Health-related quality of life and long-term sequelae in survivors of epidermal necrolysis: an observational study of 57 patients

Background Few studies have investigated the global burden of sequelae and health-related quality of life (HRQoL) for survivors of epidermal necrolysis (EN). Objectives To investigate the long-term HRQoL for survivors of EN using validated instruments. Methods We conducted a single-centre study that enrolled patients who were admitted for EN between 2010 and 2017. HRQoL was assessed via phone interview using the Short Form (SF)-36 questionnaire, Hospital Anxiety and Depression (HAD) scale, Impact of Event Scale-Revised, and general quality-of-life outcomes, including EN-specific sequelae. The primary outcome measure was the physical component summary (PCS) score of the SF-36. Results In total, 57 survivors of EN [19 (33%) with intensive care unit (ICU) admission] were interviewed via telephone at a median of 3·6 years (1·9-6·1) after hospital discharge. The median PCS score was 0·44 SDs below that of the age- and sex-matched reference population and was significantly lower for survivors of EN who were admitted to the ICU vs. those who were not [43·7 (28·7-49·3) vs. 51·2 (39·4-56·5), P = 0·042]. The proportion of patients with EN who had HAD-anxiety score ≥ 8 or HAD-depression score ≥ 5 was 54% and 21%, respectively. Physical and mental outcomes did not differ between patients with EN who were admitted to the ICU and survivors of septic shock. Reported EN-specific sequelae were cutaneous (77%), ocular (70%), psychological (60%), dental/oral (49%), genital (30%) and respiratory (18%), with median intensity on a visual analogue scale. Conclusions Our study confirms the major burden and long-term impact of EN on quality of life for survivors and emphasizes the need for prolonged close follow-up after the acute phase. What's already known about this topic? Long-term sequelae have been reported in 90% of survivors of epidermal necrolysis (EN). Few studies have investigated the global burden of sequelae and health-related quality of life (HRQoL) in survivors of EN. What does this study add? Survivors of EN, particularly those admitted to the intensive care unit, had poorer physical HRQoL than the French reference population but had comparable HRQoL to survivors of septic shock. Survivors of EN exhibited symptoms of anxiety, depression and post-traumatic stress syndrome. The most frequent sequelae were cutaneous, ocular and psychological, with visual analogue scale scores of 5/10 and 6/10. These results confirm the burden of EN on quality of life.