National Skin Centre, 1 Mandalay Road, Singapore, 308205.
Department of Dermatology, Singapore General Hospital, Singapore.
Br J Dermatol. 2015 Nov;173(5):1250-4. doi: 10.1111/bjd.13989.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare conditions characterized by extensive epidermal detachment and mucositis. Both are associated with a high mortality rate and significant long-term morbidity. Since the initial report introducing the term TEN in 1956, diagnosis of the condition has been fraught with difficulties that continue to exist today. The terms 'erythema multiforme major' (EMM) and SJS, and their relationship to TEN have also been confusing to clinicians. It is now recognized that EMM is a different entity from SJS and TEN in terms of demographics, causality and severity. SJS and TEN represent a continuum of disease, and differ only by the extent of epidermal detachment and therefore severity. The term 'epidermal necrolysis' (EN) is used in this article to describe the spectrum of disease that includes SJS and TEN. Important advances in understanding the pathomechanism and treatment of EN have been made over the years. These include the recognition of human leucocyte antigen (HLA) associations (e.g. HLA-B*1502 with carbamazepine-induced TEN) and understanding of the pathogenic roles of drug-specific cytotoxic T cells and granulysin. It was previously believed that widespread keratinocyte death in EN is predominantly mediated by soluble Fas-ligand and that intravenous immunoglobulin therapy is useful in blocking this mechanism with resultant survival benefits. Further studies have since proven these theories to be incorrect. This short review describes the key advances in the terminology, classification, causality and treatment of EN, and identifies future priorities and challenges in the understanding and management of this condition.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是罕见的疾病，其特征为广泛的表皮脱落和黏膜炎。这两种疾病都与高死亡率和显著的长期发病率相关。自 1956 年首次引入 TEN 这一术语以来，其诊断一直存在困难，这些困难至今仍然存在。术语“多形性红斑重大型”（EMM）和 SJS 及其与 TEN 的关系也一直令临床医生感到困惑。现在人们认识到，EMM 在人口统计学、病因和严重程度方面与 SJS 和 TEN 不同。SJS 和 TEN 代表疾病的连续体，仅在表皮脱落的程度和严重程度上有所不同。本文中使用“表皮坏死松解症”（EN）这一术语来描述包括 SJS 和 TEN 在内的疾病谱。近年来，人们对 EN 的发病机制和治疗方法有了重要的认识进展。这些进展包括对人类白细胞抗原（HLA）相关性的认识（例如 HLA-B*1502 与卡马西平诱导的 TEN）以及对药物特异性细胞毒性 T 细胞和颗粒溶素的致病作用的理解。以前认为，EN 中广泛的角质形成细胞死亡主要是由可溶性 Fas 配体介导的，静脉注射免疫球蛋白治疗可通过阻断这一机制发挥作用，并带来生存获益。此后的进一步研究证明了这些理论是不正确的。本文简要描述了 EN 的术语、分类、病因和治疗方面的关键进展，并确定了在理解和管理这种疾病方面未来的优先事项和挑战。
