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Table 1 Strasbourg diagnostic criteria for Schnitzler’s syndrome 1

From: Schnitzler’s syndrome: lessons from 281 cases

Obligate criteria  
  Chronic urticarial rash
  Monoclonal IgM or IgG
Minor criteria  
  Recurrent fever2
  Objective findings of abnormal bone remodeling with or without bone pain3
  A neutrophilic dermal infiltrate on skin biopsy4
  Leukocytosis and/or elevated CRP5
Definite diagnosis if  
  Two obligate criteria AND at least two minor criteria if IgM, and three minor criteria if IgG
Probable diagnosis if  
  Two obligate criteria AND at least one minor criterion if IgM, and two minor criteria if IgG
  1. 1Adopted from Simon et al., Allergy [1].
  2. 2A valid criterion if objectively measured. Must be >38°C, and otherwise unexplained. Occurs usually – but not obligatory – together with the skin rash.
  3. 3As assessed by bone scintigraphy, MRI or elevation of bone alkaline phosphatase.
  4. 4Corresponds usually to the entity described as ‘neutrophilic urticarial dermatosis’ (Medicine 2009;88:23–31); absence of fibrinoid necrosis and significant dermal edema.
  5. 5Neutrophils >10 000/mm3 and/or CRP >30 mg/L.