成年人的发病率，内科和长期死亡率以及表皮坏死的后遗症

表皮坏死（EN）的发生率包括史蒂文斯 - 约翰逊综合征（SJS）和有毒表皮坏死溶解（十），随着研究的变化。虽然院内死亡率从15％到20％不等，但很少评估长期死亡率的贡献者，并尚不清楚。评估与院内死亡率和入院后死亡率和后期死亡率和后遗症相关的因素的发生率和后遗症的患者中的EN.同龄人的同类研究使用了2013年1月1日，以及202年12月31日的法国卫生系统，并纳入了2022年12月31日，所有患者（所有人），所有患者（所有人）（所有人）（所有成人）（所有人），所有患者（所有人），所有患者（所有人）（所有人）（所有人），所有患者（所有人），所有患者均使用了2022年，所有患者（所有人），所有患者均使用。疾病的统计分类，第十个修订代码与经过验证的算法。表皮坏死溶解。处于主要成果中，评估了疾病，院内死亡率，住院后死亡率和后遗症。使用多变量COX比例危害模型评估了与死亡率相关的因素。总共包括1221例EN（中位[IQR]年龄，66 [49-79]年； 688名雌性[56.3％]）。发病率为2.6（95％CI，2.5-2.7），每百万人年。院内死亡率为19％（95％CI，17％-21％）和放电后死亡率，总死亡率为34％（95％CI，31％-36％），15％（95％CI，13％-17％）。 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）和严重性（十vs SJS：AHR，2.14; 95％CI，1.49-3.07）。癌症，肝病和痴呆症仍与入院死亡率有关（AHR，3.26 [95％CI，2.35-4.53]，1.86 [95％CI，1.11-3.13]和1.95 [95％CI，1.11-11-3.43]）。 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).主要后遗症是眼科和情绪障碍。这项队列研究的发现表明，尽管EN是一种罕见的疾病，但它与年龄较大且患有合并症的患者的院内和放电后死亡率高有关。但是，与院内死亡率相反，放病后死亡率与EN初始严重程度无关，而与急性院内并发症（例如，急性肾脏损伤和败血症）有关。需要未来的研究来构建模型以估计EN患者的长期结局和后遗症。

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.To assess the incidence of and compare factors associated with in-hospital mortality and postdischarge mortality and sequelae among patients with EN.This cohort study used French Health System data from January 1, 2013, to December 31, 2022, and included all adult patients (aged ≥18 years) with EN identified using International Statistical Classification of Diseases, Tenth Revision codes combined with a validated algorithm.Epidermal necrolysis.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.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.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.