Schnitzler's syndrome

Last reviewed 11/2022

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.

Reference:

• 1) Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E. The Schnitzler's syndrome: four new cases and review of the literature. Medicine 2001;80: 37-44.
• 2) de Koning HD, Bodar EJ, van der Meer JWM, Simon A. Schnitzler's Syndrome Study Group. Schnitzler's syndrome: beyond the case reports: review and followup of 94 patients with an emphasis on prognosis and treatment. Semin Arthritis Rheum 2007;37:137-48