Schnitzler Syndrome Without a Monoclonal Gammopathy: A Case Report.
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.


RESULTS AND 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.
['Urbanski|Michelle|M|', 'Holfeld|Karen|K|', 'Milne|Ardyth|A|', 'Abbas|Mariam|M|']
D018501:Antirheumatic Agents D003951:Diagnostic Errors D006801:Humans D053590:Interleukin 1 Receptor Antagonist Protein D008297:Male D008875:Middle Aged D019873:Schnitzler Syndrome D014581:Urticaria D014657:Vasculitis
Schnitzler syndrome autoinflammatory