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.


  1. An interesting case. I am comfortable with the diagnosis of morphea. The family history is not worrisome. You can check serogies, but will likely not be helping. On this continent, I do not think there is need to check for Borrelia. Potent topical steroids, alternating with calcipotriene should help. Don't forget about physical therapy. Daavlin offers a small UVA1 unit which could be worthwhile. Consider methotrexate.

  2. from Khalifa Sharquie, Baghdad, Iraq: "Currently I doing research on morphea and will be published soon.It is increasing over the last 10 years and all patients present with early pigmented inflammatory patches that last for months or years before changing into sclerosed white atrophied areas.This is why easily misdiagnosed at this early stage.Encoupe de sabre is commonly seen which is highly progressive and damaging if not treated.Also linear morphea especially among children is highly destructive if not diagnosed and managed in early activity of the disease .The common regime of therapy is using combination of strong topical steroid in addition to oral prednisone in combination with oral zinc sulfate.Also oral isotretinoin could be added especially in severe cases with sclerosis .Methotrexate is good alternative with other therapy but usually refused as it is cytotoxic .

  3. from Dr Cheng Leng Ong, Malaysia:
    1. A long time ago I had a young girl with morphea of less irregular margin, I remember her Antinuclear antibody was weakly positive. But in our patient under consideration, Lyme studies will be of upmost importance.
    2. A prolonged course of doxycycline is likely to benefit her, provided she is not pregnant. I have successfully treated with this regime, two women of prolonged migratory erythema/ urticaria, most probably due to Lyme disease.
    3. I cannot think of another diagnosis.


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