Schnitzler Syndrome: Clinical Case and Review of The Literature

Abstract

Schnitzler syndrome is a rare auto-inflammatory disease with a chronic relapsing course, characterized by recurrent urticarial lesions and monoclonal gammopathy. The manifestations of systemic inflammation are complemented by fever, bone and muscle pain, arthralgia/arthritis, lymphadenopathy, hepato- or splenomegaly, increased levels of acute inflammatory markers. A biological marker of the disease is the monoclonal gammopathy. Patients show high risk to develop AA-amyloidosis and lymphoproliferative diseases. A patient with a 5-year delay in diagnosis, who has responded well to systemic corticosteroids is presented. A comprehensive review of the new insights of disease pathogenesis and therapy is also highlighted.