Dodiuk-Gad Roni P, Chung Wen-Hung, Valeyrie-Allanore Laurence, Shear Neil H
Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre, 2075 Bayview Avenue, Room M1-737, Toronto, ON, M4N 3M5, Canada.
Department of Dermatology, Ha'emek Medical Center, Afula, Israel.
Am J Clin Dermatol. 2015 Dec;16(6):475-93. doi: 10.1007/s40257-015-0158-0.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous reactions, predominantly drug induced. The mortality rates for SJS and TEN are as high as 30 %, and short- and long-term morbidities are very common. SJS/TEN is one of the few dermatological diseases that constitute a true medical emergency. Early recognition and prompt and appropriate management can be lifesaving. In recent years, our understanding of the pathogenesis, clinical presentation, and management of SJS/TEN has improved. Nevertheless, in 2015, there are still no internationally accepted management guidelines. This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment. We hope these suggested guidelines serve as a practical clinical tool in the management of SJS/TEN. The classic manifestation of SJS/TEN consists of initial "flu-like" symptoms (malaise, fever, anorexia) in the prodromal phase, followed by cutaneous and mucous membrane (ocular, oral, and genital) inflammation and pain, and other systemic involvement. Symptoms usually begin 4-28 days after the onset of drug intake. Treatment is multidisciplinary and includes identification and withdrawal of the culprit drug, transfer to a specialist unit, supportive care, medical treatment, communication, and provision of appropriate information and emotional support.
史蒂文斯 - 约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)是危及生命的皮肤黏膜反应,主要由药物引起。SJS和TEN的死亡率高达30%,短期和长期发病率都很常见。SJS/TEN是少数几种构成真正医疗急症的皮肤病之一。早期识别以及迅速且恰当的处理可挽救生命。近年来,我们对SJS/TEN的发病机制、临床表现及处理的认识有所提高。然而,在2015年,仍然没有国际公认的处理指南。本综述总结了关于SJS/TEN的最新见解,并描述了评估和治疗方案。我们希望这些建议的指南能成为管理SJS/TEN的实用临床工具。SJS/TEN的典型表现包括前驱期最初的“流感样”症状(不适、发热、厌食),随后是皮肤和黏膜(眼部、口腔和生殖器)炎症及疼痛,以及其他全身受累情况。症状通常在服药后4 - 28天开始出现。治疗是多学科的,包括识别并停用致病药物、转至专科病房、支持治疗、药物治疗、沟通以及提供适当信息和情感支持。