Hama Natsumi, Aoki Shigeki, Chen Chun-Bing, Hasegawa Akito, Ogawa Youichi, Vocanson Marc, Asada Hideo, Chu Chia-Yu, Lan Cheng-Che E, Dodiuk-Gad Roni P, Fujiyama Toshiharu, Hsieh Tyng-Shiuan, Ito Kousei, Jerschow Elina, Mizukawa Yoshiko, Nakajima Saeko, Nakamura Kazutoshi, Nicolas Jean-François, Satoh Takashi K, Shiohara Tatsuo, Takahashi Hayato, Tohyama Mikiko, Ueda Takahiro, Ura Katsuaki, Watanabe Hideaki, Yamaguchi Yukie, Nordmann Thierry M, Chung Wen-Hung, Naisbitt Dean, Pincelli Carlo, Pichler Werner J, French Lars E, Phillips Elizabeth, Abe Riichiro
Division of Dermatology, Niigata University Graduate School of Medical and Dental Science, Niigata, Japan.
Br J Dermatol. 2024 Dec 23;192(1):9-18. doi: 10.1093/bjd/ljae321.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) represent a severe spectrum of rare mucocutaneous reactions that are primarily drug-induced and characterized by significant morbidity and mortality. These conditions manifest in 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 advances in understanding the immunopathogenesis of SJS/TEN are explored, emphasizing the role of drug-specific T-cell responses and human leucocyte antigen polymorphisms in disease onset. The review also addresses current therapeutic approaches, including controversies surrounding the use of immunosuppressive agents and the emerging role of tumour necrosis factor-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 the disease and its mechanisms.
史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）是一系列严重的罕见黏膜皮肤反应，主要由药物引起，具有较高的发病率和死亡率。这些病症表现为广泛的皮肤脱落，这使其有别于其他全身性皮疹。SJS/TEN的罕见性和严重性凸显了准确诊断标准和有效治疗方法的重要性，而目前这些方面缺乏共识。本综述提出了新的诊断标准，以提高特异性和全球适用性。探讨了在理解SJS/TEN免疫发病机制方面的最新进展，强调了药物特异性T细胞反应和人类白细胞抗原多态性在疾病发病中的作用。该综述还讨论了当前的治疗方法，包括围绕免疫抑制剂使用的争议以及肿瘤坏死因子-α抑制剂的新作用。讨论了针对特定致病机制（如坏死性凋亡和特定免疫细胞途径）的新型治疗策略。此外，还研究了基于这些见解开发的新药，包括靶向单克隆抗体和抑制剂。综述最后倡导跨多学科医学领域做出更有力、更协调的努力，以开发针对SJS/TEN的有效治疗方法和诊断工具，旨在改善患者预后并增进对该疾病及其机制的了解。
