Cutaneous Manifestations of Sweet Disease

Abstract

Sweet disease, also known as acute febrile neutrophilic dermatosis, is a multisystem inflammatory disorder characterized by painful erythematous plaques and aseptic neutrophilic infiltration of various organs. It is also characterized by fever, polymorphonuclear leukocytosis, and painful erythematous cutaneous plaques. Cutaneous manifestations of Sweet disease are typically painful plaque-forming erythematous papules. Skin biopsies typically demonstrate dermal infiltration with neutrophils in the absence of vasculitis. Sweet disease is often associated with a hematological malignancy, especially acute myeloid leukemia or myeloid dysplasia syndrome. Sweet disease has clinical and laboratory features similar to those of Behçet's disease. Overlap exists between the clinical manifestations of Sweet disease and Behçet's disease. The neuro-Sweet disease is similar to the neuro-Behçet's disease. This article presents information on the pathogenesis, clinical approach, treatment, and expected evolution of these manifestations including the defference between neuro-Sweet disease and neuro-Behçet's disease. The possible role in this interesting association of Behçet's disease and Sweet disease is discussed and a review of the literature is presented.