Schnitzler Syndrome with Delirium and Vertigo: the Utility of Neurologic Manifestations in Diagnosis
Tolkachjov, S.N.; Wetter, D.A.
Journal of Drugs in Dermatology Jdd 16(6): 625-627
2017
ISSN/ISBN: 1545-9616 PMID: 28686783 Document Number: 17242
Schnitzler syndrome (SS) is an autoinflammatory dermatosis that often goes undiagnosed for 5-6 years. Patients typically carry a diagnosis of urticaria; however, their cutaneous symptoms fail to respond to typical urticaria therapies and lack symptoms such as pruritus. Additionally, patients with SS may see multiple providers for nonspecific complaints of fever, lymphadenopathy, arthralgias, and bone pain. A correct diagnosis is paramount, as close to 20% of patients may develop a lymphoproliferative disorder and appropriate treatment may ameliorate all symptoms.1 We report 2 cases of SS misdiagnosed as urticaria for years in order to illuminate diagnostic pearls, histopathological findings, and treatment modalities. Additionally, we highlight the importance of neurologic disturbances in this rare but important differential diagnosis of urticaria.
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