A comprehensive cohort study of epidermal necrolysis in France revealed important findings regarding incidence, mortality rates, and associated risk factors.
The study, published in JAMA Dermatology, analyzed data from 1,221 adult patients with Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap between 2013 and 2022.
The report found epidermal necrolysis (EN) occurs at a rate of 2.6 cases per million person-years and has an overall 1-year mortality rate of 34%. At 19%, the in-hospital mortality rate is slightly higher than the post-discharge mortality rate of 15%.
The in-hospital mortality rates varied by diagnosis with 14% for SJS, 11% for SJS/TEN overlap, and 30% for TEN. Multivariable analysis identified several factors associated with in-hospital mortality including age, intensive care unit transfer, cancer, dementia, and liver disease. The post-discharge mortality rates were 18% for SJS, 15% for SJS/TEN overlap, and 11% for TEN. Notably, initial EN severity was not associated with postdischarge mortality. Instead, factors were associated with post-discharge mortality included cancer, liver disease, dementia, acute kidney injury during hospitalization, and sepsis during hospitalization.
Among the 987 patients who survived the acute stage of EN, 25% developed at least one new comorbidity within a year after discharge. Ophthalmologic disorders were more common after TEN compared to SJS/TEN overlap, and SJS. Similarly, mood disorders were more frequent following TEN than SJS/TEN overlap, and SJS. The study also reported new-onset respiratory disorders in 7.7% of TEN survivors, compared to 5.9% in SJS/TEN overlap, and 3.3% in SJS survivors.
For 109 of 174 patients who died after discharge, the primary diagnosis at the time of death was available, with 24.8% dying of cancer, 15.6% dying of infectious diseases, and 13.8% dying due to dermatologic disease.
The study, led by Thomas Bettuzzi, MD, MPH, from the Department of Dermatology at Henri Mondor Hospital in Créteil, France, used French Health System data to identify EN cases using International Statistical Classification of Diseases, Tenth Revision (ICD-10) codes combined with a validated algorithm.
The authors acknowledged several limitations of the study including, potential underestimation of SJS incidence due to stringent inclusion criteria, lack of data on racial and ethnic characteristics, absence of information on systemic pharmacologic treatments, possible overestimation of mortality rates due to selection criteria, and limited generalizability to populations outside of France
The authors declared having no competing interests.
