Engarde!

By Peter Galvin, MD

I recently came across an odd case in the “New England Journal of Medicine” that I decided to share with you. It involves an unusual presentation of the disease scleroderma. The term scleroderma comes from the Greek words skleros meaning “hard” and derma meaning “skin.” Scleroderma is an autoimmune disease that can involve the skin, blood vessels, muscles, and internal organs. Most commonly, scleroderma presents as the CREST syndrome. CREST includes Calcium deposits, Raynaud’s disease, Esophageal problems, Skin thickening, and Telangiectasias (small areas of dilated blood vessels). Risk factors for scleroderma include a family history of it (there is a genetic component) and exposure to environmental factors like silica (sand), solvents, ketones, trichloroethylene, welding fumes, and white spirits (such as turpentine and paint thinner). There is no cure for scleroderma, and the treatment for it, like other autoimmune diseases, involves suppression of the immune system.

Now the case I am referring to came from St. James Hospital in Dublin, Ireland. If, like me, you’ve been to Dublin, then you know that another facility exists in St. James Gate, namely the Guinness brewery. The tap room in the brewery is located on the 7th floor and is the highest spot in Dublin, and with its large windows it offers panoramic views of Dublin, not to mention some tasty, fresh Guinness. Anyway, the woman in the photo presented to the dermatology clinic at the hospital. She had a history of hypothyroidism and was 70 years old. She described a one-year history of progressive indentation and darkening of the skin of her forehead. The skin changes occurred along a wrinkle that had been present for 10 years. As you can see on panel A in the photo, there was a linear depression with red borders on the right side of her forehead. The depression extended up into the hairline and there was alopecia (hair loss), as seen in panel B.

A skin biopsy revealed the skin was infiltrated with white blood cells and plasma cells along with inflammation and scarring – findings consistent with mixed early- and late-stage scleroderma. A diagnosis of en coup de sabre – a linear subtype of localized scleroderma – was made. Localized scleroderma, also known as morphea, is an inflammatory, sclerotic skin disease with no known cause. It is distinct from systemic scleroderma. En coup de sabre derives its name from the characteristic scar that resembles a blow from a sword. While it can progress to involve the orbits and brain, this case did not do so. An MRI of the brain and eyes was normal. After an eight-month course of abatacept (Orencia, an immune-modulating drug) there was a reduction in the size and activity of the lesion. Slainte!

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