The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome?
Article first published online: 26 MAR 2008
© The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin
JDDG: Journal der Deutschen Dermatologischen Gesellschaft
Volume 6, Issue 8, pages 626–631, August 2008
How to Cite
Eiling, E., Schröder, J. O., Gross, W. L., Kreiselmaier, I., Mrowietz, U. and Schwarz, T. (2008), The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome?. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 6: 626–631. doi: 10.1111/j.1610-0387.2008.06627.x
- Issue published online: 29 JUL 2008
- Article first published online: 26 MAR 2008
- Submitted: 26.9.2007 | Accepted: 30.10.2007
- Schnitzler syndrome;
- chronic urticaria;
- monoclonal gammopathy;
- IL-1 receptor antagonist
Schnitzler syndrome describes the simultaneous occurrence of monoclonal gammopathy and chronic urticaria with at least two additional minor symptoms (arthralgia, bone pain, fever of uncertain origin, hepato- or splenomegaly, lymphadenopathy, increased erythrocyte sedimentation rate, leukocytosis/thrombocytosis or increased bone density). Schnitzler syndrome is not wellknown and very likely under-recognized. Comprehensive diagnostic examinations are necessary to rule out other diseases, especially those of hematologic origin. Close interdisciplinary collaboration is mandatory. The etiology of Schnitzler syndrome is unclear, but the rapid response to the interleukin-1 receptor inhibitor anakinra underlines the pivotal role which the proinflammatory cytokine interleukin-1 may play in the pathophysiology of this potentially autoinflammatory disorder.