{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T12:24:33Z","timestamp":1788006273323,"version":"build-2784847793"},"reference-count":27,"publisher":"American Medical Association (AMA)","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAMA Dermatol"],"published-print":{"date-parts":[[2024,12,1]]},"abstract":"<jats:sec id=\"ab-doi240041-4\"><jats:title>Importance<\/jats:title><jats:p>The incidence of epidermal necrolysis (EN), including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), varies across studies. While in-hospital mortality rates range from 15% to 20%, contributors to long-term mortality have been rarely evaluated and remain unknown.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-5\"><jats:title>Objective<\/jats:title><jats:p>To assess the incidence of and compare factors associated with in-hospital mortality and postdischarge mortality and sequelae among patients with EN.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-6\"><jats:title>Design, Setting, and Participants<\/jats:title><jats:p>This cohort study used French Health System data from January 1, 2013, to December 31, 2022, and included all adult patients (aged \u226518 years) with EN identified using <jats:italic>International Statistical Classification of Diseases, Tenth Revision<\/jats:italic> codes combined with a validated algorithm.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-7\"><jats:title>Exposure<\/jats:title><jats:p>Epidermal necrolysis.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-8\"><jats:title>Main Outcomes and Measures<\/jats:title><jats:p>Incidence, in-hospital mortality, postdischarge mortality, and sequelae were assessed as main outcomes. Factors associated with mortality were assessed using a multivariable Cox proportional hazards model.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-9\"><jats:title>Results<\/jats:title><jats:p>A total of 1221 adult patients with EN (median [IQR] age, 66 [49-79] years; 688 females [56.3%]) were included. Incidence was 2.6 (95% CI, 2.5-2.7) cases per million person-years. The in-hospital mortality rate was 19% (95% CI, 17%-21%) and postdischarge mortality rate, 15% (95% CI, 13%-17%) for an overall mortality of 34% (95% CI, 31%-36%). In multivariable analysis, factors associated with in-hospital mortality were age (adjusted hazard ratio [AHR], 1.03 per year of age; 95% CI, 1.02-1.04 per year of age), history of cancer (AHR, 2.04; 95% CI, 1.53-2.72), dementia (AHR, 1.85; 95% CI, 1.12-3.07), liver disease (AHR, 1.81; 95% CI, 1.24-2.64), and EN severity (TEN vs SJS: AHR, 2.14; 95% CI, 1.49-3.07). Cancer, liver disease, and dementia remained associated with postdischarge mortality (AHR, 3.26 [95% CI, 2.35-4.53], 1.86 [95% CI, 1.11-3.13], and 1.95 [95% CI, 1.11-3.43], respectively). Conversely, EN initial severity was not associated with mortality after hospital discharge (TEN vs SJS: AHR, 0.95; 95% CI, 0.60-1.47), but acute complications remained associated (AHR, 2.14 [95% CI, 1.26-3.63] and 2.44 [95% CI, 1.42-4.18] for acute kidney injury and sepsis, respectively). The main sequelae were ophthalmologic and mood disorders.<\/jats:p><\/jats:sec><jats:sec id=\"ab-doi240041-10\"><jats:title>Conclusion<\/jats:title><jats:p>The findings of this cohort study suggest that although EN is a rare condition, it is associated with high rates of in-hospital and postdischarge mortality among patients who are older and have comorbid conditions. However, in contrast with in-hospital mortality, postdischarge mortality is not associated with EN initial severity but with acute in-hospital complications (eg, acute kidney injury and sepsis). Future studies are needed to construct models to estimate long-term outcomes and sequelae in patients with EN.<\/jats:p><\/jats:sec>","DOI":"10.1001\/jamadermatol.2024.3575","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T15:36:53Z","timestamp":1727883413000},"page":"1288","source":"Crossref","is-referenced-by-count":10,"title":["Incidence, In-Hospital and Long-Term Mortality, and Sequelae of Epidermal Necrolysis in Adults"],"prefix":"10.1001","volume":"160","author":[{"given":"Thomas","family":"Bettuzzi","sequence":"first","affiliation":[{"name":"Service de Dermatologie, H\u00f4pital Henri Mondor, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Cr\u00e9teil, France"},{"name":"Epidemiology in Dermatology and Evaluation of Therapeutics (EpiDermE), Universit\u00e9 Paris Est Cr\u00e9teil (UPEC), Cr\u00e9teil, France"},{"name":"Centre de R\u00e9f\u00e9rence des Dermatoses Bulleuses Toxiques et Toxidermies Graves, TOXIBUL, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9n\u00e9dicte","family":"Lebrun-Vignes","sequence":"additional","affiliation":[{"name":"Epidemiology in Dermatology and Evaluation of Therapeutics (EpiDermE), Universit\u00e9 Paris Est Cr\u00e9teil (UPEC), Cr\u00e9teil, France"},{"name":"Centre de R\u00e9f\u00e9rence des Dermatoses Bulleuses Toxiques et Toxidermies Graves, TOXIBUL, Cr\u00e9teil, France"},{"name":"Service de Pharmacologie M\u00e9dicale, Centre R\u00e9gional de Pharmacovigilance, H\u00f4pital Piti\u00e9 Salp\u00eatri\u00e8re, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saskia","family":"Ingen-Housz-Oro","sequence":"additional","affiliation":[{"name":"Service de Dermatologie, H\u00f4pital Henri Mondor, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Cr\u00e9teil, France"},{"name":"Epidemiology in Dermatology and Evaluation of Therapeutics (EpiDermE), Universit\u00e9 Paris Est Cr\u00e9teil (UPEC), Cr\u00e9teil, France"},{"name":"Centre de R\u00e9f\u00e9rence des Dermatoses Bulleuses Toxiques et Toxidermies Graves, TOXIBUL, Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilie","family":"Sbidian","sequence":"additional","affiliation":[{"name":"Service de Dermatologie, H\u00f4pital Henri Mondor, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Cr\u00e9teil, France"},{"name":"Epidemiology in Dermatology and Evaluation of Therapeutics (EpiDermE), Universit\u00e9 Paris Est Cr\u00e9teil (UPEC), Cr\u00e9teil, France"},{"name":"Centre de R\u00e9f\u00e9rence des Dermatoses Bulleuses Toxiques et Toxidermies Graves, TOXIBUL, Cr\u00e9teil, France"},{"name":"Centre d\u2019Investigation Clinique 1430, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), Cr\u00e9teil, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10","reference":[{"issue":"3","key":"doi240041r1","doi-asserted-by":"publisher","first-page":"618","DOI":"10.1111\/bjd.18424","article-title":"Incidence of and mortality from epidermal necrolysis (Stevens-Johnson syndrome\/toxic epidermal necrolysis) in France during 2003-16: a four-source capture-recapture estimate.","volume":"182","author":"Chaby","year":"2020","journal-title":"Br J Dermatol"},{"issue":"1","key":"doi240041r2","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1001\/archderm.1990.01670250043005","article-title":"Toxic epidermal necrolysis (Lyell syndrome). 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