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Table 3 Differential diagnosis

From: Schnitzler’s syndrome: lessons from 281 cases

Immunological disorders  
  Adult-onset Still’s disease (AOSD)
  Systemic lupus erythematosus (SLE)
  Acquired C1 esterase deficiency
Hematological disorders  
  Monoclonal gammopathy of unknown significance (MGUS)
  Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome
  Waldenström’s macroglobulinemia (WM)
  Lymphoma, other than WM
  Multiple myeloma
Hereditary auto-inflammatory syndromes  
  Cryopyrin-associated periodic syndrome (CAPS)
Infectious diseases  
  Hepatitis B, C
  Chronic meningococcemia
  Chronic spontaneous urticaria
  (Hypocomplementaemic) urticarial vasculitis
  Delayed pressure urticaria
  Erdheim-Chester disease