Chronic urticaria and macroglobulinemia (Schnitzler's syndrome): Report of two cases - 12/10/17
Abstract |
Two cases of chronic urticaria associated with macroglobulinemia are reported, and the characteristics of 13 other cases are reviewed. This entity was described by Schnitzler in 1974 and has the following characteristics: chronic nonpruritic urticaria with leukocytoclastic vasculitis, bone pains with hyperostosis, intermittent fever, and a monoclonal IgM gammopathy. Liver, lymph node, and spleen enlargement may occur. Criteria for the diagnosis of Waldenström's disease are lacking (IgM level less than 10 gm/L, no overt lymphoid proliferation in bone marrow). Other immunologic findings (complement, C1 inhibitor, cryoglobulin, rheumatoid factor, antinucleaT antibodies) are negative or normal. Evolution is long-term with a long follow-up period. In one case a lymphoplasmocytic lymphoma developed. No adequate treatment has yet been found. Pathogenesis is unclear but seems to be caused by skin deposits of the IgM paraprotein, as attested to by the direct cutaneous immunofiuorescent findings in some cases.
Le texte complet de cet article est disponible en PDF.† | Presented at the 45th Annual Meeting of the American Academy of Dermatology, New Orleans, La., Dec. 6–11, 1986. |
Vol 20 - N° 2P1
P. 206-211 - février 1989 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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