Faculty Collaboration Database

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Multicenter Retrospective Study of 377 Adult Patients from the United States. J Invest Dermatol 2018 Nov;138(11):2315-2321

Date

05/15/2018

Pubmed ID

29758282

DOI

10.1016/j.jid.2018.04.027

Scopus ID

2-s2.0-85050476271 (requires institutional sign-in at Scopus site)   105 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.

Author List

Micheletti RG, Chiesa-Fuxench Z, Noe MH, Stephen S, Aleshin M, Agarwal A, Boggs J, Cardones AR, Chen JK, Cotliar J, Davis MDP, Dominguez A, Fox LP, Gordon S, Hamrick R, Ho B, Hughey LC, Jones LM, Kaffenberger BH, Kindley K, Kroshinsky D, Kwong BY, Miller DD, Mostaghimi A, Musiek A, Ortega-Loayza AG, Patel R, Posligua A, Rani M, Saluja S, Sharon VR, Shinkai K, John JS, Strickland N, Summers EM, Sun N, Wanat KA, Wetter DA, Worswick S, Yang C, Margolis DJ, Gelfand JM, Rosenbach M

Author

Karolyn A. Wanat MD Chair, Professor in the Dermatology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adrenal Cortex Hormones
Adult
Aged
Cohort Studies
Critical Care
Female
Humans
Immunoglobulins, Intravenous
Male
Middle Aged
Retrospective Studies
Stevens-Johnson Syndrome
Sulfamethoxazole
Survival Analysis
Trimethoprim
United States