Scleromyxedema

Scleromyxedema (also known as lichen myxedematosus or papular mucinosis) is a rare cutaneous disorder characterized by lichenoid waxy papules, sclerosis, and a characteristic paraproteinemia. Rarely, if ever, is there systemic involvement. The cause and pathogenesis remain a mystery, however, a recent report has linked a serum factor with fibroblast proliferation in the skin. Treatment is often ineffective, although systemic melphalan may lead to improvement in 50 to 60 percent of patients. Morbidity and mortality due to melphalan used in the treatment of scleromyxedema have been reported, therefore, this therapy should be reserved for only the most extreme cases, if at all.