Successful treatment of juvenile en coup de sabre with combination of systemic glucocorticoid and methotrexate

Abstract

A 13-year-old girl presented an indurated linear erythema on the mid-forehead with a 3-month history. Based on the clinical and histological findings, she was diagnosed as having scleroderma en coup de sabre. Since no efficacy of treatment with a glucocorticoid ointment and ultraviolet therapy had been observed, systemic glucocorticoid and methotrexate were administered. Her erythema disappeared after 4 weeks, and she was able to stop taking any medication 2 years later without any symptoms. No adverse events were observed. To date, there have been few case reports of scleroderma en coup de sabre treated with systemic glucocorticoids and methotrexate in Japan. This combination therapy is efficacious and comparatively safe. Thus, it should be considered as one of the treatment options for scleroderma en coup de sabre.