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J Invest Dermatol. 2018 Nov;138(11):2315-2321. doi: 10.1016/j.jid.2018.04.027. Epub 2018 Jul 27.

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

Author information

1
Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
2
Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
3
Division of Dermatology, University of California–Los Angeles, Los Angeles, California, USA.
4
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
5
Department of Dermatology, Duke University, Durham, North Carolina, USA.
6
Department of Dermatology, Stanford Hospital and Clinics, Redwood City, California, USA.
7
Science 37, Los Angeles, California, USA.
8
Department of Dermatology, University of Texas, Southwestern, Dallas, Texas, USA.
9
Department of Dermatology, University of California–San Francisco, San Francisco, California, USA.
10
Division of Dermatology, Washington University School of Medicine, St. Louis, Missouri, USA.
11
Department of Dermatology, University of Minnesota, Minneapolis, Minnesota, USA.
12
Department of Dermatology, University of California–Davis, Davis, California, USA.
13
Department of Dermatology, University of Alabama, Birmingham, Birmingham, Alabama, USA.
14
Department of Surgery, Ohio State University College of Medicine, Columbus, Ohio, USA.
15
Division of Dermatology, Ohio State University College of Medicine, Columbus, Ohio, USA.
16
Department of Dermatology, University of Virginia, Charlottesville, Virginia, USA.
17
Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA.
18
Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA.
19
Department of Dermatology, Oregon Health & Science University, Portland, Oregon, USA.
20
True Dermatology, Alabaster, Alabama, USA.
21
Department of Dermatology, State University of New York–Buffalo, Buffalo, New York, USA.
22
Dermatology and Aesthetics, Chicago, Illinois, USA.
23
Department of Dermatology, University of Utah, Salt Lake City, Utah, USA.
24
Department of Dermatology, Hofstra Northwell School of Medicine, New Hyde Park, New York, USA.
25
Department of Dermatology, Harvard University, Boston, Massachusetts, USA.
26
Department of Dermatology, University of North Carolina, Chapel Hill, Chapel Hill, North Carolina, USA.
27
Department of Dermatology, University of Iowa, Iowa City, Iowa, USA.
28
Department of Dermatology, Brown University, Providence, Rhode Island, USA

Erratum in

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.

PMID:
29758282
DOI:
10.1016/j.jid.2018.04.027
[Indexed for MEDLINE]
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