Scleromyxedema: Successful treatment with IVIg and lenalidomide

Abstract

Scleromyxedema is a rare progressive disorder associated with severe morbidity due to altered coarse facial features. The disease etiology is unknown. Various therapies have been investigated like the use of corticosteroids, IVIg (intravenous immunoglobulin), plasmapheresis, and thalidomide. The disorder being rare, evidence from controlled therapeutic trials is very limited. This report describes the case of a 37-year-old male with progressive scleromyxedema, without gammopathy. The patient was treated with prednisolone followed by IVIg. He showed remarkable improvement initially. Subsequent treatment with lenalidomide for one year showed complete resolution of the skin lesions with no side effects of the therapy.