Orphanet: Schnitzler syndrome

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Schnitzler syndrome

Disease definition

Schnitzler syndrome is a rare, underdiagnosed disorder in adults characterized by recurrent febrile rash, bone and/or joint pain, enlarged lymph nodes, fatigue, a monoclonal IgM component, leukocytosis and systemic inflammatory response.


Classification level: Disorder
  • Synonym(s):
    • Chronic urticaria with gammopathy
    • Chronic urticaria with macroglobulinemia
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: L50.8
  • OMIM: -
  • UMLS: C0524988
  • MeSH: D019873
  • GARD: 12390
  • MedDRA: 10062908

Detailed information

Article for general public


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