von den Driesch P
Dept. of Dermatology, University of Erlangen-Nürnberg, Germany.
J Am Acad Dermatol. 1994 Oct;31(4):535-56; quiz 557-60. doi: 10.1016/s0190-9622(94)70215-2.
Acute febrile neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, has been termed Sweet's syndrome. Classic Sweet's syndrome occurs in middle-aged women after a nonspecific infection of the respiratory or gastrointestinal tract. Raised erythematous plaques with pseudoblistering and occasionally pustules occur on the face, neck, chest, and extremities, accompanied by fever and general malaise. Involvement of the eyes, joints, and oral mucosa as well as internal manifestations of Sweet's syndrome in the lung, liver, kidneys, and central nervous system has been described. The disease is thought to be a hypersensitivity reaction. Parainflammatory (e.g., infections, autoimmune disorders, vaccination) and paraneoplastic (e.g., hemoproliferative disorders, solid malignant tumors) occurrence is found in approximately 25% of the cases and 2% are associated with pregnancy. Sweet's syndrome responds rapidly to systemic therapy with corticosteroids but recurs in about 25% of the cases. Alternative treatment modalities (e.g., potassium iodide, colchicine, dapsone, clofazimine, cyclosporine) have also been used. This article presents data from 38 patients with Sweet's syndrome and reviews its epidemiology, clinical spectrum, histologic features, laboratory results, differential diagnosis, pathogenic mechanisms, associated diseases, and treatment.
急性发热性嗜中性皮病于1964年由罗伯特·道格拉斯·斯威特首次描述，现被称为斯威特综合征。典型的斯威特综合征发生于中年女性，常在呼吸道或胃肠道非特异性感染后出现。面部、颈部、胸部和四肢出现伴有假水疱形成且偶尔有脓疱的隆起性红斑斑块，同时伴有发热和全身不适。该病还可累及眼睛、关节和口腔黏膜，以及肺部、肝脏、肾脏和中枢神经系统等斯威特综合征的内部表现。本病被认为是一种超敏反应。约25%的病例存在副炎症性（如感染、自身免疫性疾病、疫苗接种）和副肿瘤性（如血液增殖性疾病、实体恶性肿瘤）发病情况，2%的病例与妊娠有关。斯威特综合征对全身应用糖皮质激素治疗反应迅速，但约25%的病例会复发。也使用过其他治疗方式（如碘化钾、秋水仙碱、氨苯砜、氯法齐明、环孢素）。本文介绍了38例斯威特综合征患者的数据，并对其流行病学、临床谱、组织学特征、实验室检查结果、鉴别诊断、发病机制、相关疾病及治疗进行了综述。
