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The rare clinical entity Schnitzler syndrome combines chronic urticarial dermatosis with an IgM monoclonal gammopathy and at least two of the following features: bone pain, fever, lymphadenopathy, hepatosplenomegaly, arthralgia/arthritis, or leukocytosis. The histopathology of Schnitzler syndrome is most often characterized by a neutrophilic, perivascular infiltration. Patients with this disease may have or may develop a lymphoproliferative disorder. Treatment with anakinra or related drugs has controlled the disease (also see Two New Treatments for Schnitzler Syndrome in this issue).
These authors retrospectively examined 20 Schnitzler syndrome patients seen at Mayo Clinic between 1972 and July 2011. Roughly 65% were men, and the mean age w…