G Chaby1, C Maldini2, C Haddad1,3, B Lebrun-Vignes1,3, F Hemery4, S Ingen-Housz-Oro1,5, S Gonzalez-Chiappe2, P Wolkenstein1,5, O Chosidow1,5,6, A Mahr2, L Fardet1,5. 1. EA 7379 EpiDermE, Université Paris Est Créteil, France. 2. Department of Internal Medicine, Hôpital Saint Louis, AP-HP, Paris, France. 3. Regional Center of Pharmacovigilance, Hôpital Pitié-Salpêtrière, AP-HP, Paris, France. 4. Department of Medical Informatics, Hôpital Henri-Mondor, AP-HP, Créteil, France. 5. Department of Dermatology and Reference Center for Toxic Bullous Diseases, Hôpital Henri-Mondor, AP-HP, Créteil, France. 6. INSERM, Centre d'Investigation Clinique 1430, Créteil, France.
Abstract
BACKGROUND: Because of its rarity, the exact incidence of and mortality from epidermal necrolysis (Stevens-Johnson syndrome/toxic epidermal necrolysis) is difficult to establish and closely depends on the size and type of the data source. OBJECTIVES: To estimate the incidence of and mortality due to epidermal necrolysis in France over a 14-year period. METHODS: Data from four national databases were analysed. A capture-recapture analysis was performed. RESULTS: A total of 2635 incident cases of epidermal necrolysis were recorded in at least one of the four databases during the study period [males: 47·9%; median age: 52 (interquartile range 25-72) years]. On capture-recapture analysis, the estimated total number of cases was 5686, for an overall estimated annual incidence of 6·5 (95% confidence interval 4·1-8·9) cases per million inhabitants. The estimated annual incidence rates were 4·1 (0·3-7·9) cases per million inhabitants < 20 years of age, 3·9 (1·5-6·3) cases per million inhabitants aged 20-64 years and 13·7 (5·4-22·0) cases per million inhabitants ≥ 65 years of age. The estimated overall annual mortality rate from epidermal necrolysis was 0·9 (0·1-1·8) case per million inhabitants. It was 0·6 (0·1-1·5) case per million inhabitants aged 20-64 years and 2·8 (0·9-6·6) cases per million inhabitants ≥ 65 years of age (deaths in people < 20 years old were too rare to provide an accurate estimate). CONCLUSIONS: The annual incidence of epidermal necrolysis is higher than the one to five cases per million inhabitants usually reported. Such estimations could be helpful in establishing appropriate healthcare plans for people with epidermal necrolysis, in particular the need for specialized care units. What's already known about this topic? Few data are available regarding incidence of and mortality from epidermal necrolysis in the general population. Experts in epidermal necrolysis have recently proposed an annual incidence of one to five cases per million individuals. The overall mortality rate is usually reported to be between 10% and 20%. What does this study add? Using a four-source capture-recapture method and data from a 14-year period (2003-16), the annual incidence of and mortality from epidermal necrolysis were estimated to be 6·5 (95% confidence interval 4·1-8·9) and 0·9 (0·1-1·8) cases per million French inhabitants, respectively. Such estimations could be helpful in establishing appropriate healthcare plans, in particular the need for specialized care units.
BACKGROUND: Because of its rarity, the exact incidence of and mortality from epidermal necrolysis (Stevens-Johnson syndrome/toxic epidermal necrolysis) is difficult to establish and closely depends on the size and type of the data source. OBJECTIVES: To estimate the incidence of and mortality due to epidermal necrolysis in France over a 14-year period. METHODS: Data from four national databases were analysed. A capture-recapture analysis was performed. RESULTS: A total of 2635 incident cases of epidermal necrolysis were recorded in at least one of the four databases during the study period [males: 47·9%; median age: 52 (interquartile range 25-72) years]. On capture-recapture analysis, the estimated total number of cases was 5686, for an overall estimated annual incidence of 6·5 (95% confidence interval 4·1-8·9) cases per million inhabitants. The estimated annual incidence rates were 4·1 (0·3-7·9) cases per million inhabitants < 20 years of age, 3·9 (1·5-6·3) cases per million inhabitants aged 20-64 years and 13·7 (5·4-22·0) cases per million inhabitants ≥ 65 years of age. The estimated overall annual mortality rate from epidermal necrolysis was 0·9 (0·1-1·8) case per million inhabitants. It was 0·6 (0·1-1·5) case per million inhabitants aged 20-64 years and 2·8 (0·9-6·6) cases per million inhabitants ≥ 65 years of age (deaths in people < 20 years old were too rare to provide an accurate estimate). CONCLUSIONS: The annual incidence of epidermal necrolysis is higher than the one to five cases per million inhabitants usually reported. Such estimations could be helpful in establishing appropriate healthcare plans for people with epidermal necrolysis, in particular the need for specialized care units. What's already known about this topic? Few data are available regarding incidence of and mortality from epidermal necrolysis in the general population. Experts in epidermal necrolysis have recently proposed an annual incidence of one to five cases per million individuals. The overall mortality rate is usually reported to be between 10% and 20%. What does this study add? Using a four-source capture-recapture method and data from a 14-year period (2003-16), the annual incidence of and mortality from epidermal necrolysis were estimated to be 6·5 (95% confidence interval 4·1-8·9) and 0·9 (0·1-1·8) cases per million French inhabitants, respectively. Such estimations could be helpful in establishing appropriate healthcare plans, in particular the need for specialized care units.
Authors: Pauline O'Reilly; Pauline Meskell; Barbara Whelan; Catriona Kennedy; Bart Ramsay; Alice Coffey; Donal G Fortune; Sarah Walsh; Saskia Ingen-Housz-Oro; Christopher B Bunker; Donna M Wilson; Isabelle Delaunois; Liz Dore; Siobhan Howard; Sheila Ryan Journal: PLoS One Date: 2022-06-27 Impact factor: 3.752
Authors: D Thorel; S Ingen-Housz-Oro; G Royer; A Delcampe; N Bellon; C Bodemer; A Welfringer-Morin; D Bremond-Gignac; M P Robert; M Tauber; F Malecaze; O Dereure; V Daien; A Colin; C Bernier; C Couret; B Vabres; F Tetart; B Milpied; T Cornut; B Ben Said; C Burillon; N Cordel; L Beral; N de Prost; P Wolkenstein; M Muraine; J Gueudry Journal: Orphanet J Rare Dis Date: 2020-09-22 Impact factor: 4.123