Monday, October 16, 2017

Linear Scleroderma in a 40 year-old Woman

Presented by Hamish McDougall
Cape Breton, Nova Scotia

The patient is a 37 year old woman with a four year history of a slowly progressively asymptomatic area of induration on the posterior aspect of the left thigh (photo).  A biopsy showed thickened collagen bundles in the reticular dermis and a sparse superficial and deep lymphoplasmacytic infiltrate consistent with morphea.

Strangely, her father-in-law is seriously ill with systemic sclerosis.  He lives far from the patient and her husband.  At this time, we have discovered no common exposures the patient and her father-in-law have.  The patient lives in an endemic area for Lyme Disease and serological testing will be offered.

Is there any value in obtaining serologies, other than Lyme studies, on this woman?
What treatment might be of value? 
Do you have alternate diagnoses?

1. Localized Scleroderma Review Article (Like a chapter in a text book)
2. Morphea Sculpted in Silica: A Case Report of Limited Cutaneous Systemic Sclerosis in a Woman with Long-Time Exposure to Silica Dust.

Pedro Gomes J, Shoenfeld Y.  Free Full Text.
3.  "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, et. al. J Am Acad Dermatol. 2009 Feb;60(2):248-55. 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.