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Schnitzler syndrome (SchS) is a rare, acquired, autoinflammatory disease that is sometimes associated with a fever of unknown origin (FUO). Elevated alkaline phosphatase (ALP) stemming from abnormal bone remodeling is a characteristic laboratory finding of SchS and is included in the diagnostic criteria. However, its utility as a clue to the diagnosis of SchS has been under-emphasized. We herein report a case of SchS presenting with a FUO and highly elevated ALP concentration, which led to repeated, unnecessary liver biopsies.

A patient with Schnitzler's syndrome is described presenting with fever of unknown origin. Although he had all characteristic features of the syndrome (urticarial vasculitis, hyperostosis, lymphadenopathy, fever and serum IgM monoclonal component), it was recognized very late in the diagnostic process. Cytokines were measured to get more insight into the role of cytokines in this syndrome, but only interleukin-6 was elevated. It is important for internists and rheumatologist to recognize this entity in order to prevent unnecessary diagnostic procedures.