[Arndt-Gottron scleromyxedema and associated phenomena].
Scleromyxedema is an uncommon fibromucinous connective tissue disease characterized by accumulation of mucinous material in the dermis. A monoclonal paraprotein is regularly identified. A review of the literature (57 cases) shows the exceptional association of scleromyxedema with multiple myeloma (8.7%) and macroglobulinemia Waldenström (3.5%). A man with scleromyxedema, IgG lambda paraproteinemia, and sclerodactylia--as a special sign of scleromyxedema--is reported. Melphalan is the drug of choice in serious cases, but not effective in sclerodactylia.
D005385:Fingers; D006801:Humans; D006942:Hypergammaglobulinemia / Q000473:pathology; D007074:Immunoglobulin G; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008854:Microscopy, Electron; D008875:Middle Aged; D009101:Multiple Myeloma / Q000473:pathology; D010265:Paraproteinemias / Q000188:drug therapy / Q000473:pathology*; D012594:Scleroderma, Localized / Q000473:pathology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000188:drug therapy / Q000473:pathology*; D013577:Syndrome; D008258:Waldenstrom Macroglobulinemia / Q000473:pathology
D007074:Immunoglobulin G; D008558:Melphalan
Skleromyxödem Arndt-Gottron und assoziierte Phänomene.