title: Schnitzler Syndrome Without a Monoclonal Gammopathy: A Case Report.
abstract: BACKGROUND
  Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a recurrent urticarial rash and a monoclonal immunoglobulin M gammopathy, as well as 2 of the following minor criteria: recurrent fever (>38°C), objective signs of abnormal bone remodeling, elevated C-reactive protein level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. Alternatively, a monoclonal immunoglobulin G gammopathy may be present along with 3 minor criteria for diagnosis.
  
  
  OBJECTIVE
  To report a rare case of SS without monoclonal gammopathy and inform physicians of this possible clinical presentation so that treatment is not delayed.
  
  
  METHODS
  We report a case of a 62-year-old white man with a clinical diagnosis of SS without monoclonal gammopathy. He presented with chronic urticaria unresponsive to conventional therapy.
  
  
  CONCLUSIONS
  To our knowledge, there have only been 3 case reports of SS in the absence of monoclonal gammopathy documented in the literature. SS should be considered based on clinical presentation, even in the absence of monoclonal gammopathy, to facilitate appropriate management.
authors.affiliation: Division of Dermatology, Department of Medicine, University of Saskatchewan, Regina, SK, Canada.
authors.forename: Michelle
authors.initials: M
authors.lastname: Urbanski
authors.affiliation: Division of Dermatology, Department of Medicine, University of Saskatchewan, Regina, SK, Canada karenandbarry@sasktel.net.
authors.forename: Karen
authors.initials: K
authors.lastname: Holfeld
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Saskatchewan, Regina, SK, Canada.
authors.forename: Ardyth
authors.initials: A
authors.lastname: Milne
authors.affiliation: Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, AB, Canada.
authors.forename: Mariam
authors.initials: M
authors.lastname: Abbas
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use; D003951:Diagnostic Errors; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D019873:Schnitzler Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D014581:Urticaria / Q000209:etiology; D014657:Vasculitis / Q000175:diagnosis*
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein
keywords: Schnitzler syndrome; autoinflammatory