Schnitzler Syndrome

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A 48-year-old man with typical features of Schnitzler Syndrome is presented, followed by a discussion of the pathogenesis and clinical aspects of this rare disease. Some of the challenges we and others have faced in diagnosing and treating this disease are also discussed. Clinical pearls and pitfalls are emphasized to aid clinicians from varying specialties in recognizing this syndrome and providing appropriate therapy. Schnitzler Syndrome is associated with high morbidity and increased risk of lymphoproliferative disorders. Accurate diagnosis is vital in restoring quality of life and ensuring appropriate long-term monitoring.

Keywords: Anakinra; Schnitzler syndrome; canakinumab; interleukin-1; monoclonal gammopathy; neutrophilic urticarial dermatosis; rituximab; urticaria

Document Type: Research Article


Affiliations: 1School of Medicine, University of Kansas Medical Center, Kansas City, Kansas

Publication date: January 1, 2014

More about this publication?
  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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