Intravenous immunoglobulin therapy for scleromyxedema: a case report and review of literature.

Scleromyxedema is a chronic, idiopathic disorder characterized by cutaneous dermal mucin deposition in association with increased dermal collagen and absence of thyroid disease. The clinical features involve sclerosis of skin with numerous, 2-3 mm, firm, waxy, papules in a symmetrical distribution pattern. The skin lesions may progress to widespread and indurated plaques resulting in patient disability via decreased mobility, sclerodactyly and microstomia. Abnormalities in the muscular, neurologic, rheumatologic, pulmonary, renal and cardiovascular systems may accompany the cutaneous lesions. Multiple attempts at therapeutic interventions have failed due to inconsistent, unsatisfactory results and/or severe adverse effects. Herein, the authors describe a case of scleromyxedema successfully treated with high-dose intravenous immunoglobulin (hdIVIG).