Subhadarshani Sweta, Griffith Taylor, Thornton Zachary, Nicholas Andrew
Department of Dermatology, University of Pennsylvania, Perelman School of Medicine, Philadelphia, Pennsylvania, USA.
University of Cincinnati, College of Medicine, Cincinnati, Ohio, USA.
Lupus. 2025 Oct;34(12):1221-1229. doi: 10.1177/09612033251371801. Epub 2025 Aug 26.
BackgroundToxic epidermal necrolysis like lupus erythematosus (TEN-like LE) is a dermatological emergency associated with significant morbidity and mortality. It requires prompt recognition and differentiation from its mimics including drug induced toxic epidermal necrolysis, erythema multiforme, drug rash with eosinophilia and systemic symptoms, and reactive infectious mucocutaneous eruptions.ObjectiveTo assess systemically the clinical, pathological, and immunological features of patients with TEN-like LE.MethodsWe performed a structured search systematic review from April-May 2024, of studies on TEN-like LE published between 1977 and 2024., searching PubMed, Medline, Embase, SCOPUS, VHL, ProQuest, clinicaltrials.gov, COCHRANE, POPLINE, ISI, mRCT, UC library, and ResearchGate.ConclusionThere were 16 case series and 52 case reports/conference papers yielding 112 unique cases. The mean age was 43.39 years, and 86.6% of cases were female. TEN-like LE was the presenting manifestation of lupus erythematosus (LE) in 48.21% of patients. Antinuclear antibody (ANA) was positive in all 109 patients where it was reported with SSA/Ro being most commonly positive (60.55%), followed by dsDNA (50%). Lesions were reported to be photodistributed in 78.57%, palmoplantar involvement was seen in 20.54%, and mucosal involvement was seen in 56.25%, with 26.78% patients having significant mucosal involvement. Lupus non-specific lesions were observed in 25% patients. Anemia, pulmonary and GI involvement were positively associated with hemodynamic instability, and anti-Ro antibodies were negatively associated. Positive dsDNA, La positivity, and low C4 were associated with increased odds of systemic involvement, whereas Ro positivity was associated with reduced odds. Seven patients had a paraneoplastic association. TEN-like LE can be the first manifestation of LE in up to half of cases. Photodistribution, lupus non-specific lesions, ANA positivity, or change in ANA type/titre can be important diagnostic clues. Unlike previously believed, mucosal involvement is not uncommon and can be severe.
中毒性表皮坏死松解症样红斑狼疮（TEN样LE）是一种皮肤急症，具有较高的发病率和死亡率。它需要迅速识别并与其他类似疾病相鉴别，包括药物性中毒性表皮坏死松解症、多形红斑、伴有嗜酸性粒细胞增多和全身症状的药疹以及反应性感染性黏膜皮肤疹。
全面评估TEN样LE患者的临床、病理和免疫特征。
我们于2024年4月至5月进行了一项结构化检索的系统综述，纳入1977年至2024年间发表的关于TEN样LE的研究，检索了PubMed、Medline、Embase、SCOPUS、VHL、ProQuest、clinicaltrials.gov、COCHRANE、POPLINE、ISI、mRCT、加州大学图书馆和ResearchGate。
共有16个病例系列以及52篇病例报告/会议论文，涉及112例独特病例。平均年龄为43.39岁，86.6%的病例为女性。48.21%的患者中，TEN样LE是红斑狼疮（LE）的首发表现。在报告的109例患者中，抗核抗体（ANA）均为阳性，其中SSA/Ro最常见呈阳性（60.55%），其次是双链DNA（dsDNA，50%）。据报告，78.57%的病变呈光分布，20.54%可见掌跖受累，56.25%可见黏膜受累，26.78%的患者有严重黏膜受累。25%的患者观察到狼疮非特异性病变。贫血、肺部和胃肠道受累与血流动力学不稳定呈正相关，而抗Ro抗体与之呈负相关。双链DNA阳性、La阳性和低C4与全身受累几率增加相关，而Ro阳性与几率降低相关。7例患者有副肿瘤关联。在多达一半的病例中，TEN样LE可能是LE的首发表现。光分布、狼疮非特异性病变、ANA阳性或ANA类型/滴度变化可能是重要的诊断线索。与之前的认识不同，黏膜受累并不罕见且可能很严重。
