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Ibrutinib for the Management of Schnitzler Syndrome: A Novel Therapy for a Rare Condition.

Schnitzler syndrome is a rare dermatologic disorder that accompanies IgMmonoclonal gammopathy and is characterized by a chronic urticarial-type eruption in association with intermittent fever, arthralgia or arthritis, and lymphadenopathy. The etiology of Schnitzler syndrome remains unknown; however, most hypotheses suggest the involvementof autoreactive antibodies. The interleukin-1 (IL-1) cellular pathway is implicated in its pathogenesis, and IL-1 receptor blockade results in rapid skin clearance. Recurrences are the normwithin 48 to 72 hours after the discontinuation of anti–IL-1 treatment.