Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis: a multicenter retrospective study of 377 adult patients from the United States.

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. 377 adult patients with SJS/TEN between 1/1/2000 and 6/1/2015 were entered, including 69.0% from 2010 onward. The most frequent cause of SJS/TEN was medication reaction (89.7%), most often trimethoprim / sulfamethoxazole (27.2%). The majority of patients were managed in an intensive care (27.2%) or burn unit (41.0%). Most received pharmacologic therapy (70.7%) versus supportive care alone (29.3%)-typically corticosteroids (42.5%), IVIG (35.3%), or both therapies (20.3%). Based on Day 1 SCORTEN predicted mortality, 78 in-hospital deaths were expected (21%), while the observed mortality of 54 patients (14.7%) was significantly lower (SMR 0.70; CI 0.58, 0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and IVIG (0.52; CI 0.21, 0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, while substantial, was significantly lower than predicted. While the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and IVIG, among other therapies, may warrant further study.