Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondentation, and a monoclonal IgM dysproteinemia, but without criteria of lymphoproliferative disease. We report a patient with chronic urticaria and macroglobulinemia. In addition, he had double monoconal dysproteinemia IgM kappa (31.3 g/l) and IgA lambda, osteocondensation, and some cutaneous lesions of pseudoxanthoma elasticum. Only 20 cases of Schnitzler's syndrome have been reported hitherto. This is the first case associated with pseudoxanthoma elasticum, which was localized and discovered at the same time as Schnitzler's syndrome. We discuss the possible role of monoclonal immunoglobulin in the occurrence of localized elastorrexhis.
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PMID:Schnitzler's syndrome (urticaria and macroglobulinemia) associated with pseudoxanthoma elasticum. 135 Jan 35

The authors report the case of a 65-year-old man who had an evolving case of Schnitzler's syndrome with lytic bone lesions which was unresponsive to several drugs, and which dramatically improved with small quantities of corticosteroids. Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondensation, and monoclonal IgM dysproteinemia without features of lymphoproliferative disease, and is associated with fever, deterioration of the general health and biological signs of inflammation. Only 22 cases of Schnitzler's syndrome have been reported hitherto and there is no known effective treatment, H1 and H2 blockers, dapsone, colchicine, chloroquine, cyclophosphamide, chlorambucil and azathioprine having proven useless in the few patients treated. Corticosteroids may be effective, however, especially against the urticaria.
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PMID:Schnitzler's syndrome (urticaria and macroglobulinemia) dramatically improved with corticosteroids. 777 12