Tóth Gábor, Lukács Andrea, Schirra Frank, Sándor Gábor L, Killik Petra, Maneschg Otto A, Nagy Zoltán Z, Szentmáry Nóra
Dr. Rolf M. Schwiete Center for Limbal Stem Cell and Congenital Aniridia Research, Saarland University, Kirrberger Str. 100, 66424, Homburg/Saar, Germany.
Department of Ophthalmology, Semmelweis University, Mária Utca 39, 1085, Budapest, Hungary.
Ophthalmol Ther. 2023 Aug;12(4):1795-1811. doi: 10.1007/s40123-023-00725-w. Epub 2023 May 4.
The aim of our review article was to summarize the current literature on Stevens-Johnson syndrome (SJS) and its severe form, toxic epidermal necrolysis (TEN). SJS/TEN is a serious, rare multi-system, immune-mediated, mucocutaneous disease with a significant mortality rate that can lead to severe ocular surface sequelae and even to bilateral blindness. Restoration of the ocular surface in acute and chronic SJS/TEN is challenging. There are only limited local or systemic treatment options for SJS/TEN. Early diagnosis, timely amniotic membrane transplantation and aggressive topical management in acute SJS/TEN are necessary to prevent long-term, chronic ocular complications. Although the primary aim of acute care is to save the life of the patient, ophthalmologists should regularly examine patients already in the acute phase, which should also be followed by systematic ophthalmic examination in the chronic phase. Herein, we summarize actual knowledge on the epidemiology, aetiology, pathology, clinical appearance and treatment of SJS/TEN.
我们这篇综述文章的目的是总结当前关于史蒂文斯-约翰逊综合征（SJS）及其严重形式中毒性表皮坏死松解症（TEN）的文献。SJS/TEN是一种严重、罕见的多系统免疫介导性黏膜皮肤疾病，死亡率颇高，可导致严重的眼表后遗症甚至双侧失明。在急性和慢性SJS/TEN中恢复眼表功能具有挑战性。对于SJS/TEN，局部或全身治疗选择有限。在急性SJS/TEN中进行早期诊断、及时的羊膜移植和积极的局部处理对于预防长期慢性眼部并发症是必要的。尽管急性护理的主要目标是挽救患者生命，但眼科医生应定期检查处于急性期的患者，在慢性期还应进行系统的眼科检查。在此，我们总结了关于SJS/TEN的流行病学、病因、病理、临床表现及治疗的实际知识。
