Comparison of incidence, causes and prognosis of adult and paediatric epidermal necrolysis: a French population-based study

Abstract

Abstract Background Epidermal necrolysis (EN), comprising Stevens–Johnson syndrome and toxic EN, is a rare and severe blistering reaction, mainly induced by drugs. Differences between paediatric and adult patients regarding incidence, causes and outcomes have been discussed but are based on a limited number of patients from small case series. Objectives To directly compare the incidence, cause and prognosis of adult and paediatric EN. Methods We used data from the French Health System Database (1 January 2013–31 December 2022). We identified adult and paediatric patients hospitalized for EN using the International Classification of Diseases, 10th Revision codes combined with validated algorithms. Outcomes were the incidence of EN; the presence of a suspected drug before EN onset (defined as a new drug dispensation from 5 to 56 days prehospitalization); and in-hospital mortality. To estimate the association between paediatric EN and the presence of a suspect drug, we computed a multivariable logistic regression with odd ratios (ORs). To estimate the association with mortality, we computed a multivariable Cox proportional hazard ratio (HR) model. Results A total of 1440 patients [799 (55.5%) female] with EN were included, comprising 219 children and 1221 adults. Among children, the incidence of EN was 1.5 cases [95% confidence interval (CI) 1.3–1.7] per 1 million person-years vs. 2.6 cases (95% CI 2.5–2.7) in adults. Moreover, children had less chance of being given a culprit drug before the onset of EN [n = 93/219 (42.5%) vs. n = 829/1221 (67.9%)], with an adjusted OR of 0.43 (95% CI 0.32–0.59; P < 0.001), together with a better prognosis: the mortality rate in paediatric patients was 1.4% (95% CI 0.4–3.7) vs. 19.4% (95% CI 17.3–21.7) in adults. The adjusted HR for in-hospital mortality in children was 0.12 (95% CI 0.04–0.38; P < 0.001). Conclusions Paediatric EN appears to be rarer, with less chance of being caused by drugs and has a better prognosis than adult EN. These results suggest the existence of different underlying pathophysiological mechanisms and clinical particularities between adult and paediatric patients with EN.