Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access
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Case Report

Volume 10, Number 3, June 2021, pages 143-146

Schnitzler’s Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria

Figure

Figure 1.
Figure 1. Skin punch biopsy of right forearm. Histopathologic examination revealed neutrophilic and eosinophilic infiltration of the dermis, including perivascular mixed inflammatory infiltrate suggestive of urticaria (hematoxylin and eosin (H&E), × 20). Perivascular mixed infiltrate of lymphocytes, eosinophils and neutrophils (upper arrow); surrounding sparse mixed inflammatory interstitial infiltrate with accompanying dermal edema (lower arrow).

Table

Table 1. Strasbourg Diagnostic Criteria
 
aA valid criterion if objectively measured; it must be > 38 °C, and otherwise unexplained; occurs usually, but not obligatory, together with the skin rash. bAs assessed by bone scintigraphy, MRI or elevation of bone alkaline phosphatase. cCorresponds usually to the entity described as “neutrophilic urticarial dermatosis” [4]; absence of fibrinoid necrosis and significant dermal edema. dNeutrophils > 10,000/mm3 and/or CRP > 30 mg/L. Ig: immunoglobulin; CRP: C-reactive protein; MRI: magnetic resonance imaging.
Obligate criteria
  Chronic urticarial rash +
  Monoclonal IgM or IgG
Minor criteria
  Recurrent fevera
  Objective findings of abnormal bone remodeling with or without bone painb
  A neutrophilic dermal infiltrate on skin biopsyc
  Leukocytosis and/or elevated CRPd
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