Successful Treatment of Scleromyxedema with IVIG: A Case Report

Scleromyxedema (SM), a rare progressive cutaneous mucinosis with a systemic involvement, presents with progressive dermal mucin depositions, causing skin thickening. In addition to the skin involvement and paraproteinemia, patients with SM also have other complications. The systems which are commonly involved include the gastrointestinal, musculoskeletal, pulmonary, cardiovascular, renal, and central nervous systems. Scleromyxedema is difficult to treat. We present a patient with severe Scleromyxedema with cutaneous, gastrointestinal, musculoskeletal and renal manifestations. Following failed therapeutic attempts, she responded to treatment with intravenous gammaglobulin (IVIG), prednisolone, and azathioprine. As a result of treatment with IVIG, the patient showed dramatic improvement in her clinical symptoms and remained in remission throughout the course of combination IVIG infusions and prednisolone. We concluded that an IVIG-prednisolone combination may be an effective novel treatment of Scleromyxedema.