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

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.

To describe the prevalence and severity of EN sequelae, and their associations with acute-phase disease characteristics and long-term functional outcomes.

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.

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).

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.