[Sweet syndrome: personal experience and review of the literature]

The Sweet's syndrome or acute febrile neutrophilic dermatosis is a well characterized cutaneous disease frequently associated with inflammatory and neoplastic diseases, particularly haematologic malignancies, and usually precedes the underlying disorders for months or years. Much of the evidence for this is based on case reports, small series of cases and reviews of the literature. Recently, immunologic theories suggest that the pathogenesis of Sweet's syndrome is probably mediated through helper T cell type 1 cytokines (IL-2, gammaINF) rather than helper T cell type 2 cytokines (IL-4). This results in the stimulation of a cytokine cascade, which might be responsible for the local and systemic activation of neutrophils and histiocytes. Clinically, Sweet's syndrome is characterized by the acute onset of an eruption of painful erythematous or violaceous plaques or nodules with fever, malaise, neutrophil leucocytosis and a raised erythrocyte sedimentation rate. Personal experience in 4 cases observed during five years is described. All of them presented diagnostic criteria of this disease and had a good response to corticotherapy and/or indomethacin. A review of the literature on this uncommon syndrome is also reported.