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类天疱疮疾病的叙述性综述:关联、合并症与管理之间的桥梁

A Narrative Review of Pemphigoid Diseases: Bridging Associations, Comorbidities, and Management.

作者信息

Myers Emma L, Culton Donna A

机构信息

University of North Carolina School of Medicine, 321 S Columbia Street, Chapel Hill, NC, 27599, USA.

Department of Dermatology, University of North Carolina, Chapel Hill, NC, USA.

出版信息

Dermatol Ther (Heidelb). 2025 May 24. doi: 10.1007/s13555-025-01444-9.

Abstract

Pemphigoid diseases are a group of rare, chronic autoimmune blistering disorders (AIBD) characterized by subepidermal blister formation due to autoantibody-mediated targeting of structural components of the basement membrane zone (BMZ). This narrative review provides an updated overview of four key subtypes: bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA bullous dermatosis (LABD), and epidermolysis bullosa acquisita (EBA) with specific emphasis on disease associations that dermatologists should be aware of for comprehensive patient care. BP, the most common subepidermal blistering disorder, is frequently associated with neurological and cardiovascular comorbidities, while MMP primarily affects mucosal surfaces, often leading to significant scarring and functional impairment and can be associated with malignancy in certain subtypes. LABD and EBA are less common but can be linked with systemic autoimmune conditions such as inflammatory bowel disease (IBD). Shared challenges across these subtypes include difficulties in diagnosis due to overlapping clinical and histopathologic features, treatment-related complications from prolonged corticosteroid use, and the substantial impact on patients' quality of life. Advances in therapeutic strategies, including biologic agents such as rituximab and dupilumab, offer promising alternatives to traditional treatments, yet access to new targeted therapies remains a significant barrier. This review emphasizes the need for large-scale studies to better understand disease associations, the identification of biomarkers for personalized therapy, improved treatment options, and the implementation of multidisciplinary care approaches.

摘要

类天疱疮疾病是一组罕见的慢性自身免疫性大疱性疾病(AIBD),其特征是由于自身抗体介导靶向基底膜带(BMZ)的结构成分而形成表皮下疱。本叙述性综述提供了四种关键亚型的最新概述:大疱性类天疱疮(BP)、黏膜类天疱疮(MMP)、线状IgA大疱性皮病(LABD)和获得性大疱性表皮松解症(EBA),特别强调了皮肤科医生为全面的患者护理应了解的疾病关联。BP是最常见的表皮下疱性疾病,常与神经和心血管合并症相关,而MMP主要影响黏膜表面,常导致严重瘢痕形成和功能障碍,并且在某些亚型中可能与恶性肿瘤相关。LABD和EBA较不常见,但可能与全身性自身免疫性疾病如炎症性肠病(IBD)有关。这些亚型共有的挑战包括由于临床和组织病理学特征重叠导致的诊断困难、长期使用皮质类固醇引起的治疗相关并发症以及对患者生活质量的重大影响。治疗策略的进展,包括使用利妥昔单抗和度普利尤单抗等生物制剂,为传统治疗提供了有前景的替代方案,但获得新的靶向治疗仍然是一个重大障碍。本综述强调需要进行大规模研究,以更好地了解疾病关联、识别个性化治疗的生物标志物、改善治疗选择以及实施多学科护理方法。

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