An unusual presentation of immunoglobulin A gammopathy in a patient with Schnitzler's syndrome

Abstract

This case summarizes a 69-year-old woman who presented with a history of recurrent fevers, widespread urticarial rash and generalized myalgias for several years with an eventual diagnosis of Schnitzler's syndrome. This is a rare autoinflammatory condition which typically involves a chronic urticarial rash, monoclonal immunoglobulin M (IgM) or IgG gammopathy. Rapid improvement in above symptoms were noted with anakinra, an interleukin-1 receptor antagonist. We report an unusual case of a 69-year-old woman who presented with an isolated IgA monoclonal gammopathy.

Keywords: IL-1 receptor antagonist; IgA gammopathy; Schnitzler's syndrome; Schnitzler-like syndromes; anakinra.