Incomplete Schnitzler Syndrome
Mattias A S Henning1, Gregor B E Jemec, Kristina S Ibler
1Mattias A.S. Henning, MD, Department of Dermatology, Zealand University Hospital, Sygehusvej 5, 4000 Roskilde, Denmark; maahe@regionsjaelland.dk.
Abstract:
Schnitzler syndrome (SS) is a rare autoinflammatory disease that presents with chronic urticaria and monoclonal immunoglobulin (Ig) M or G, accompanied by fever, abnormal bone remodeling, skin biopsy with a neutrophilic dermal infiltrate, leukocytosis, or elevated C-reactive protein. It is usually refractory to antihistamines and immunosuppression. We present a case report of clinical SS without monoclonal Ig with robust response to interleukin-1 inhibitor anakinra. This suggests the possible existence of an incomplete form of SS and underlines the risk of false negative diagnosis in individuals with such "incomplete SS".
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