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Table 1 Clinical features of Schnitzler’s syndrome

From: Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease

Major Criteria

 • urticarial rash

 • monoclonal IgM gammopathy (IgG less common)

Minor Criteria

 • recurrent fever

 • arthralgia or arthritis

 • bone pain

 • lymphadenopathy

 • hepato- and/or splenomegaly

 • elevated ESR and/or leucocytosis

 • bone abnormalities

  1. Diagnostic criteria for Schnitzler’s Syndrome: the diagnosis is made when two major criteria are combined with at least two minor criteria
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172