Epidermal Necrolysis Tied to 34% Mortality in French Study

TOPLINE:

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.

METHODOLOGY:

• Researchers conducted a nationwide cohort study using the French Health System database that included 1221 adult patients (median age, 66 years; women, 56.3%) who were hospitalized with SJS, SJS and TEN overlap, and TEN between January 2013 and December 2022.
• The median length of hospital stay was 17 days.
• The primary outcome was the incidence of epidermal EN per million person-years; secondary outcomes included inhospital mortality and post-discharge mortality within 1 year.

TAKEAWAY:

• The overall incidence of EN was 2.6 cases per million person-years, with SJS at 1.0, SJS/TEN at 0.7, and TEN at 1.0 cases per million person-years.
• The mortality rate at 1 year for EN was 34%. The overall inhospital mortality rate was 19%, and the post-discharge mortality rate up to 1 year was 15%.
• Age, diagnosis of TEN, transfer to an intensive care unit, and comorbidities such as dementia, liver disease, and cancer were associated with higher inhospital mortality. Comorbidities including cancer, liver disease, and dementia and acute kidney injury and sepsis during hospitalization were associated with higher post-discharge mortality.
• Among 987 patients (80.3%) who survived the acute stage, 25% developed at least one new comorbidity within a year, with higher rates of ophthalmologic, mood, and respiratory disorders observed in those with TEN compared with SJS or SJS/TEN.

IN PRACTICE:

“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.

SOURCE:

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.

LIMITATIONS:

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.

DISCLOSURES:

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

This article was created using several editorial tools, including AI, as part of the process. Human editors reviewed this content before publication.