Schnitzler syndrome: A rare disease with neutrophilic dermatosis and monoclonal gammopathy

Abstract

Schnitzler syndrome is a rare chronic auto-inflammatory disease characterized by neutrophilic dermatosis and a monoclonal gammopathy of IgM type. The clinical features of this disease like fever, rash, arthralgias and lymphadenopathy often overlaps with the presenting symptoms of other diseases like Castleman’s disease, adultonset Still’s disease and lymphomas. But the line of treatment is different for each of these clinical conditions. Schnitzler syndrome is associated with over-production of IL-1 and therapies are directed against this interleukin. Here, we present a case of Schnitzler syndrome, who was initially diagnosed as multi-centric Castleman’s disease and was treated with only partial resolution of symptoms.