SWEETS-SYNDROME (ACUTE FEBRILE NEUTROPHILIC DERMATOSIS)

Acute febrile neutrophilic dermatosis, first described in 1964 by Robe rt Douglas Sweet, has been termed Sweet's syndrome. Classic Sweet's sy ndrome occurs in middle-aged women after a nonspecific infection of th e respiratory or gastrointestinal tract. Raised erythematous plaques w ith pseudoblistering and occasionally pustules occur on the face, neck , chest, and extremities, accompanied by fever and general malaise. In volvement of the eyes, joints, and oral mucosa as well as internal man ifestations of Sweet's syndrome in the lung, liver, kidneys, and centr al nervous system has been described. The disease is thought to be a h ypersensitivity reaction. Parainflammatory (e.g., infections, autoimmu ne disorders, vaccination) and paraneoplastic (e.g., hemoproliferative disorders, solid malignant tumors) occurrence is found in approximate ly 25% of the cases and 2% are associated with pregnancy. Sweet's synd rome responds rapidly to systemic therapy with corticosteroids but rec urs in about 25% of the cases. Alternative treatment modalities (e.g., potassium iodide, colchicine, dapsone, clofazimine, cyclosporine) hav e also been used. This article presents data from 38 patients with Swe et's syndrome and reviews its epidemiology, clinical spectrum, histolo gic features, laboratory re suits, differential diagnosis, pathogenic mechanisms, associated diseases, and treatment.