Incidence of and mortality from epidermal necrolysis (Stevens–Johnson syndrome/toxic epidermal necrolysis) in France during 2003–16: a four‐source capture–recapture estimate

Abstract

Summary 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.

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement

“…Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN, or Lyell syndrome) are serious and rare diseases, most often drug-induced, and their incidence has recently been re-estimated at 6 cases/million/year in France [ 1 ]. SJS and TEN are characterized by widespread necrosis of the epidermis and the mucous membrane, sometimes complicated during the acute phase by multi-organ failure that can be fatal.…”

“…Owing to the lack of effective immunomodulatory treatment that can reduce mortality and muco-cutaneous scarring, the main treatment in the acute phase is symptomatic or supportive care [ 1 ]. So far, there has been no standardized protocol and no consensus in the literature.…”

Shaping specialist treatment of Stevens–Johnson syndrome/toxic epidermal necrolysis

“…Additionally, and perhaps more importantly, reporting of adverse incidents relating to drugs is culturally embedded in clinical practice, in hospitals and the community. For these reasons, publications such as the one in this issue of the BJD , describing the incidence of and mortality from Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), are welcomed for the robust nature of the primary data …”

“…More recent epidemiological studies suggested a higher incidence of 1–5 cases per million per year . Now, the study by Chaby et al ., using rigorous epidemiological methodology, suggests a still higher rate of 6·5 cases per million/year. Assuming that the aetiological factors leading to SJS/TEN in France are the same as in the U.K., then the up‐to‐date incidence predicts 436 cases occurring each year in the U.K. (current population: 67 million).…”

“…The recognition of a higher, and more costly, disease burden will have an impact on attempts to allocate resources for the establishment of regional specialist centres for the management of TEN. This aspiration, already ratified by NHS England, is endorsed by the highly impressive SJS/TEN mortality figures in France published in the article by Chaby et al . The lower‐than‐previously‐reported mortality data in this study compel the reader to infer that the French system, which concentrates expertise in the management of patients with SJS/TEN in centres across the country, is essential to improving outcomes in this disease.…”