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史蒂文斯-约翰逊综合征和中毒性表皮坏死松解症——识别与管理的挑战

Stevens-Johnson syndrome and toxic epidermal necrolysis-challenges of recognition and management.

作者信息

Sane S P, Bhatt A D

机构信息

Novartis India Limited, Medical Department, Khetan Bhavan, 198, J Tata Road, Churchgate, Mumbai-400 020.

出版信息

J Assoc Physicians India. 2000 Oct;48(10):999-1003.

PMID:11200928
Abstract

Skin adverse drug reactions (ADRs) generally present as transient erythematous macular/papular rashes. However these can many a times be the initial presentation of serious muco-cutaneous ADRs such as Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN). The incidence of SJS varies from 1.2 to 6 per million patient-years and that of TEN to be 0.4 to 1.2 per million patient-years. The pathophysiological mechanism of SJS and TEN have not been fully elucidated. The aetiological factors of SJS and TEN are diverse; drugs being the cause in more than 80% cases of TEN and about 40-50% cases of SJS. Mucous membranes are affected in nearly all cases. The extent of epidermal sloughing may vary and forms a basis for the classification of an individual case as SJS or TEN. Prognosis of SJS is better than that of TEN; mortality rates being about 5% and 30%-40% respectively. Specific therapy for these conditions is yet not available. The use of systemic corticosteroids has been controversial. Early diagnoses can prevent/reduce the morbidity of such serious ADRs. This article provides a brief review of the clinical presentation and management of SJS and TEN.

摘要

皮肤药物不良反应(ADR)通常表现为短暂性红斑丘疹性皮疹。然而,这些皮疹很多时候可能是严重黏膜皮肤ADR的初始表现,如史蒂文斯-约翰逊综合征(SJS)或中毒性表皮坏死松解症(TEN)。SJS的发病率为每百万患者年1.2至6例,TEN的发病率为每百万患者年0.4至1.2例。SJS和TEN的病理生理机制尚未完全阐明。SJS和TEN的病因多种多样;药物是TEN超过80%病例以及SJS约40%-50%病例的病因。几乎所有病例都会累及黏膜。表皮剥脱的程度可能有所不同,这构成了将个体病例分类为SJS或TEN的基础。SJS的预后优于TEN;死亡率分别约为5%和30%-40%。目前尚无针对这些病症的特效治疗方法。全身使用糖皮质激素一直存在争议。早期诊断可预防/降低此类严重ADR的发病率。本文简要综述了SJS和TEN的临床表现及治疗。

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