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

Sahar Kohanim; Sotiria Palioura; Hajirah N Saeed; Esen K Akpek; Guillermo Amescua; Sayan Basu; Preston H Blomquist; Charles S Bouchard; John K Dart; Xiaowu Gai; José A P Gomes; Darren G Gregory; Geetha Iyer; Deborah S Jacobs; Anthony J Johnson; Shigeru Kinoshita; Iason S Mantagos; Jodhbir S Mehta; Victor L Perez; Stephen C Pflugfelder; Virender S Sangwan; Kimberly C Sippel; Chie Sotozono; Bhaskar Srinivasan; Donald T H Tan; Radhika Tandon; Scheffer C G Tseng; Mayumi Ueta; James Chodosh

doi:10.1016/j.jtos.2015.10.002

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

Ocul Surf. 2016 Jan;14(1):2-19. doi: 10.1016/j.jtos.2015.10.002. Epub 2015 Nov 5.

Authors

Sahar Kohanim¹, Sotiria Palioura², Hajirah N Saeed³, Esen K Akpek⁴, Guillermo Amescua², Sayan Basu⁵, Preston H Blomquist⁶, Charles S Bouchard⁷, John K Dart⁸, Xiaowu Gai³, José A P Gomes⁹, Darren G Gregory¹⁰, Geetha Iyer¹¹, Deborah S Jacobs¹², Anthony J Johnson¹³, Shigeru Kinoshita¹⁴, Iason S Mantagos¹⁵, Jodhbir S Mehta¹⁶, Victor L Perez², Stephen C Pflugfelder¹⁷, Virender S Sangwan⁵, Kimberly C Sippel¹⁸, Chie Sotozono¹⁴, Bhaskar Srinivasan¹¹, Donald T H Tan¹⁶, Radhika Tandon¹⁹, Scheffer C G Tseng²⁰, Mayumi Ueta¹⁴, James Chodosh²¹

Affiliations

¹ Vanderbilt Eye Institute, Vanderbilt University School of Medicine, Nashville, TN.
² Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL.
³ Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
⁴ The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD.
⁵ LV Prasad Eye Institute, Hyderabad, India.
⁶ University of Texas Southwestern Medical Center, Dallas, TX.
⁷ Loyola University, Chicago, IL.
⁸ Moorfields Eye Hospital, NHS Foundation Trust, London, UK.
⁹ Federal University of São Paulo, Brazil.
¹⁰ Rocky Mountain Lions Eye Institute, University of Colorado School of Medicine, Aurora, CO.
¹¹ Dr G Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Sankara Nethralaya, India.
¹² Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA; Boston Foundation for Sight, Boston, MA.
¹³ United States Army Institute of Surgical Research, San Antonio, TX.
¹⁴ Kyoto Prefectural University of Medicine, Kyoto, Japan.
¹⁵ Boston Children's Hospital, Harvard Medical School, Boston, MA.
¹⁶ Singapore National Eye Centre, Singapore Eye Research Institute, Singapore.
¹⁷ Cullen Eye Institute, Baylor College of Medicine, Houston, TX.
¹⁸ Weill Cornell Medical College, New York, NY.
¹⁹ Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Delhi, India.
²⁰ Ocular Surface Center, Ocular Surface Research & Education Foundation, Miami, FL.
²¹ Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA. Electronic address: James_Chodosh@meei.harvard.edu.

PMID: 26549248
DOI: 10.1016/j.jtos.2015.10.002

Abstract

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.

Keywords: apoptosis; drug-induced disease; immune-mediated disease; keratinocyte death Stevens-Johnson Syndrome; toxic epidermal necrolysis.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Disease Management*
Humans
Ophthalmology / methods*
Practice Guidelines as Topic*
Stevens-Johnson Syndrome / therapy*