Schnitzler’s syndrome: A diagnostic crossroads

Abstract

Schnitzler syndrome is a rare disease, in Colombia it is considered an orphan disease, of an auto-inflammatory nature, classified as a complex acquired inflammatory type of disease, which classically produces urticarial rash, long-standing fever, adenomegalies, and arthralgias coexisting with monoclonal gamma peak typically of the IgM type. We present the case of a young woman, with a larval picture that started with urticarial rash, with clinical characteristics compatible with the syndrome and evidence of monoclonal peak in protein electrophoresis meeting Lipsker-Baltimore 2001 criteria and Strasbourg 2013 criteria for diagnosis.