Lipsker et al listed criteria for making the diagnosis of Schnitzler's Syndrome. The authors are from Hopitaux Universitaries Strasbourg and Hopital d'Instruction des armees Legouest (Metz) in France.
Major criteria - both of the following:
(1) urticarial skin rash
(2) monoclonal IgM component
Minor criteria - 2 or more of the following:
(1) fever
(2) arthralgias or arthritis
(3) bone pain
(4) palpable lymph nodes
(5) liver or spleen enlargement
(6) elevated erythrocyte sedimentation rate (ESR)
(7) leukocytosis
(8) abnormal findings on bone morphological investigations (increased bone density, periosteal apposition, other)
Exclusion of other explanations for findings including:
(1) hyper IgD syndrome
(2) adult-onset Still's syndrome
(3) cryoglobulinemia
(4) acquired C1 inhibitor deficiency
where:
• The authors also excluded based on hypcomplementemic urticarial vasculitis, which were considered key features of Schnitzler's syndrome in the previous section. It can also be seen in a number of other conditions such as SLE.
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