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Media type:
E-Article
Title:
The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome?
Contributor:
Eiling, Elisabeth;
Schröder, Johann O.;
Gross, Wolfgang L.;
Kreiselmaier, Inga;
Mrowietz, Ulrich;
Schwarz, Thomas
Published:
Wiley, 2008
Published in:
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 6 (2008) 8, Seite 626-631
Language:
English
DOI:
10.1111/j.1610-0387.2008.06627.x
ISSN:
1610-0387;
1610-0379
Origination:
Footnote:
Description:
SummarySchnitzler 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.