Epidermal necrolysis (EN) -- which includes Stevens-Johnson syndrome (SJS), toxic EN (TEN), and SJS and TEN overlap -- had an incidence of 2.6 cases per million person-years in a French study of adults.

"We found that despite EN being a rare condition, it is associated with high inhospital and post-discharge mortality and a high burden of sequelae," the authors wrote. More studies "are warranted to replicate these findings, to compare EN post-discharge mortality with other conditions, and to construct models to estimate long-term outcomes and sequelae in patients with EN," they added.

The study was led by Thomas Bettuzzi, MD, MPH, Service de Dermatologie, Hôpital Henri Mondor, Assistance Publique-Hôpitaux de Paris, Paris, France, and was published online on October 2 in JAMA Dermatology.

The selection criteria for inclusion of patients with SJS may have led to an underestimation of the incidence and an overestimation of the mortality. Important variables such as heart rate, urea, or bicarbonate levels were not accessible, which could have affected the analysis. Furthermore, the study lacked data on racial and ethnic disparities, causes of death, and systemic pharmacologic treatments, and the results may not be generalizable to other countries.

The study was supported by a grant from Filléres de Santé Maladies Rares. No relevant conflicts of interest were disclosed by the authors.