红斑狼疮等中毒性表皮坏死松解的临床、病理和免疫学特征:系统综述和诊断标准建议。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2025-10-01 Epub Date: 2025-08-26 DOI:10.1177/09612033251371801
Sweta Subhadarshani, Taylor Griffith, Zachary Thornton, Andrew Nicholas
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引用次数: 0

摘要

背景:毒性表皮坏死松解如红斑狼疮(TEN-like LE)是一种与显著发病率和死亡率相关的皮肤病急症。它需要及时识别和区分药物引起的毒性表皮坏死松解、多形性红斑、伴嗜酸性粒细胞增多和全身性症状的药物性皮疹以及反应性感染性皮肤粘膜疹。目的系统评价ten样LE患者的临床、病理和免疫学特征。方法对1977 - 2024年间发表的TEN-like LE研究进行结构化检索系统回顾。,检索PubMed、Medline、Embase、SCOPUS、VHL、ProQuest、clinicaltrials.gov、COCHRANE、POPLINE、ISI、mRCT、UC library和ResearchGate。结论16个病例系列,52篇病例报告/会议论文,112例独特病例。平均年龄43.39岁,女性占86.6%。48.21%的患者以10样LE为红斑狼疮(LE)的主要表现。109例患者抗核抗体(ANA)均呈阳性,其中SSA/Ro最常见(60.55%),其次是dsDNA(50%)。病变光分布占78.57%,掌跖受累占20.54%,粘膜受累占56.25%,其中26.78%的患者有明显的粘膜受累。在25%的患者中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,抗ro抗体呈负相关。dsDNA阳性、La阳性和低C4与全身性受累的几率增加相关,而Ro阳性则与降低的几率相关。7例患者有副肿瘤关联。在多达一半的病例中,TEN-like LE可能是LE的第一表现。光分布、狼疮非特异性病变、ANA阳性或ANA类型/滴度的变化可能是重要的诊断线索。与以前认为的不同,粘膜受累并不罕见,而且可能很严重。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical, pathological and immunological features of toxic-epidermal necrolysis like lupus erythematosus: A systematic review and proposal for diagnostic criteria.

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.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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