成人表皮坏死的发生率、住院死亡率、长期死亡率及后遗症

表皮坏死（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），其发生率在不同研究中有所差异。住院死亡率通常在15%至20%之间，但对长期死亡率的评估较少，且其影响尚不明确。为了评估EN患者的发病率、住院及出院后死亡率与后遗症的相关因素，本队列研究利用2013年1月1日至2022年12月31日法国卫生系统的数据，纳入所有使用国际疾病分类第十版（ICD-10）编码并经过验证的成人EN患者（≥18岁）。主要观察指标为发病率、住院死亡率、出院后死亡率及后遗症。采用多变量Cox比例风险模型分析与死亡相关的因素。最终纳入1221例成人EN患者（中位年龄66岁，IQR 49-79岁；女性688名，占56.3%）。发病率为每百万人年2.6例（95% CI，2.5—2.7）。住院死亡率为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，31%—36%）。多变量分析显示，住院死亡的危险因素包括年龄（调整风险比[AHR]，每增加1岁为1.03；95% CI，1.02—1.04）、癌症史（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）以及EN严重程度（TEN相较SJS：AHR，2.14；95% CI，1.49—3.07）。癌症、肝病和痴呆也与出院后死亡相关（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的初始严重程度与出院后死亡无关（TEN对SJS：AHR，0.95；95% CI，0.60—1.47），但急性并发症仍显著相关（急性肾损伤和败血症：AHR，分别为2.14 [95% CI，1.26—3.63] 和 2.44 [95% CI，1.42—4.18]）。主要后遗症包括眼科和情绪障碍。该队列研究结果提示，虽然EN较为罕见，但与高发的住院和出院后死亡率相关，且老年患者及具有合并症者风险更高。然而，与住院死亡不同，出院后死亡与EN的初始严重程度无关，而与急性期并发症相关。未来需建立模型以估算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.