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

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.

Individual‐ and hospital‐level factors associated with epidermal necrolysis mortality: a nationwide multilevel study, France, 2012–2016

“…In our study, the overall mortality rate was 16% when excluding patients with a possible diagnosis of EM; this is similar to the rate recently reported in a large North American study (15%) . As previously reported, the percentage of detached body surface area is a major risk factor for mortality, especially because of the risk of infections, which are more frequent in TEN than in SJS or SJS/TEN overlap…”

“…In most cases, EN is caused by drugs, via a type IV‐c hypersensitivity mechanism . Overall published mortality rates range from 15% to 30% . Individuals' characteristics, including the percentage of epidermal detachment, have been identified as predictors of mortality .…”

HLA‐associated antiepileptic drug‐induced cutaneous adverse reactions

“…Immediate drug cessation is generally recommended when cADRs develop and, in the cases of SJS/TEN and HSS/DRESS, hospitalisation for intensive treatment. 6 One of the leading classes of medications resulting in cADRs are the aromatic antiepileptic drugs (AEDs), four of which are commonly prescribed; carbamazepine, oxcarbazepine, lamotrigine and phenytoin 7 (Figure 1). Carbamazepine and lamotrigine are first-line treatments for epilepsy, one of the most common neurological disorders (affecting 3%-4% of the population).…”

Health‐related quality of life and long‐term sequelae in survivors of epidermal necrolysis: an observational study of 57 patients

“…In most cases EN is caused by drugs . Overall published mortality rates at the acute phase vary from 14·7% to 29·4% . Supportive care is the cornerstone of treatment.…”