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. PflugfelderVirender S. Sangwan, Kimberly C. Sippel, Chie Sotozono, Bhaskar Srinivasan, Donald T H Tan, Radhika Tandon, Scheffer C G Tseng, Mayumi Ueta, James Chodosh

Research output: Contribution to journalReview article

48 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)2-19
Number of pages18
JournalOcular Surface
Volume14
Issue number1
DOIs
StatePublished - Jan 1 2016

Keywords

  • apoptosis
  • drug-induced disease
  • immune-mediated disease
  • keratinocyte death Stevens-Johnson Syndrome
  • toxic epidermal necrolysis

ASJC Scopus subject areas

  • Ophthalmology

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    Kohanim, S., Palioura, S., Saeed, H. N., Akpek, E. K., Amescua, G., Basu, S., Blomquist, P. H., Bouchard, C. S., Dart, J. K., Gai, X., Gomes, J. A. P., Gregory, D. G., Iyer, G., Jacobs, D. S., Johnson, A. J., Kinoshita, S., Mantagos, I. S., Mehta, J. S., Perez, V. L., ... Chodosh, J. (2016). Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis - A Comprehensive Review and Guide to Therapy. I. Systemic Disease. Ocular Surface, 14(1), 2-19. https://doi.org/10.1016/j.jtos.2015.10.002