Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis - A Comprehensive Review and Guide to Therapy. I. Systemic Disease

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• 作者：Sahar Kohanim ; MD¹ ; ^&lowast ; ; Sotiria Palioura ; MD ; PhD² ; ^&lowast ; ; Hajirah N. Saeed ; MD³ ; ^&lowast ; ; Esen K. Akpek ; MD⁴ ; Guillermo Amescua ; MD² ; Sayan Basu ; MBBS ; MS⁵ ; Preston H. Blomquist ; MD⁶ ; Charles S. Bouchard ; MD⁷ ; John K. Dart ; DM ; FRCOphth⁸ ; Xiaowu Gai ; PhD³ ; José ; A.P. Gomes ; MD⁹ ; Darren G. Gregory ; MD¹⁰ ; Geetha Iyer ; MD ; FRCS Glasgow¹¹ ; Deborah S. Jacobs ; MD³ ; ¹² ; Anthony J. Johnson ; MD¹³ ; Shigeru Kinoshita ; MD ; PhD¹⁴ ; Iason S. Mantagos ; MD¹⁵ ; Jodhbir S. Mehta ; MBBS¹⁶ ; Victor L. Perez ; MD² ; Stephen C. Pflugfelder ; MD¹⁷ ; Virender S. Sangwan ; MBBS ; MS⁵ ; Kimberly C. Sippel ; MD¹⁸ ; Chie Sotozono ; MD ; PhD¹⁴ ; Bhaskar Srinivasan ; MD ; MS¹¹ ; Donald T.H. Tan ; FRCS ; FRCOphth ; FAMS¹⁶ ; Radhika Tandon ; MD ; FRCOphth ; FRCSEd¹⁹ ; Scheffer C.G. Tseng ; MD ; PhD²⁰ ; Mayumi Ueta ; MD ; PhD¹⁴ ; James Chodosh ; MD ; MPH³ ; ^{James_Chodosh@meei.harvard.edu" class="auth_mail" title="E-mail the corresponding author}
• 关键词：apoptosis ; drug-induced disease ; immune-mediated disease ; keratinocyte death Stevens-Johnson Syndrome ; toxic epidermal necrolysis
• 刊名：The Ocular Surface
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：14
• 期：1
• 页码：2-19
• 全文大小：1209 K

文摘

The intent of this review is to comprehensively appraise the state of the art with regard to Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), with particular attention to the ocular surface complications and their management. SJS and TEN represent two ends of a spectrum of immune-mediated, dermatobullous disease, characterized in the acute phase by a febrile illness followed by skin and mucous membrane necrosis and detachment. The widespread keratinocyte death seen in SJS/TEN is rapid and irreversible, and even with early and aggressive intervention, morbidity is severe and mortality not uncommon. We have divided this review into two parts. Part I summarizes the epidemiology and immunopathogenesis of SJS/TEN and discusses systemic therapy and its possible benefits. We hope this review will help the ophthalmologist better understand the mechanisms of disease in SJS/TEN and enhance their care of patients with this complex and often debilitating disease. Part II (April 2016 issue) will focus on ophthalmic manifestations.