title: Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis--A Comprehensive Review and Guide to Therapy. I. Systemic Disease.
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.
authors.affiliation: Vanderbilt Eye Institute, Vanderbilt University School of Medicine, Nashville, TN.
authors.forename: Sahar
authors.initials: S
authors.lastname: Kohanim
authors.affiliation: Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL.
authors.forename: Sotiria
authors.initials: S
authors.lastname: Palioura
authors.affiliation: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
authors.forename: Hajirah N
authors.initials: HN
authors.lastname: Saeed
authors.affiliation: The Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Esen K
authors.initials: EK
authors.lastname: Akpek
authors.affiliation: Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL.
authors.forename: Guillermo
authors.initials: G
authors.lastname: Amescua
authors.affiliation: LV Prasad Eye Institute, Hyderabad, India.
authors.forename: Sayan
authors.initials: S
authors.lastname: Basu
authors.affiliation: University of Texas Southwestern Medical Center, Dallas, TX.
authors.forename: Preston H
authors.initials: PH
authors.lastname: Blomquist
authors.affiliation: Loyola University, Chicago, IL.
authors.forename: Charles S
authors.initials: CS
authors.lastname: Bouchard
authors.affiliation: Moorfields Eye Hospital, NHS Foundation Trust, London, UK.
authors.forename: John K
authors.initials: JK
authors.lastname: Dart
authors.affiliation: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
authors.forename: Xiaowu
authors.initials: X
authors.lastname: Gai
authors.affiliation: Federal University of São Paulo, Brazil.
authors.forename: José A P
authors.initials: JA
authors.lastname: Gomes
authors.affiliation: Rocky Mountain Lions Eye Institute, University of Colorado School of Medicine, Aurora, CO.
authors.forename: Darren G
authors.initials: DG
authors.lastname: Gregory
authors.affiliation: Dr G Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Sankara Nethralaya, India.
authors.forename: Geetha
authors.initials: G
authors.lastname: Iyer
authors.affiliation: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA; Boston Foundation for Sight, Boston, MA.
authors.forename: Deborah S
authors.initials: DS
authors.lastname: Jacobs
authors.affiliation: United States Army Institute of Surgical Research, San Antonio, TX.
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Johnson
authors.affiliation: Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Shigeru
authors.initials: S
authors.lastname: Kinoshita
authors.affiliation: Boston Children's Hospital, Harvard Medical School, Boston, MA.
authors.forename: Iason S
authors.initials: IS
authors.lastname: Mantagos
authors.affiliation: Singapore National Eye Centre, Singapore Eye Research Institute, Singapore.
authors.forename: Jodhbir S
authors.initials: JS
authors.lastname: Mehta
authors.affiliation: Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, FL.
authors.forename: Victor L
authors.initials: VL
authors.lastname: Perez
authors.affiliation: Cullen Eye Institute, Baylor College of Medicine, Houston, TX.
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Pflugfelder
authors.affiliation: LV Prasad Eye Institute, Hyderabad, India.
authors.forename: Virender S
authors.initials: VS
authors.lastname: Sangwan
authors.affiliation: Weill Cornell Medical College, New York, NY.
authors.forename: Kimberly C
authors.initials: KC
authors.lastname: Sippel
authors.affiliation: Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Chie
authors.initials: C
authors.lastname: Sotozono
authors.affiliation: Dr G Sitalakshmi Memorial Clinic for Ocular Surface Disorders, Sankara Nethralaya, India.
authors.forename: Bhaskar
authors.initials: B
authors.lastname: Srinivasan
authors.affiliation: Singapore National Eye Centre, Singapore Eye Research Institute, Singapore.
authors.forename: Donald T H
authors.initials: DT
authors.lastname: Tan
authors.affiliation: Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Delhi, India.
authors.forename: Radhika
authors.initials: R
authors.lastname: Tandon
authors.affiliation: Ocular Surface Center, Ocular Surface Research & Education Foundation, Miami, FL.
authors.forename: Scheffer C G
authors.initials: SC
authors.lastname: Tseng
authors.affiliation: Kyoto Prefectural University of Medicine, Kyoto, Japan.
authors.forename: Mayumi
authors.initials: M
authors.lastname: Ueta
authors.affiliation: Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA. Electronic address: James_Chodosh@meei.harvard.edu.
authors.forename: James
authors.initials: J
authors.lastname: Chodosh
mesh_terms: D019468:Disease Management; D006801:Humans; D009885:Ophthalmology / Q000379:methods*; D017410:Practice Guidelines as Topic; D013262:Stevens-Johnson Syndrome / Q000628:therapy*
keywords: apoptosis; drug-induced disease; immune-mediated disease; keratinocyte death Stevens-Johnson Syndrome; toxic epidermal necrolysis