史蒂文斯-约翰逊综合征/中毒性表皮坏死症的最新进展:诊断标准、发病机制和治疗。

IF 11 1区 医学 Q1 DERMATOLOGY
Natsumi Hama, Shigeki Aoki, Chun-Bing Chen, Akito Hasegawa, Youichi Ogawa, Marc Vocanson, Hideo Asada, Chia-Yu Chu, Cheng-Che E Lan, Roni P Dodiuk-Gad, Toshiharu Fujiyama, Tyng-Shiuan Hsieh, Kousei Ito, Elina Jerschow, Yoshiko Mizukawa, Saeko Nakajima, Kazutoshi Nakamura, Jean-François Nicolas, Takashi K Satoh, Tatsuo Shiohara, Hayato Takahashi, Mikiko Tohyama, Takahiro Ueda, Katsuaki Ura, Hideaki Watanabe, Yukie Yamaguchi, Thierry M Nordmann, Wen-Hung Chung, Dean Naisbitt, Carlo Pincelli, Werner J Pichler, Lars E French, Elizabeth Phillips, Riichiro Abe
{"title":"史蒂文斯-约翰逊综合征/中毒性表皮坏死症的最新进展:诊断标准、发病机制和治疗。","authors":"Natsumi Hama, Shigeki Aoki, Chun-Bing Chen, Akito Hasegawa, Youichi Ogawa, Marc Vocanson, Hideo Asada, Chia-Yu Chu, Cheng-Che E Lan, Roni P Dodiuk-Gad, Toshiharu Fujiyama, Tyng-Shiuan Hsieh, Kousei Ito, Elina Jerschow, Yoshiko Mizukawa, Saeko Nakajima, Kazutoshi Nakamura, Jean-François Nicolas, Takashi K Satoh, Tatsuo Shiohara, Hayato Takahashi, Mikiko Tohyama, Takahiro Ueda, Katsuaki Ura, Hideaki Watanabe, Yukie Yamaguchi, Thierry M Nordmann, Wen-Hung Chung, Dean Naisbitt, Carlo Pincelli, Werner J Pichler, Lars E French, Elizabeth Phillips, Riichiro Abe","doi":"10.1093/bjd/ljae321","DOIUrl":null,"url":null,"abstract":"<p><p>Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions, primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest through extensive skin detachment, distinguishing them from other generalized skin eruptions. The rarity and severity of SJS/TEN underscore the importance of accurate diagnostic criteria and effective treatments, which are currently lacking consensus. This review proposes new diagnostic criteria to improve specificity and global applicability. Recent advancements in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T cell responses and HLA polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of TNF-α inhibitors. Novel therapeutic strategies targeting specific pathogenic mechanisms, such as necroptosis and specific immune cell pathways, are discussed. Furthermore, the development of new drugs based on these insights, including targeted monoclonal antibodies and inhibitors, are examined. The review concludes by advocating for more robust and coordinated efforts across multidisciplinary medical fields to develop effective treatments and diagnostic tools for SJS/TEN, with the aim of improving patient outcomes and understanding of the disease and its mechanisms.</p>","PeriodicalId":9238,"journal":{"name":"British Journal of Dermatology","volume":null,"pages":null},"PeriodicalIF":11.0000,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Recent progress of Stevens-Johnson syndrome/toxic epidermal necrolysis: Diagnosis criteria, pathogenesis and therapy.\",\"authors\":\"Natsumi Hama, Shigeki Aoki, Chun-Bing Chen, Akito Hasegawa, Youichi Ogawa, Marc Vocanson, Hideo Asada, Chia-Yu Chu, Cheng-Che E Lan, Roni P Dodiuk-Gad, Toshiharu Fujiyama, Tyng-Shiuan Hsieh, Kousei Ito, Elina Jerschow, Yoshiko Mizukawa, Saeko Nakajima, Kazutoshi Nakamura, Jean-François Nicolas, Takashi K Satoh, Tatsuo Shiohara, Hayato Takahashi, Mikiko Tohyama, Takahiro Ueda, Katsuaki Ura, Hideaki Watanabe, Yukie Yamaguchi, Thierry M Nordmann, Wen-Hung Chung, Dean Naisbitt, Carlo Pincelli, Werner J Pichler, Lars E French, Elizabeth Phillips, Riichiro Abe\",\"doi\":\"10.1093/bjd/ljae321\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions, primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest through extensive skin detachment, distinguishing them from other generalized skin eruptions. The rarity and severity of SJS/TEN underscore the importance of accurate diagnostic criteria and effective treatments, which are currently lacking consensus. This review proposes new diagnostic criteria to improve specificity and global applicability. Recent advancements in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T cell responses and HLA polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of TNF-α inhibitors. Novel therapeutic strategies targeting specific pathogenic mechanisms, such as necroptosis and specific immune cell pathways, are discussed. Furthermore, the development of new drugs based on these insights, including targeted monoclonal antibodies and inhibitors, are examined. The review concludes by advocating for more robust and coordinated efforts across multidisciplinary medical fields to develop effective treatments and diagnostic tools for SJS/TEN, with the aim of improving patient outcomes and understanding of the disease and its mechanisms.</p>\",\"PeriodicalId\":9238,\"journal\":{\"name\":\"British Journal of Dermatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":11.0000,\"publicationDate\":\"2024-08-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"British Journal of Dermatology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1093/bjd/ljae321\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"British Journal of Dermatology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/bjd/ljae321","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

史蒂文斯-约翰逊综合征(SJS)和中毒性表皮坏死溶解症(TEN)是一种严重的罕见皮肤粘膜反应,主要由药物引起,发病率和死亡率都很高。这些病症表现为大面积皮肤脱落,有别于其他全身性皮肤糜烂。SJS/TEN 的罕见性和严重性凸显了准确诊断标准和有效治疗的重要性,而目前对此还缺乏共识。本综述提出了新的诊断标准,以提高特异性和全球适用性。本综述探讨了在了解 SJS/TEN 免疫发病机制方面的最新进展,强调了药物特异性 T 细胞反应和 HLA 多态性在发病中的作用。综述还探讨了当前的治疗方法,包括围绕使用免疫抑制剂的争议和 TNF-α 抑制剂的新作用。还讨论了针对特定致病机制(如坏死和特定免疫细胞通路)的新型治疗策略。此外,还探讨了基于这些见解的新药开发,包括靶向单克隆抗体和抑制剂。综述最后提倡在多学科医学领域开展更有力、更协调的工作,开发针对 SJS/TEN 的有效治疗方法和诊断工具,以改善患者的治疗效果,加深对该疾病及其机制的了解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Recent progress of Stevens-Johnson syndrome/toxic epidermal necrolysis: Diagnosis criteria, pathogenesis and therapy.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions, primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest through extensive skin detachment, distinguishing them from other generalized skin eruptions. The rarity and severity of SJS/TEN underscore the importance of accurate diagnostic criteria and effective treatments, which are currently lacking consensus. This review proposes new diagnostic criteria to improve specificity and global applicability. Recent advancements in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T cell responses and HLA polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of TNF-α inhibitors. Novel therapeutic strategies targeting specific pathogenic mechanisms, such as necroptosis and specific immune cell pathways, are discussed. Furthermore, the development of new drugs based on these insights, including targeted monoclonal antibodies and inhibitors, are examined. The review concludes by advocating for more robust and coordinated efforts across multidisciplinary medical fields to develop effective treatments and diagnostic tools for SJS/TEN, with the aim of improving patient outcomes and understanding of the disease and its mechanisms.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
British Journal of Dermatology
British Journal of Dermatology 医学-皮肤病学
CiteScore
16.30
自引率
3.90%
发文量
1062
审稿时长
2-4 weeks
期刊介绍: The British Journal of Dermatology (BJD) is committed to publishing the highest quality dermatological research. Through its publications, the journal seeks to advance the understanding, management, and treatment of skin diseases, ultimately aiming to improve patient outcomes.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信