Scleromyxoedema: treatment of cutaneous and systemic manifestations with high-dose intravenous immunoglobulin.
Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-lambda paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.
Kulczycki
A
A
Division of Allergy and Immunology, Department of Medicine, Washington School of Medicine. St Louis, MO, U.S.A. akulczyc@wustl.edu
Nelson
M
M
Eisen
A
A
Heffernan
M
M
D000368:Aged; D004334:Drug Administration Schedule; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D018459:Lichen Sclerosus et Atrophicus / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009230:Myxedema / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy*; D012598:Sclerosis; D012867:Skin / Q000473:pathology
D016756:Immunoglobulins, Intravenous