MORPHEA - SCLERODERMA - LYME DISEASE- BORRELIA

I was recently asked if Morphea could be a symptom of Lyme Disease.

A Google search found some interesting results which I post below. I draw attention to Eisendles findings. He uses Focus-floating microscopy and finds this more reliable than PCR. I heard Eisendle present at the Lyme Disease Action 2008 conference, very impressive.

I have to wonder how many patients are PCR tested negative for Borrelia when indeed they actually still have an active infection?

so in no order but all very interesting:-

http://www.thedoctorsdoctor.com/diseases/scleroderma_morphea.htm#ddx
Scleroderma-Morphea

Background
Scleroderma means hard skin. It exists in two clinical variants. The localized form is commonly referred to as morphea. The systemic form is usually referred to as systemic scleroderma. Morphea is a disease of skin the underlying connective tissue. Several variants have been described, as discussed below. Systemic scleroderma may affect multiple organ systems and is usually classified under autoimmune diseases. Occasionally, morphea and scleroderma may occur in the same patient. In these cases, morphea arises first followed by a milder and nonprogressive form of systemic scleroderma.

LYME DISEASE/BORRELIA INFECTION

J Am Acad Dermatol. 2009 Feb;60(2):248-55. Abstract quote
"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, Pótó L, Battyáni Z, Ruzicka T.
Department of Dermatology, University of Munich, Munich, Germany.
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.

https://canlyme.com/?s=Morphea&lang=enhttps://canlyme.com/?s=Morphea&lang=en



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.

http://www.nature.com/jid/journal/v100/n5/abs/5611650a.html

Further Evidence for Borrelia burgdorferi Infection in Morphea and Lichen Sclerosus et Atrophicus Confirmed by DNA Amplification
Christoph Schempp1, Hubertus Bocklage2, Robert Lange2, Hans W Kölmel2, Constantin E Orfanos1 and Harald Gollnick1
Abstract
We present further evidence in support of the notion that Borrelia burgdorferi is possibly involved in the pathogenesis of morphea and lichen sclerosus et atrophicus (LSA). Running a nested polymerase chain reaction (PCR) with a primer set specific for the flagellin gene of B. burgdorferi enabled us to demonstrate the presence of Borrelia DNA in skin biopsies of patients with morphea (nine of nine) of LSA (six of six). Biopsy specimens obtained from patients with erythema chronicum migrans (two patients, four of four samples) and acrodermatitis chronica atrophicans (one patient, one of one sample) also showed positive PCR results. By contrast, there was no amplification of Borrelia DNA in control biopsies either from patients with chronic eczema (three of three) or psoriasis (two of two) or from normal skin (three of three). Antibodies directed against B. burgdorferi were only detected in the serum of patients with erythema chronicum migrans (two of two) and acrodermatitis chronica atrophicans (one of one) but were not present in cases of morphea (five of five), LSA (three of three), or in control subjects (three of three). These data suggest that B. burgdorferi may play a role in the pathogenesis of both morphea and LSA. Furthermore, we conclude that PCR analysis provides an important diagnostic tool, even in seronegative Borrelia infections.
http://www.ncbi.nlm.nih.gov/pubmed/17941947

Morphoea: a manifestation of infection with Borrelia species?
Eisendle K, Grabner T, Zelger B.
Department of Dermatology and Venereology, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck, Austria. klaus.eisendle@uki.ac.at
Abstract
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.
PMID: 17941947 [PubMed - indexed for MEDLINE]




http://www.ncbi.nlm.nih.gov/pubmed/15649200

Eur Acad Dermatol Venereol. 2005 Jan;19(1):93-6.
Acute exacerbation of systemic scleroderma in Borrelia burgdorferi infection.
Wackernagel A, Bergmann AR, Aberer E.
Department of Dermatology, Medical University, Graz, Auenbruggerplatz 8, A-8036 Graz, Austria.
Abstract
In recent years a possible aetiological connection between skin sclerosis and an infection with Borrelia burgdorferi has been discussed, but this association has not yet been reported for systemic scleroderma. Several treatment modalities are suggested for systemic scleroderma, but no treatment has yet been found to alter the overall course of the disease. This report describes a 61-year-old woman with Raynaud's phenomenon, nail-fold changes and circulating anticentromere antibodies, who showed an abrupt onset of erythemas and doughy swellings involving the face and upper trunk, followed by thickening and induration of the skin mimicking diffuse systemic scleroderma. Laboratory tests including enzyme-linked immunosorbent assay (ELISA), immunoblot and urine polymerase chain reaction (PCR) showed an infection with B. burgdorferi sensu lato that was successfully treated with intravenous ceftriaxone, an antibiotic recommended for Lyme borreliosis. Fourteen days after the end of treatment the skin was no longer stiff and indurated and had returned to its normal predisease state. This case suggests that Lyme disease should be considered in atypical cases of skin sclerosis in patients predisposed to the development of systemic scleroderma.
PMID: 15649200 [PubMed - indexed for MEDLINE]