Background
Epidermal necrolysis (EN), comprising Stevens Johnson syndrome and toxic epidermal necrolysis is a rare and severe blistering reaction, mainly induced by drugs. Differences have been discussed between pediatric and adult patients regarding incidence, causes, and outcomes, but only based on a limited number of patients, in small case-series.
Objectives
The objective was to directly compare incidence, causes and prognosis between adult and pediatric EN patients.
Methods
We used the French Health System Database from January 1st 2013 to December 31st 2022. We included adult and pediatric patients hospitalized for EN using the international classification of diseases, 10th revision codes combined with validated algorithms. The outcomes were incidence of EN; presence of a suspected drug before EN onset, defined by a new drug dispensation from 5 to 56 days before hospitalization; and in-hospital mortality. To estimate the association between pediatric EN and the presence of a suspected drug, we computed a multivariable logistic regression with odd ratios (OR). To estimate the association with mortality, we computed a multivariable Cox proportional hazard ratio (HR) model.
Results
A total of 1440 EN patients were included, with 799 (55.5%) females, comprising 219 children and 1221 adults. Among children, EN incidence was 1.5 cases (95%CI: 1.3-1.7) per million people year compared with 2.6 cases (95%CI: 2.5-2.7) in adults. Moreover, children had less chances to have a culprit drug before EN onset (93/219 (42.5%) versus 829/1221 (67.9%)), with an adjusted OR of 0.43 (95%CI: 0.32-0.59), p < 0.0001, together with a better prognosis, with death rates of 1.4% (95%CI: 0.4%-3.7%) in pediatric patients compared with 19.4% (95%CI: 17.3%-21.7%) in adult patients, and an adjusted HR of 0.12 (95%CI: 0.04-0.38), p = 0.0003 for in-hospital mortality.
Conclusion
Pediatric EN seems to be rarer, with less chances to be caused by drugs, together with a better prognosis than adult EN.
- Epidermal necrolysis, comprising Stevens-Johnson syndrome, toxic epidermal necrolysis and overlap forms, is a rare disease.
- Differences have been postulated between pediatric and adult patients but evidence remain scarce, only based on a limited number of patients, in small case-series.
In this study, pediatric epidermal necrolysis was characterized by a lower incidence than adult epidermal necrolysis.
Moreover, pediatric patients were less likely to be administered a culprit drug before epidermal necrolysis onset, and presented a better prognosis than adults.
These results suggest the existence of different underlying pathophysiological mechanisms and clinical particularities between adult and pediatric patients.

Comparison of incidence, causes and prognosis of adult and pediatric epidermal necrolysis: a population-based study in France
Thomas Bettuzzi, Anne Welfringer-Morin, Saskia Ingen-Housz-Oro, Pauline Bataille, Bénédicte Lebrun-Vignes, Christine Bodemer, Emilie Sbidian
British Journal of Dermatology, ljae240, doi:10.1093/bjd/ljae240
Published: 08 June 2024