disease in the first place.
Radiation. Radiation therapy has been reported to cause local patches of scleroderma (morphea) or worsen preexisting scleroderma in a few patients. In some cases, scleroderma may occur years after radiation treatments.
Infections
Researchers think that infections may play a role in triggering the process leading to some cases of scleroderma. There is no real evidence of any single type of bacteria or other organism that might be responsible, although some are of particular interest.
Some studies reported an association between Borrelia burgdorferi, the cause of Lyme disease, and some cases of morphea (localized scleroderma). However, the evidence is weak. If there is a connection, it is possibly limited to a specific type of the bacteria in Europe and Asia. There is no connection between systemic scleroderma and Lyme disease.
Other infections associated with scleroderma include parvovirus and hepatitis C. However, there is no evidence of a cause-and-effect relationship.
Risk Factors
Scleroderma is uncommon. It afflicts about 300,000 Americans, but only about 49,000 have the systemic form of the disease. The cause of scleroderma has not been determined, and there are few specific risk factors. The incidence tends to be higher in certain groups, however.
Age. Systemic scleroderma usually develops between the ages of 35 and 55. Localized scleroderma is more common in children than adults, but is extremely rare even in the young age group. It occurs in between 0.2 and 0.4 per 100,000 people. Systemic scleroderma in children is even more rare.
Gender. The incidence of scleroderma is three to eight times higher in women than in men. This may reflect a different cause of the disease in these two genders. (It should be noted that pregnancy itself is not a risk factor for scleroderma.)
Family History. A family history is the strongest risk factor for scleroderma, but even among family members, the risk is very low (less than 1%).
Genetics. Genetic factors appear to play a role in triggering the disease, but most cases are unlikely to be inherited. Preliminary research suggests that patients with certain gene variations may be more susceptible to scleroderma than those who do not carry these variations.
Ethnicity. Limited data on risk by ethnic group in the United States suggests that the risk from highest to lowest is the following: Choctaw Native Americans (highest), African-Americans, Hispanics, Caucasians, Japanese Americans. African-Americans have a higher rate of diffuse scleroderma, lung involvement, and a worse prognosis than Caucasians. Other studies also found lower survival rates among Japanese Americans.
Genetic factors affect population groups differently. Studies are finding that ethnic groups differ in the number of specific scleroderma-related antibodies they produce. Caucasians, for instance, have a higher rate of anti-centromere antibodies, which are associated with limited disease, while African-American patients have higher rates of autoantibodies and genetic factors that are associated with a more severe condition.
Geography. There appears to be certain geographic clusters of scleroderma, or specific types of scleroderma related to location. This may suggest an infectious or genetic factor at work, but the reasons are largely unknown. The following are some examples:
* Studies reported significantly higher-than-average scleroderma mortality rates in male patients (both African-American and Caucasian) who live in two specific regions of the Southeast: one cluster around Coffee, Tennessee, and two others near Northampton, North Carolina.
* A cluster of scleroderma cases has been observed in South Boston, Massachusetts.
Prognosis
At this time there is no cure for scleroderma and no treatment to change its course, but outlook varies widely. Many patients, even those with systemic scleroderma, can expect a normal lifespan.
General Outlook of Localized Scleroderma. Localized scleroderma nearly always carries a good prognosis and a normal life span. Even localized scleroderma, however, can cause some severe effects in children, including impaired growth, limb imbalance, and problems in flexing and bending muscles.
General Outlook of Systemic Scleroderma. The outlook for patients with systemic scleroderma has generally improved over the years. Ten-year survival rates rose from 54% in 1972 to 66% in 2001.
The causes of death related to systemic scleroderma also have changed. The proportion of deaths from kidney crises dropped significantly, from 42% to just 6% in that time period; however, the proportion of deaths from pulmonary fibrosis increased from 6% to 33%. Today, lung complications account for 60% of scleroderma-related deaths.
* Limited Scleroderma. Patients with limited CREST scleroderma can usually expect a favorable outlook and normal lifespan if the disease affects only the hands and face. The course of this type of scleroderma still tends to be slowly progressive and, in some cases, may affect internal organs.
* Diffuse Scleroderma. The severity of diffuse scleroderma varies widely, and it is very difficult to predict its course. It generally follows one of two paths: If it is acute or rapidly progressing, it may be a life-threatening condition that affects internal organs. The most critical period for rapid progression is usually within the first 2 - 5 years of the start of the disease. In the absence of rapid progression, or if the patient survives the initial acute progression, the disease tends to progress very slowly. The more severe the condition of the skin is at the start of the disease, the poorer the survival rates.
Many patients with systemic scleroderma experience a plateau in which the condition stabilizes. This plateau is followed by a period of improvement and skin softening. No one knows why this occurs, and it can happen regardless of treatment. In one study, patients with systemic scleroderma who experienced such improvements also had better survival rates (80% at 10 years) than those whose skin did not improve (60% 10-year survival rate).
Impact on Quality of Life
The many complications of scleroderma can have a major impact on a person's sense of well-being. Patients are greatly concerned about changes in their appearance, particularly those changes caused by tightening of the facial skin. A 2002 study on scleroderma patients reported that 63% experienced at least mild pain, and half of them had some degree of depression. Depression had the greatest impact, even more than pain, in reducing patients' ability to function socially.
Diagnosis
There are no specific tests for scleroderma. The doctor may suspect scleroderma after taking a history of the symptoms and performing a physical examination. As part of this examination, the doctor does the following:
* Checks the skin for thickened and hardened areas. The major signs of scleroderma are hardening and thickening of the skin in any areas on the fingers and toes.
* Presses affected tendons and joints to detect crackling or grating sensations, which can indicate changes related to scleroderma beneath the skin.
* Examines the fingernails underneath a microscope. The doctor may find changes in capillaries that are characteristic of scleroderma and mixed connective tissue disease.
Scientists recently found that antibodies that are often found in patients with scleroderma and systemic lupus erythematosus (SLE) bind to different parts of a single protein. Scientists hope this finding will one day lead to a specific diagnostic test for scleroderma.
Tests for Antinuclear Antibodies
Tests may be done to detect immune factors called antinuclear antibodies (ANAs). Detecting specific types of ANAs may help diagnose scleroderma. ANA subtypes include the following:
* Rheumatoid factor, anti-single-stranded DNA, and antihistone antibodies are autoantibodies associated with scleroderma, but they are also common in other autoimmune disorders, such as rheumatoid arthritis and systemic lupus erythematosus. Some ANAs attack RNA or DNA, the genetic material in cells.
* Anti-RNA polymerase III, anti-topoisomerase I (also called anti-DNA topo 1) and anti-centromere antibodies (ACA) are three other autoantibodies. Most patients with systemic scleroderma (but not localized scleroderma) have one or more of these autoantibodies. They do not appear at the same time, and seem to relate to different phases of the disease process. For example, anti-DNA topo 1 often occurs with diffuse skin scleroderma and lung complications. Anti-centromere antibodies usually occur with a less severe form of the disease.
* Higher-than-normal levels of autoantibodies to fibrillin 1, a protein found in muscle and other connective tissues, is more common in patients with both systemic and localized scleroderma. This autoantibody in localized scleroderma is more common in some ethnic groups (such as Japanese and Native Americans) than in others (Caucasians). It is not found in other autoimmune diseases.
These antibodies are also found in other rheumatologic disorders, so detecting them does not necessarily prove that a patient has scleroderma. At the same time, studies have found that specific antibodies are associated with specific aspects of the disease. Therefore, identifying their presence could help diagnose, treat, and monitor people with scleroderma. Here are a few examples:
* Anti-U1-RNP and anti U3-RNP are associated with muscle inflammation.
* ACA is commonly associated with pulmonary hypertension and vascular disease.
* TOPO is associated with pulmonary fibrosis.
* RNA Polymerase III (Pol 3) is rarely linked to severe interstitial fibrosis, although this autoantibody is strongly present in patients with kidney crisis.
* Patients with diffuse scleroderma who have Pol 3 have the best survival rate.
Diagnosing Systemic Complications
Diagnosing Lung Complications. Changes in the lungs may occur early in scleroderma lung disease, and prompt treatment is very important to prevent complications. For this reason, once a diagnosis is made, the doctor will check for lung changes in the following ways:
* Listen to the lungs through a stethoscope. Rales, a crackling sound at the base of the lungs as the patient breathes in, is a sign of pulmonary fibrosis, even if breath function is normal.
* Perform respiratory function tests to determine lung capacity.
* Take a chest x-ray (however, x-rays do not always find lung disease, especially in children).
* Have patients inhale nitric oxide to test the ability of blood vessels to open.
* Perform more extensive tests, such as high-resolution computed tomography (CT) scans and bronchoalveolar lavage, if the doctor suspects severe lung scarring.
Newer tests showing promise in diagnosing lung complications include:
* The induced sputum test, which looks at cells taken from coughed-up phlegm
* Another test that uses the inhaled chemical, technetium-labeled diethylenetriamine pentaacetate (99mTC-DTPA), to detect lung damage.
Diagnosing Heart Complications. Patients with suspected heart complications should have the following tests:
* Electrocardiography (ECG): A test of the heart's electrical activity
* Echocardiography: A look at the beating heart through the use of sound waves
* Radionucleotide ventriculography: An evaluation of the working heart using a radioactive dye
Advanced imaging techniques, which provide a more detailed picture of the heart, may also be useful to determine the extent of heart complications in scleroderma patients.
Diagnosing Pulmonary Hypertension. Echocardiography is a noninvasive imaging technique for detecting pulmonary hypertension, a common and life-threatening complication of scleroderma. (Neither materials nor equipment are put into the body.) To confirm the diagnosis, doctors sometimes use an invasive procedure called right-heart catheterization. Right-heart catheterization involves the passage of a catheter (a thin flexible tube) into the right side of the heart to get diagnostic information about the heart.
Diagnosing Gastrointestinal (Digestive) Complications. Endoscopy may detect gastrointestinal problems. Endoscopy is an invasive procedure in which a tube is inserted down the esophagus. The tube contains a small camera and other instruments. Another diagnostic test is manometry, which measures the pressure that the muscles in the esophagus apply.
Electrogastrography (EGG) measures the electrical activity in muscles in the stomach, and may be an effective method for detecting stomach problems.
Diagnosing problems in growth of blood vessels. Capillaroscopy is the microscopic examination of blood vessels under the skin. It is now considered a useful tool for identifying problems with the growth of blood vessels, because more than 95% of patients will have some capillary abnormalities. Such problems can show the severity and progression of scleroderma. In a technique called nailfold capillaroscopy, the doctor places a drop of oil on the nailfolds (the skin at the base of the fingernails), and then looks at the nailfold under a microscope for signs of changes in the capillaries that may indicate a connective tissue disease such as scleroderma.
Ruling out Other Conditions
Other Autoimmune and Connective Tissue Disorders. Several other autoimmune conditions that affect connective tissue can strongly resemble, or occur together with, scleroderma. They include the following:
* Rheumatoid arthritis
* Systemic lupus erythematosus
* Polymyositis
Symptoms of such diseases may also include fever, arthritis, muscle aches, rash, and lung and heart problems.
Eosinophilic Fasciitis. Eosinophilic fasciitis is a muscle disorder that is known to occur after intense hard work. It can cause symptoms similar to scleroderma, including pain, swelling, and tenderness in the hands and feet, as well as skin thickening. The disorder can be ruled out with blood tests.
Although Raynaud's phenomenon occurs in most scleroderma patients, over 80% of the cases of Raynaud's phenomenon are harmless. In one study, only 12% of Raynaud's cases were associated with some other condition, and few of those were scleroderma. The following are other problems that might accompany or cause Raynaud's phenomenon:
* Other autoimmune connective tissue diseases
* Diabetes (patients with diabetes may develop Raynaud's phenomenon and other scleroderma-like symptoms)
* Certain drugs, including bleomycin, ergot derivatives (used for migraines), and methysergide
* Hereditary hemorrhagic telangiectasia (a very rare condition that is very similar to CREST syndrome)
* Repetitive stress injuries (particularly from vibrating tools)
* Hypothyroidism
Treatment
Scleroderma treatments vary depending on these variables:
* Is it local or systemic, and if systemic, is it limited or diffuse?
* If the disease is systemic, what organs, if any, are involved?
Although there is still no treatment for the underlying process of scleroderma, specific drugs and treatments help combat the various mechanisms and consequences of the disease.
* Some medications keep blood vessels open (prostacylins, endothelin receptor antagonists, ACE inhibitors, phosphodiesterase 5 inhibitors, and others) and are used to treat Raynaud's phenomenon, heart and kidney problems, and pulmonary hypertension.
* Other drugs reduce inflammation and block damaging immune factors. These treatments, which include cyclophosphamide, penicillamine, bone marrow transplantation, and others may be helpful for improving skin thickness and reducing scarring, even in the lungs.
* Doctors use other treatments for specific complications, such as proton pump inhibitors and pro-kinetic agents for gastrointestinal problems, or light treatments for skin thickening.
* Various investigative approaches exist, including stem-cell transplants.
Patients should receive treatments for specific complications as early as possible in the course of the disease, to reduce progression before irreversible hardening of tissues occurs.
There is no cure for scleroderma. Many drugs that are useful for other autoimmune inflammatory disorders have not proven to be very effective for scleroderma. Experimental work is ongoing to develop procedures or to find drugs that can treat the underlying processes that cause damage. Developing effective treatments for scleroderma is very problematic, however, for the following reasons:
* The course of scleroderma is hard to predict, making it one of the most difficult rheumatic diseases to treat. It also makes drug development complicated.
* The disease, when advanced, affects many organs. Designing treatment strategies that will improve symptoms in some organs without affecting other organs is very difficult.
* The disease is so uncommon that there are few patients available for clinical trials. Studies, then, are very small, sometimes having only four or five patients. It is very difficult to design studies of this size that can provide strong evidence on treatment effects. Drugs that seem promising on small groups of patients often fail to show effectiveness on larger groups.
Treating the Whole Patient
The disease can evolve slowly over time with few symptoms, or progress rapidly and become very severe. The patient, then, must live with considerable uncertainty and emotional stress. Support associations, non-medical aids to help relieve symptoms, and other lifestyle measures can be extremely important and helpful.
Medications
Calcium-channel blockers are the standard drugs to open the blood vessels, and may be used for pulmonary artery hypertension and Raynaud's phenomenon. Short- or sustained-release nifedipine (Adalat, Procardia) is the gold standard.
Other drugs used include diltiazem (Cardizem, Dilacor), and the newer dihydropyridines (felodipine, amlodipine, and isradipine). Side effects vary among different medications, and may include fluid buildup in the feet, constipation, fatigue, gingivitis, impotence, flushing, and allergic symptoms. Calcium channel blockers should not be taken with grapefruit juice, as it appears to boost the effects of these drugs. [The medications listed below are also discussed under many of the sections covering treatment complications.
Nitrates
Nitrates relax smooth muscles and open arteries, and are therefore sometimes used for the short-term management of Raynaud's phenomenon. They are available in topical and oral (by mouth) forms. Side effects of nitrates include headaches, dizziness, nausea, blurred vision, fast heartbeat, and sweating. Lying down with the legs elevated can relieve low blood pressure and dizziness.
Alcohol, beta blockers, calcium-channel blockers, and certain antidepressants can significantly worsen these side effects. Withdrawal from nitrates should be gradual. Some severe reactions have occurred when people have stopped taking these drugs too quickly.
Prostacyclins (also called Prostaglandins)
Prostacyclins open blood vessels and also have anti-blood-clotting properties. One or all of these drugs is used to treat pulmonary artery hypertension and Raynaud's phenomenon. Several prostacyclins are being used for scleroderma, although none have been approved specifically for the condition. Promising prostacyclins or similar drugs include iloprost (Ventavis), alprostadil (prostaglandin E1), epoprostenol (Flolan), and treprostinil (Remodulin).
Endothelin Receptor Antagonists
Bosentan (Tracleer) is a drug taken by mouth. It is called an endothelin receptor antagonist. It controls endothelin, a powerful molecule that causes blood vessels to narrow. It improves blood flow and is becoming important for treating patients with scleroderma, especially for preventing finger ulcers and improving hand function. This drug is also a treatment option for pulmonary hypertension.
ACE Inhibitors and Angiotensin II Receptors
The most effective approach at this time for preventing kidney (renal) crises is to start aggressive blood pressure-lowering treatment before blood tests show kidney damage has occurred.
Angiotensin Converting Enzyme (ACE) Inhibitors. Many medications are available for controlling blood pressure, but ACE inhibitors appear to be the most effective for scleroderma patients, because of their protective actions in the kidney. These drugs are also used to treat patients with evidence of kidney damage, whether or not they have high blood pressure.
ACE inhibitors include captopril (Capoten), enalapril (Vasotec), quinapril (Accupril), benazepril, and lisinopril (Prinivil, Zestril). Side effects are uncommon, but may include an irritating cough, large drops in blood pressure, and allergic reactions. The drug picotamide can help reduce the frequency of coughs.
Angiotensin II Receptor Antagonists. Angiotensin II receptor antagonists (losartan, candesartan cilexetil, and valsartan) have benefits similar to ACE inhibitors and may have fewer or less severe side effects, including coughing. They may also have positive effects on blood vessels.
Small studies showing improvement in Raynaud's phenomenon warrant further research.
Treatments that Affect the Immune System
One major approach to scleroderma is to use treatments that suppress the immune system, and therefore reduce the activity of the harmful processes that lead to scleroderma. Such treatments are used effectively in other autoimmune diseases. Their use in scleroderma varies, depending on the location and severity of the disease process.
Cyclophosphamide (Cytoxan). Cyclophosphamide is the most important immunosuppressant currently used for scleroderma. This drug can be taken through a vein (intravenous) or by mouth. It blocks some of the destructive actions of scleroderma in the lungs. Intravenous cyclophosphamide can be life-saving for patients with pneumonia caused by interstitial lung disease. Side effects of this drug include hair loss, infection, and bleeding into the urinary tract. To date, no other immunosuppressive drugs have shown any significant benefits for scleroderma.
Other drugs used to suppress the immune system may be useful in specific cases. They include D-penicillamine (which may be useful for skin symptoms), methotrexate (Rheumatrex), corticosteroids, cyclosporine (Sandimmune, Neoral), and chlorambucil (Leukeran). All of these drugs have potentially severe side effects.
Other Treatments
Tumor-Necrosis Factor Modifiers. Tumor-necrosis factor (TNF) modifiers are major breakthroughs in the treatment of rheumatoid arthritis. They interfere with specific parts of TNF, a powerful immune factor. Researchers believe they should be tested in other inflammatory conditions, including scleroderma. The current agents include infliximab (Remicade), etanercept (Enbrel), alefacept (Amevive), and adalimumab (Humira).
Blood Exchange (Plasmapheresis or photopheresis). Plasmapheresis is a process in which the liquid part of the blood, called plasma, is separated from blood cells. The procedure involves first withdrawing blood from the patient. The plasma, which contains the active immune factors, is discarded and replaced with other fluids. The blood is then returned to the patient.
Autologous Stem-Cell Transplantation. Researchers are investigating a possible benefit of transplanting the patient's own stem cells (an autologous transplant). (Patients with autoimmune diseases cannot be given cells from donors.) The transplant procedures introduce normal white blood cells that replace the abnormal autoimmune cells. The procedure has improved or stabilized systemic scleroderma in some patients, with remissions lasting up to 5 years or more, and improvements in skin and overall function.
Initial results of ASTIS, a major study evaluating stem-cell transplants and high-dose immunosuppressive therapy in severe scleroderma, indicate that this combination has led to sustained remission in more than one-third of patients. Randomized controlled trials comparing stem cell transplants to monthly cyclophosphamide therapy are underway in Europe and the U.S.
Although the risk of death from having a transplant is now less than 10%, the procedure has serious side effects. Experts suggest that the best candidates are those at high risk for complications from scleroderma. In general, such patients would have diffuse scleroderma, experienced their first symptoms within the previous three years, and have evidence of at least mild abnormalities in the heart, lungs, or kidney. In general, patients with advanced scleroderma would not be the best candidates, because the risks of the procedure would outweigh the risks from the disease.
Extracorporeal Photopheresis: Another phototherapy treatment under investigation, extracorporeal photopheresis, involves withdrawing the patient's blood and treating it with ultraviolet light. Little data exists on its effectiveness. One study found that the therapy improved skin and joint symptoms, but the authors say it's possible that a placebo effect was at least partly responsible for the results. Experts do not recommend photopheresis at this time, but some feel that it does hold promise and warrants more research.
Intravenous immunoglobulin (IVIg). Animal studies have found that administration of IVIg, an agent that modifies the immune system, may reduce the severity of scleroderma and other autoimmune diseases. So far, only extremely small studies including fewer than 10 patients have been conducted, but the treatment is showing promise for relieving joint pain and tenderness and improving function. The exact role of this therapy in scleroderma treatment, if any, has yet to be determined.
Herbs and Supplements
Because of the difficulty in treating scleroderma, many patients are tempted to try high-dose supplements or other alternative treatments. Some natural treatments have been evaluated for the treatment of scleroderma, including para-aminobenzoic acid, vitamin E, evening primrose oil, and an avocado/soybean extract. However, these treatments have not been proven effective, and using alternative remedies can be dangerous.
There is almost no published research on the use of herbal remedies for patients with scleroderma. Generally, manufacturers of herbal remedies and dietary supplements do not need FDA approval to sell their products. Just like a drug, herbs and supplements can affect the body's chemistry, and therefore have the potential to produce side effects that may be harmful. There have been numerous reported cases of serious and even deadly side effects from herbal products. Always check with your doctor before using any herbal remedies or dietary supplements.
Treatment for Raynaud's Phenomenon
The following are some lifestyle tips for managing Raynaud's phenomenon:
* Keeping warm is the primary goal for preventing the onset of Raynaud's phenomenon. Air-conditioning and exposure to refrigeration can trigger this syndrome. If patients go out in cold weather, they should dress warmly with many layers. Wearing a hat is essential.
* Living in a warm climate may help relieve symptoms, although a recent study found that weather changes themselves had little effect on the disorder.
* Exercise is helpful for maintaining a sense of well-being, keeping warm, and sustaining skin flexibility. Patients with Raynaud's phenomenon may want to avoid exercising outdoors in cold weather, however.
* Quitting smoking is, of course, essential for anyone, but it is critical for people with scleroderma.
* Learning relaxation and anti-stress techniques might help reduce some triggers of Raynaud's phenomenon.
* Using moisturizers and antibiotic ointments may be helpful for keeping skin flexible and preventing infections in the fingers.
* Avoiding medications such as nonselective beta blockers (such as propranolol), certain common cold preparations, and narcotics, can help avoid aggravating Raynaud's phenomenon.
Medications Used in the Treatment of Raynaud's Phenomenon
Vasodilators. Vasodilators open blood vessels and so are important for Raynaud's phenomenon.
Calcium-channel blockers, including diltiazem (Cardizem, Dilacor) and nifedipine (Adalat, Procardia) are the standard vasodilating drugs used for Raynaud's phenomenon. Nifedipine is the best studied of these drugs, but there are also newer dihydropyridines, including felodipine, amlodipine, and isradipine.
Nitrates, available in topical or oral forms, are vasodilators that are also used for Raynaud's phenomenon, and for short-term relief.
Prostacylins. Iloprost and other prostacylins are proving to be effective agents for Raynaud's phenomenon. Small but well done studies seem to show these drugs to be helpful for this condition, and possibly as effective as calcium channel blocker drugs such as nifedipine.
Evidence shows that intravenous iloprost given at progressively increasing doses over 3-month cycles can reduce the duration and frequency of attacks. In general, these drugs are used when a patient's symptoms are severe, particularly when the doctor is considering amputating a finger.
Endothelin receptor agonists have also been shown to help with Raynaud's phenomenon.
Anti-Platelet Drugs. Aspirin, dipyridamole, and other drugs that prevent blood clotting and keep blood flowing freely are sometimes recommended to patients with Raynaud's phenomenon. However, these drugs haven't shown much benefit in studies.
Estrogen Therapy in Women. Short-term treatment with estrogen may benefit older women with Raynaud's phenomenon and scleroderma. It is important to note, however, that hormone replacement therapy for more than 5 years can increase a woman's risk for breast cancer, heart attacks, strokes, and blood clots.
PDE5 Inhibitors. Studies have suggested that a class of drugs called PDE5 inhibitors, which includes sildenafil, helps improve symptoms and blood flow, and speeds ulcer healing in patients with Raynaud's phenomenon. This treatment is still experimental.
Surgical Treatments for Problems of the Hands
Sympathectomy and Hand Surgeries. Sympathectomy uses procedures that block or remove the nerve responsible for narrowing blood vessels in the hand. The result is increased blood flow in the hand.
The local anesthetics lidocaine or bupivacaine may be very effective in temporarily restoring blood flow and reducing pain.
For finger ulcers that won't heal and are resistant to standard treatments, sympathectomy surgery may be done.
Treatment for Skin Thickening
Nitroglycerin is a quick acting nitrate and is used as an ointment (Nitro-Bid, Nitrol, Nitrong, Nitrostat) to treat hardened skin. Before applying it, remove any ointment that remains from the previous application.
Phototherapy
UVA-1 Phototherapy. Phototherapy (light therapy) is now considered by some experts to be the treatment of choice for local scleroderma. Specifically, doctors favor an approach called ultraviolet A-1 (UVA-1) radiation. This treatment produces long UVA wave lengths that do not cause sunburn and may actually repair DNA in damaged skin cells. Research suggests that UVA-1 therapy blocks inflammatory immune factors and the process leading to over-production of collagen, addressing the underlying mechanisms of scleroderma.
The procedure is effective for all stages of morphea. It increases skin elasticity and in some cases, completely clears up symptoms. In one small study, patients with localized scleroderma received 30 phototherapy treatments over a period of 12 weeks. In the majority of patients, 80% of the skin patches disappeared or significantly improved. There were no side effects.
UVA-1 phototherapy is quite expensive and requires a special light source not available everywhere. In addition, studies are reporting an increased risk with UVA radiation. Whether this applies to UVA-1 phototherapy is not yet clear. Nonetheless, phototherapy is still an effective and important treatment of scleroderma. It may prove to be even more beneficial when combined with certain medications, such as calcipotriene (Dovonex), a form of vitamin D3.
PUVA. An alternative phototherapy regimen called PUVA uses drugs called psoralens taken by mouth before UVA treatment. PUVA has been used for other skin diseases, including psoriasis. It may prove useful for patients with early-onset diffuse scleroderma. In one study, most patients treated with PUVA 2 days a month for up to 8 years experienced improvement or stabilization in nearly all scleroderma symptoms. Tests for kidney function remained normal. This treatment is known to increase the risk for skin cancer.
Phototherapy with Psoralen Water Bath. Yet another procedure uses UVA light therapy after patients take a bath containing a solution of psoralen 8-methoxypsoralen (8-MOP). This treatment is safe and well tolerated, although benefits appear to be minor and occur only in a small subset of patients.
Vitamin D3 Analogs
A form of vitamin D3, calcipotriene (Dovonex), appears to help block skin cell production. This vitamin is called calcipotriol in Europe. It also has anti-inflammatory properties, and is being investigated as a rub-on treatment and oral treatment for local scleroderma. It may prove beneficial when combined with low-dose ultraviolet A1 phototherapy.
Immunosuppressive Agents
D-penicillamine is proving to be an effective agent for softening skin and reducing thickness. (Improvements in thickness with this drug have also been associated with improved survival.) Methotrexate (Rheumatrex) is another commonly used drug, and may be even more effective than penicillamine.
Corticosteroids taken by mouth, such as prednisolone and prednisone, are also often used.
Treatment for Other Complications
Pilocarpine (Salagen) has been approved for treating dry mouth in people with scleroderma and Sj�gren syndrome. In one study, patients with Sj�gren syndrome experienced increased salivation after the first dose. Patients reported improvement in speaking, sleeping, and swallowing food without drinking. Side effects of this drug include sweating, increased need to urinate, chills, and flushing.
Surgical Treatments for Problems of the Hands
Other Surgeries. Disabling deformity of the hand is a common feature of scleroderma. Various surgical procedures can relieve pain, prevent tissue loss, protect hand function, and improve the appearance of the hands.
Treatment for Lung Complications
Pulmonary Fibrosis
Cyclophosphamide. Cyclophosphamide (Cytoxan), an immunosuppressive drug, may be effective for preventing lung deterioration and is the important medication for treating pulmonary fibrosis, particularly when given early in the course of the disease.
Use of this drug may improve survival in patients who show early signs of lung deterioration, notably inflammation in the small lung airways (alveolitis). The drug is not recommended for patents with existing stable pulmonary fibrosis and no signs of inflammation. In one study, patients with early signs of lung inflammation were given a course of intravenous pulses of the corticosteroid methylprednisolone (MP) and cyclophosphamide. Nearly all patients experienced improvement or stabilization during the first year, although the disease had progressed in two-thirds of them after 2 years.
Other Treatments. Lung transplantation may offer hope for people with advanced pulmonary hypertension or interstitial fibrosis that does not respond to conservative treatments.
Pulmonary Hypertension
Several types of drugs are used to treat pulmonary hypertension. Anticoagulants taken by mouth, such as warfarin (Coumadin), are a standard treatment used to prevent blood clots from forming. Diuretic treatment and supplemental oxygen are recommended for patients with fluid retention and low blood oxygen, respectively.
Vasodilators help open blood vessels and relieve pressure in arteries of the lungs. Vasodilators used to treat pulmonary hypertension fall into several different drug classes:
Calcium Channel Blockers (CCBs). Some patients with pulmonary hypertension benefit from these drugs. They help relax blood vessels in the heart and lungs, and increase the supply of oxygen. However, calcium channel blockers are only appropriate for patients who meet certain diagnostic criteria, including those who don't have right-sided heart failure. Long-acting nifedipine or diltiazem, or amlodipine, are the preferred calcium channel blockers.
Prostacyclins (Prostaglandins). Prostacyclins, which open blood vessels, are now the primary agents for treating pulmonary hypertension.
* Iloprost (Ventavis) is available in inhaled and intravenous forms. Studies suggest that the inhaled form improves exercise capacity and survival in some patients with pulmonary hypertension. In addition, infusions of iloprost remain effective over long periods (up to 3 years) of use.
* Treprostinil (Remodulin) is similar to epoprostenol but is more stable. It can also be administered using a portable pump that delivers the drug under the skin. This is less expensive, cumbersome, and invasive than the delivery methods for epoprostenol.
* Epoprostenol (Flolan), which is administered intravenously, has improved exercise capacity and symptoms in both the short and long term in a number of patients. In some patients, survival is increased significantly. However, not all patients respond to this drug. The implanted catheter needed to deliver the drug can also cause serious complications.
Endothelin Receptor Antagonists. Bosentan (Tracleer) was the first drug taken by mouth that was approved for pulmonary hypertension. Bosentan controls endothelin, a powerful substance that causes blood vessels to narrow. Studies have reported improved exercise capacity in patients with pulmonary hypertension. Sitaxsentan and ambrisentan (Letairis) are two new drugs being studied.
PDE5 Inhibitors. Sildenafil (Revatio) was approved in 2005 as the first pill for patients with early-stage pulmonary hypertension.
Sildenafil is the same medication contained in the erectile dysfunction drug Viagra. However, Revatio is prescribed at a lower dosage than Viagra, and is a different color and shape than Viagra pills.
Other Treatments. Lung transplantation may offer hope for people with advanced pulmonary hypertension that does not respond to conservative measures.
Treatment for Gastrointestinal Problems
Treatments for abnormalities in the esophagus and stomach are generally the same as those for gastroesophageal reflux (GERD) or heartburn. Many non-prescription agents are available for the relief of heartburn.
Proton-pump or acid-pump inhibitors are probably the best drug treatments for reflux symptoms related to scleroderma. They work by inhibiting the so-called gastric acid pump that is required for the cells of the stomach to release acid. The standard drug has been omeprazole (Prilosec).
Newer drugs -- including lansoprazole (Prevacid), pantoprazole (Protonix), esomeprazole (Nexium), and rabeprazole (Aciphex) -- are more potent, but few comparison studies have been done on them.
Side Effects. Side effects are uncommon, but can include allergic reaction, headache, stomach pain, diarrhea, and flatulence. Of some concern was a report of a very severe and widespread skin rash caused by omeprazole in a woman with scleroderma. It should be noted that this is only one incident, but patients should be cautious about any skin change when taking this medication.
Agents for Impaired Stomach Muscle Contractions
Metoclopramide. Metoclopramide (Reglan) is sometimes used for patients who have delayed stomach emptying.
Octreotide. Octreotide (Sandostatin) is related to a natural hormone that suppresses growth hormone, and may prove to be very helpful for scleroderma patients. Small studies have reported that this drug improved weight and nutrition. It may even help other symptoms of scleroderma.
Agents for Constipation
Prokinetics. Prokinetics improve the muscle action of the esophagus and enhance stomach emptying. Prucalopride is an investigative pro-kinetic agent that significantly improved symptoms and relieved constipation. Similar medications, such as cisapride (Propulsid), are showing promise; however these types of drugs can have serious side effects.
Treatments for Malabsorption
Antibiotics may be effective for the malabsorption syndrome associated with an increase in bacteria. Octeotride may also be used for this problem.
Surgeries
Strictures (abnormally narrowed regions in the esophagus) may need to be opened with surgery.
Melanie Reber
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"Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases. Prinz JC, Kutasi Z, Weisenseel P, P�t� L, Batty�ni Z, Ruzicka T. J Am Acad Dermatol. 2009 Feb;60(2):248-55.
BACKGROUND: Morphea is an inflammatory autoimmune skin sclerosis of unknown etiology. A causative role of Borrelia burgdorferi infection has been controversially discussed, but no conclusive solution has yet been achieved.
OBJECTIVE: Intrigued by 3 young patients with severe Borrelia-associated morphea and high-titer antinuclear antibodies, we retrospectively examined the relationship between Borrelia exposure, serologic autoimmune phenomena and age at disease onset in morphea patients.
METHODS: In 90 morphea patients the presence of Borrelia-specific serum antibodies was correlated to the age at disease onset and the presence and titers of antinuclear antibodies. Patients with active Borrelia infection or high-titer antinuclear antibodies due to systemic sclerosis or lupus erythematosus served as controls.
RESULTS: We observed a statistically highly significant association between morphea, serologic evidence of Borrelia infection, and high-titer antinuclear antibodies when disease onset was in childhood or adolescence.
LIMITATIONS: Because pathogenic Borrelia species may vary in different geographic regions the relevance of Borrelia infection in morphea induction may show regional variations.
CONCLUSION: B burgdorferi infection may be relevant for the induction of a distinct autoimmune type of scleroderma; it may be called "Borrelia-associated early onset morphea" and is characterized by the combination of disease onset at younger age, infection with B burgdorferi, and evident autoimmune phenomena as reflected by high-titer antinuclear antibodies. As exemplified by the case reports, it may take a particularly severe course and require treatment of both infection and skin inflammation.
Melanie Reber
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Borrelial Fasciitis: Diffuse Fasciitis and Peripheral Eosinophilia Associated with Borrelia Infection Granter, Scott R. M.D.; Barnhill, Raymond L. M.D.; Duray, Paul H. M.D. The American Journal of Dermatopathology: October 1996 - Volume 18 - Issue 5 - pp 465-473
Abstract
We present four cases of diffuse fasciitis (DF) associated with peripheral eosinophilia in which spirochetal organisms were identified. Two patients had borderline positive results on serologic evaluation for Borrelia burgdorferi.
Deep biopsy showed dermal sclerosis associated with variable degrees of perivascular mononuclear inflammation. Diffuse fasciitis, septal panniculitis, and myositis with mononuclear cell infiltrates and varying numbers of eosinophils were observed. All cases showed a striking lymphocytic vasculopathy associated with atypical reactive endothelial cells. Using modified Dieterle and Steiner silver stains, multiple organisms were seen in one specimen, a single unequivocal organism detected in two specimens.
In one case, no organisms were detected on silver stain; however, organisms were demonstrated using rabbit polyclonal antibodies against B. burgdorferi. B. burgdorferi-specific DNA was identified in one patient by the polymerase chain reaction.
These results indicate that some cases of eosinophilic fasciitis are an expression of Lyme disease. We have previously proposed the more specific term borrelial fasciitis to describe such lesions.
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Melanie Reber
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Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases. De Vito JR, Merogi AJ, Vo T, Boh EE, Fung HK, Freeman SM, Cockerell C, Stewart K, Marrogi AJ. Department of Dermatology, Talane University School of Medicine, New Orleans, Louisiana 70112, USA. J Cutan Pathol. 1996 Aug;23(4):350-8.
Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases.
The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues.
The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings.
These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.
PMID: 8864923 [PubMed - indexed for MEDLINE]
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Melanie Reber
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Chronic borreliosis presenting with morphea- and lichen sclerosus et atrophicus-like cutaneous lesions. a case report. Kaya G, Berset M, Prins C, Chavaz P, Saurat JH. Department of Dermatology, DHURDV, University Hospital of Geneva, Switzerland. [email protected] Dermatology. 2001;202(4):373-5.
We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans.
Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.
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Melanie Reber
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Bacterial Infection as the Cause of Scleroderma: A Guide to Antibiotic Therapy
CantwellAR Jr, Ganger P
Abstract Scleroderma is a disease characterized by thickening and hardening of the skin. While most traditional and established treatments provide temporary improvement at best, remission is rare -- and cure impossible. Furthermore, the various medications commonly prescribed have serious side effects. There is urgent need for better treatment.
Scleroderma is erroneously considered an incurable disease of unknown cause. This communication provides evidence implicating infectious bacteria as the cause of scleroderma; and proposes antibiotic therapy as a rational and beneficial treatment for this disease.
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Melanie Reber
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from 14th International Scientific Conference On Lyme Disease And Other Tick-Borne Disorders 2001
Clinical Aspects of Lyme Disease: Dermatologic, Cardiac, GI, and Gestational CME Disclosures
Harry Goldhagen, MS Julie Rawlings, MPH
Morphea and Lyme Disease: Are They Related? Morphea is a rare and untreatable dermatologic condition characterized by thickening and induration of the skin from excess collagen deposition. There are at least 5 forms of the disease: localized, generalized, guttate, linear, and coup de sabre (an indentation that can extend to and damage the underlying muscle and bone). The cause is generally not known, but as with any idiopathic condition, proposed etiologies abound, including radiation damage, autoimmunity, infection, vaccination, trauma, and genetic predisposition. One of the leading infectious disease candidates in the pathogenesis of morphea is B burgdorferi, although this association is a subject of controversy.[1] A number of European studies have found a correlation, while most US studies, including a frequently cited study from the Mayo Clinic,[2] have found no evidence of B burgdorferi in morphea lesions.
Andrew G. Franks, Jr, MD,[3] of New York University School of Medicine (NYU), believes there is a connection between the 2 diseases. For instance, it can be difficult to differentiate between EM and certain morphea lesions, especially if the EM lesion is not the typical bull's-eye with central clearing. The differential diagnosis for EM-like lesions is varied and can include spider bites, herpes simplex or zoster, cellulitis, fungus or tinea, granuloma annulare, drug eruption, erythema multiforme, and subacute lupus erythematosus. In many cases, it can be difficult to culture B burgdorferi from EM lesions.
Part of the disagreement between the US and other studies may be due to differences in B burgdorferi serotypes and strains that cause morphea, according to Dr. Franks. For instance, only the sensu stricto strain is found in the United States, but all 3 pathogenic strains are found in Europe. Two skin diseases -- acrodermatitis (similar in appearance to scleroderma) and lymphocytoma -- have been identified in Europe as linked to B burgdorferi; the former is associated with the B afzelii strain, and the latter with the B garinii strain.
Dr. Franks believes that serologic testing is unreliable in morphea. He said that in published US studies of B burgdorferi antibodies in morphea patients, the investigators did not check whether the subjects received antibiotics recently. Antibiotic treatment can alter the results of serologic testing. Polymerase chain reaction (PCR) testing for B burgdorferi DNA in morphea patients may also be inaccurate in the US. For instance, the PCR tests may have been too specific and missed strains or serotypes more likely to cause morphea-like skin disease. European studies have detected B burgdorferi DNA by PCR in patients with morphea. For instance, a PCR study by Fujiwara and colleagues[4] tested for afzelii and garinii strains and found evidence of B burgdorferi in non-US cases. US PCR amplification has generally only tested for the sensu stricto strain.
Dr. Franks and colleagues at NYU have recently completed an unpublished study of the association of B burgdorferi, autoimmunity, and morphea. In this study, 82 patients (average age of 27 years; range, 2-61 years) with new-onset morphea were enrolled; 14 were male. The investigators tested for antibody to autoimmune disease and to B burgdorferi by IgG and IgM Western blot (WB), with all bands reported. They used their own criteria for diagnosis of underlying Lyme disease: more than 1 IgG band on WB was taken as a positive result for Lyme. None of the subjects had clinically defined Lyme disease during the trial. For autoimmune serology, any positive test was defined as positive.
For treatment purposes, they classified the patients into 4 groups, depending on whether they were positive by WB and/or autoimmune serology. Generally, patients with positive WB received 6 weeks of doxycycline (or an alternative antibiotic), and those with negative WB were given 3 weeks of doxycycline, with an optional additional 3 weeks if improvement was seen during the first 3 weeks.
Of the 61 patients who completed the study, 34 had clinical improvement. The WB-positive, autoimmune serology-negative group was the most likely to respond to antibiotic therapy. The Lyme WB converted to negative in many treated patients.
Dr. Franks and colleagues believe that all patients with morphea are candidates for a therapeutic antibiotic trial, regardless of serologic test results. IgM and IgG WB may be helpful in the diagnosis, but current Lyme tests are unreliable in such patients. Because the risks are minimal with antibiotics, he believes it is worth trying this approach, especially since there is no effective treatment for morphea at the present time.
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Melanie Reber
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Special Thank Yous to...
Lonestartick, Trueblue, Tree-hugger, and 5dana8 for providing the links to some of these latest posts!
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seekhelp
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Melanie, are you a researcher? I've never seen one post so many articles!!! Posts: 7545 | From The 5th Dimension - The Twilight Zone | Registered: Mar 2008
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