The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy – an autoinflammatory syndrome?

Elisabeth Eiling, Johann O. Schröder, Wolfgang L. Gross, Inga Kreiselmaier, Ulrich Mrowietz, Thomas Schwarz*

*Corresponding author for this work
30 Citations (Scopus)


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.

Translated title of the contributionDas Schnitzler-syndrom: Chronische urtikaria und monoklonale gammopathie - Ein autoinflammatorisches syndrom?
Original languageEnglish
JournalJDDG - Journal of the German Society of Dermatology
Issue number8
Pages (from-to)626-631
Number of pages6
Publication statusPublished - 08.2008

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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