Epidermal necrolysis sequelae: A cohort study on prevalence and risk factors

BACKGROUND: Epidermal necrolysis (EN), including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), is a rare but severe cutaneous reaction with a high risk of long-term sequelae. However, data on their frequency, severity and socio-professional impact remain limited. OBJECTIVE: To describe the prevalence and severity of EN sequelae, and their associations with acute-phase disease characteristics and long-term functional outcomes. METHODS: This single-centre retrospective study from 2015 to 2022 included all adult EN survivors with ≥ 3 months of follow-up. Sequelae were classified according to anatomical systems and were graded by severity. Associations between sequelae and clinical variables were analysed with multivariable analyses and unsupervised clustering. RESULTS: We included 129 patients. One or more sequelae were observed in 93.8% of patients. Cutaneous (76%) and ocular (71.3%) sequelae were the most frequent. Severe ocular sequelae were associated with TEN diagnosis (OR: 2.07, 95% CI: 1.19-3.71), number of mucous membranes involved (OR: 1.56, 95% CI: 1.05-2.44) and length of stay (OR: 1.01 per day, 95% CI: 1.00-1.01), cutaneous sequelae were associated with TEN diagnosis (OR: 1.84, 95% CI: 1.15-2.97), while severe psychological sequelae (29.4%) only correlated with prior psychiatric history (OR: 2.33, 95% CI: 1.35-3.97). Clustering analysis identified three patient profiles: mild or no sequelae, severe cutaneous and pulmonary sequelae, and severe ocular, oral and genital sequelae. Almost half of active patients (43/88, 48.9%) did not return to work, mainly due to psychological sequelae (p = 0.01). CONCLUSION: EN survivors frequently experience multiple and severe sequelae, resulting in significant burden. Long-term, multidisciplinary and personalized follow-up is essential to improve patients' quality of life.