Schnitzler's syndrome: lessons from 281 cases
Open Access
- 1 January 2014
- journal article
- review article
- Published by Wiley in Clinical and Translational Allergy
- Vol. 4 (1), 1-15
- https://doi.org/10.1186/2045-7022-4-41
Abstract
Schnitzler’s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.Keywords
This publication has 146 references indexed in Scilit:
- Identification of 15 new psoriasis susceptibility loci highlights the role of innate immunityNature Genetics, 2012
- Schnitzler's Disease as an Important Differential Diagnosis of Chronic Recurrent Multifocal Osteomyelitis: A Case ReportCase Reports in Rheumatology, 2012
- High incidence of NLRP3 somatic mosaicism in patients with chronic infantile neurologic, cutaneous, articular syndrome: Results of an international multicenter collaborative studyArthritis & Rheumatism, 2011
- Induction of a Chronic Disease State in Patients With Smoldering or Indolent Multiple Myeloma by Targeting Interleukin 1β-Induced Interleukin 6 Production and the Myeloma Proliferative ComponentMayo Clinic Proceedings, 2009
- A rare cause of a common symptom, Anakinra is effective in the urticaria of Schnitzler Syndrome: a case reportCases Journal, 2008
- Coexistent MEFV and CIAS1 mutations manifesting as familial Mediterranean fever plus deafnessAnnals Of The Rheumatic Diseases, 2007
- Prompt response of refractory Schnitzler syndrome to treatment with anakinraJournal of the American Academy of Dermatology, 2007
- Refractory auto-inflammatory syndrome associated with digenic transmission of low-penetrance tumour necrosis factor receptor-associated periodic syndrome and cryopyrin-associated periodic syndrome mutationsAnnals Of The Rheumatic Diseases, 2006
- Schnitzler's syndrome associated with sensorimotor neuropathyJournal of the American Academy of Dermatology, 1994
- Urticarial dermatosis associated with Waldenstrom's macroglobulinemiaJournal of the American Academy of Dermatology, 1989