SCHNITZLER SYNDROME

Clinical

A multiple year history of 1-2 cm urticarial lesions occurring mainly on the abdomen and legs is characteristic. The lesions appear and clear within 24 hours, ala urticaria. Fever, fatigue, weight loss, arthralgias and muscle or bone pain are also typical and may accompany the outbreaks. Lymph node enlargement and hepatosplenomegaly commonly occur. An IgM paraprotein confirms the diagnosis.

Since urticaria is so common, the question arises, "When should one work up a patient with urticaria for Schnitzler syndrome?" It seems logical to do so in the patient with urticaria that is unresponsive and long-lasting and is associated with bone pain and/or neutrophilic urticaria histologically.

Workup

Referral to a hematologist/oncologist is appropriate. CBC, routine blood studies, CXR, bone survey and bone marrow biopsy to exclude Waldenstrom's disease may be necessary. Lymphoplasmacytic lymphoma develops in approximately 10-15% of patients [JAAD 1989;20;206]; thus close follow-up is warranted.

Treatment

H1 and H2 blocking antihistamines usually are not helpful. Ibuprofen, colchicine, and even high dose corticosteroid therapy may be tried. Dapsone 50 mg/d was successful in one case.

References

JAAD November 2010 Volume 63, Issue 5, Pages 918–920

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