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史蒂文斯-约翰逊综合征与中毒性表皮坏死松解症的差异。

Differences between Stevens-Johnson syndrome versus toxic epidermal necrolysis.

机构信息

Mayo Clinic Medical Scientist Training Program, Mayo Clinic Alix School of Medicine and Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic, Rochester, MN, USA.

Department of Dermatology, Mayo Clinic, Rochester, MN, USA.

出版信息

Int J Dermatol. 2021 Jan;60(1):53-59. doi: 10.1111/ijd.15287. Epub 2020 Nov 30.

Abstract

BACKGROUND

To retrospectively review the outcomes of two rare cutaneous diseases, Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), and to question the practice of averaging the mortality rate on the assumption that they are one disease.

METHODS

A retrospective chart review of all patients diagnosed with SJS and TEN by a dermatologist between January 1, 2000, and January 1, 2020, at our institution was performed. Seventy-one patients were identified (21 pediatric and 50 adults). Pathology slides from 32 adult patients (64%) were evaluated by a blinded board-certified dermatopathologist.

RESULTS

Of the adult patients, 31 had SJS, two had SJS-TEN overlap, and 17 had TEN. All 21 patients in the pediatric group were diagnosed with SJS mainly caused by Mycoplasma. Mortality rates were 6.5% for SJS among adults and 35.3% for TEN. Chemotherapy-induced TEN is a trigger with 50% mortality.

CONCLUSIONS

SJS was more common in adults and pediatric cases than TEN (3:1) and had a much better prognosis and outcome. Combining and averaging the mortality rates of TEN and SJS are not advised as SJS is mainly a mucocutaneous disorder with good prognosis versus TEN, a systemic toxicity of multiple organs with deep skin detachment.

摘要

背景

回顾两种罕见的皮肤疾病史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死松解症(TEN)的结果,并质疑将这两种疾病平均死亡率的做法,因为它们被认为是一种疾病。

方法

对 2000 年 1 月 1 日至 2020 年 1 月 1 日期间我院皮肤科诊断为 SJS 和 TEN 的所有患者进行回顾性图表审查。共确定了 71 例患者(21 例儿科和 50 例成人)。对 32 例成年患者(64%)的病理切片由一位盲法认证的皮肤科病理学家进行评估。

结果

在成年患者中,31 例为 SJS,2 例为 SJS-TEN 重叠,17 例为 TEN。儿科组的 21 例患者均被诊断为主要由支原体引起的 SJS。成人 SJS 的死亡率为 6.5%,TEN 的死亡率为 35.3%。化疗诱导的 TEN 是一个死亡率为 50%的触发因素。

结论

SJS 在成人和儿科病例中比 TEN 更常见(3:1),且预后和结局要好得多。不建议将 TEN 和 SJS 的死亡率合并和平均化,因为 SJS 主要是一种黏膜皮肤疾病,预后良好,而 TEN 则是一种多器官系统性毒性,伴有深度皮肤脱落。

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