表皮坏死松解症（EN），包括史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）的发生率在不同研究中存在差异。虽然院内死亡率为 15% 至 20%，但长期死亡率的影响因素很少被评估且仍然未知。 EN。这项队列研究使用了2013年1月1日至2022年12月31日期间法国卫生系统的数据，并纳入了所有患有EN的成年患者（年龄≥18岁），这些患者是使用国际疾病统计分类第十次修订版代码结合经过验证的算法。表皮坏死松解症。主要结局是评估发病率、院内死亡率、出院后死亡率和后遗症。使用多变量 Cox 比例风险模型评估与死亡率相关的因素。总共纳入 1221 名成年 EN 患者（中位 [IQR] 年龄，66 [49-79] 岁；688 名女性 [56.3%]）。发病率为每百万人年 2.6 例（95% CI，2.5-2.7）例。院内死亡率为 19%（95% CI，17%-21%），出院后死亡率为 15%（95% CI，13%-17%），总死亡率为 34%（95% CI，17%-17%）。 31%-36%）。在多变量分析中，与院内死亡率相关的因素包括年龄（调整后风险比 [AHR]，每岁 1.03；95% CI，每岁 1.02-1.04）、癌症病史（AHR，2.04；95% CI，1.53-2.72）、痴呆（AHR，1.85；95% CI，1.12-3.07）、肝病（AHR，1.81；95% CI，1.24-2.64）和 EN 严重程度（TEN 与 SJS：AHR，2.14； 95% CI，1.49-3.07）。癌症、肝脏疾病和痴呆仍然与出院后死亡率相关（AHR，分别为 3.26 [95% CI，2.35-4.53]、1.86 [95% CI，1.11-3.13] 和 1.95 [95% CI，1.11-3.43] ）。相反，EN 初始严重程度与出院后死亡率无关（TEN 与 SJS：AHR，0.95；95% CI，0.60-1.47），但急性并发症仍然相关（AHR，2.14 [95% CI，1.26-3.63] 和急性肾损伤和脓毒症分别为 2.44 [95% CI，1.42-4.18]）。主要后遗症是眼科和情绪障碍。这项队列研究的结果表明，虽然 EN 是一种罕见疾病，但它与年龄较大且患有合并症的患者院内和出院后死亡率较高有关。然而，与院内死亡率相反，出院后死亡率与 EN 初始严重程度无关，但与急性院内并发症（例如急性肾损伤和脓毒症）相关。未来的研究需要构建模型来估计 EN 患者的长期结果和后遗症。

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.