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Sweet's Syndrome as Precursor of Hairy Cell Leukemia.

A 57-year-old man reported a 6-month history of skin lesions without systemic symptoms. We found succulent erythematous plaques concentrated mainly on the trunk. Based on the clinical findings and confirmatory histology, we diagnosed Sweet’s syndrome. An extensive work-up to rule out malignancy (differential blood count, thoracic and abdominal computed tomography, esophagogastroduodenoscopy, and colonoscopy) revealed no abnormal findings. The patient was treated with prednisolone in varying doses accompanied by 100 mg/d dapsone, with moderate success. Over the ensuing 12 months he developed progressive pancytopenia. Examination of a bone marrow sample to exclude hematological disease showed infiltration by a B-cell non-Hodgkin lymphoma (CD103+) with inhibition of hematopoiesis, consistent with hairy cell leukemia. The patient received a cycle of treatment with the cytostatic drug cladribine and has been in hematological remission ever since. The skin lesions healed a short time later. Sweet’s syndrome is often found together with infection, but in 10% to 20% of cases it is accompanied by a hematological malignancy, and occasionally a solid tumor. Chronic recurrence of Sweet’s syndrome should prompt investigation for an associated malignancy.