Scleromyxedema: a complete response to prednisone.
Scleromyxedema is a disorder characterized by a typical rash due to the accumulation of mucin in the dermis. It is always associated with a monoclonal protein in the serum and can have a wide variety of systemic manifestations. We describe a 40-year-old woman who had scleromyxedema associated with a monoclonal G lambda protein. Severe systemic symptoms included fatigue, esophageal dysmotility, and myopathy. Symptoms resolved completely with oral prednisone therapy, and she remained in clinical remission 24 months after use of prednisone was discontinued. Scleromyxedema is commonly treated with alkylating agents, which have been associated with pronounced morbidity and mortality. We suggest that oral corticosteroid therapy may be a reasonable initial choice for treating this disease and that alkylating agents be reserved for corticosteroid-refractory disease.
D000328:Adult; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D017520:Mucinoses / Q000097:blood / Q000188:drug therapy / Q000473:pathology; D009230:Myxedema / Q000097:blood / Q000188:drug therapy* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use*; D012867:Skin / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome