Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States

Robert G. Micheletti, Zelma Chiesa-Fuxench, Megan H. Noe, Sasha Stephen, Maria Aleshin, Ashwin Agarwal, Jennifer Boggs, Adela R. Cardones, Jennifer K. Chen, Jonathan Cotliar, Mark D.P. Davis, Arturo Dominguez, Lindy P. Fox, Shayna Gordon, Ronald Hamrick, Baran Ho, Lauren C. Hughey, Larry M. Jones, Benjamin H. Kaffenberger, Kimball KindleyDaniela Kroshinsky, Bernice Y. Kwong, Daniel D. Miller, Arash Mostaghimi, Amy Musiek, Alex G. Ortega-Loayza, Raj Patel, Alba Posligua, Monica Rani, Sandeep Saluja, Victoria R. Sharon, Kanade Shinkai, Jessica St. John, Nicole Strickland, Natalie Sun, Karolyn A. Wanat, David A. Wetter, Scott Worswick, Caroline Yang, David J. Margolis, Joel M. Gelfand, Misha Rosenbach

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)—typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58–0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21–0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.

Original languageEnglish (US)
Pages (from-to)2315-2321
Number of pages7
JournalJournal of Investigative Dermatology
Volume138
Issue number11
DOIs
StatePublished - Nov 2018

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Dermatology
  • Cell Biology

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    Micheletti, R. G., Chiesa-Fuxench, Z., Noe, M. H., Stephen, S., Aleshin, M., Agarwal, A., Boggs, J., Cardones, A. R., Chen, J. K., Cotliar, J., Davis, M. D. P., Dominguez, A., Fox, L. P., Gordon, S., Hamrick, R., Ho, B., Hughey, L. C., Jones, L. M., Kaffenberger, B. H., ... Rosenbach, M. (2018). Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States. Journal of Investigative Dermatology, 138(11), 2315-2321. https://doi.org/10.1016/j.jid.2018.04.027