A case of urticarial vasculitis associated with macroglobulinemia (Schnitzler's syndrome).
We report a case of urticarial vasculitis associated with macroglobulinemia in a 50-year-old woman. She suffered from intermittent fever with wheals persisting for more than 24 hours. Skin histology revealed perivascular neutrophilic infiltrates with nuclear dusts. Laboratory study demonstrated leucocytosis and a high serum IgM level (2350 mg/dl) with an M component. No overt lymphoid proliferative disease was found. As far as we know, this is the first report of such a case in Japan. Predonizolone and nonsteroidal anti-inflammatory drugs have proved transiently effective for controlling the urticaria, but not for the IgM gammopathy.
Morita
A
A
Department of Dermatology, Nagoya City University Medical School, Japan.
Sakakibara
S
S
Yokota
M
M
Tsuji
T
T
D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000473:pathology*; D007075:Immunoglobulin M / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D011239:Prednisolone / Q000627:therapeutic use; D013577:Syndrome; D014581:Urticaria / Q000473:pathology* / Q000517:prevention & control; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000473:pathology* / Q000517:prevention & control; D008258:Waldenstrom Macroglobulinemia / Q000473:pathology*
D000894:Anti-Inflammatory Agents, Non-Steroidal; D007075:Immunoglobulin M; D007145:Immunoglobulin kappa-Chains; D011239:Prednisolone