Schnitzler syndrome is a rare disorder characterized by chronic urticarial rashes and a monoclonal IgM gammopathy, variably combined with intermittent fever, arthralgia or arthritis, lymphadenopathy, hepatomegaly and/or splenomegaly, leukocytosis, and an elevated erythrocyte sedimentation rate (1).
Patients have an increased risk for Waldenstrom macroglobulinemia and amyloidosis.
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