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妊娠期自身免疫性大疱性疾病:发病机制、临床表现、诊断及现有治疗方法概述

Autoimmune bullous diseases in pregnancy: an overview of pathogenesis, clinical presentations, diagnostics and available therapies.

作者信息

Vičić Marijana, Marinović Branka

机构信息

Department of Dermatovenereology, Faculty of Medicine, Clinical Hospital Centre of Rijeka, University of Rijeka, Rijeka, Croatia.

Department of Dermatovenereology, Faculty of Medicine, University Hospital Centre of Zagreb, University of Zagreb, Zagreb, Croatia -

出版信息

Ital J Dermatol Venerol. 2023 Apr;158(2):99-109. doi: 10.23736/S2784-8671.23.07553-9.

Abstract

Autoimmune bullous diseases (AIBDs) are rare organ-specific diseases characterized by the appearance of blisters and erosions on the skin and mucous membranes. These dermatoses are marked by the development of autoantibodies targeting the autoantigens located in intercellular junctions, i.e., between keratinocytes or in the basement membrane area. Therefore, the fundamental division of AIBDs into the pemphigus and pemphigoid groups exists. Although AIBDs are uncommon in the general population, their overall incidence is somewhat higher in women of all ages, for which a pregnant women can be likely affected too. While the pemphigoid gestationis is exclusive bullous dermatosis of pregnancy, the other AIBDs can also start or worsen during this period. The appearance of AIBDs in childbearing women is a particularly sensitive situation requiring exceptional clinicians' caution due to the possibility of pregnancy complications with adverse effects and risks to the mother and the child. Also, there are numerous management difficulties in the period of pregnancy and lactation related to the drugs' choice and safety. This paper aimed to outline the pathophysiologic mechanisms, clinical manifestations, diagnostic approach and therapy of the most commonly recognized AIBDs in pregnancy.

摘要

自身免疫性大疱性疾病(AIBDs)是罕见的器官特异性疾病,其特征是皮肤和黏膜上出现水疱和糜烂。这些皮肤病的特点是针对位于细胞间连接(即角质形成细胞之间或基底膜区域)的自身抗原产生自身抗体。因此,AIBDs基本分为天疱疮和类天疱疮两组。虽然AIBDs在普通人群中并不常见,但在各年龄段女性中的总体发病率略高,孕妇也可能受到影响。妊娠类天疱疮是妊娠期特有的大疱性皮肤病,而其他AIBDs也可能在此期间发病或加重。育龄妇女出现AIBDs是一种特别敏感的情况,由于可能出现对母亲和胎儿有不良影响和风险的妊娠并发症,需要临床医生格外谨慎。此外,在妊娠和哺乳期,药物的选择和安全性方面存在诸多管理难题。本文旨在概述妊娠期最常见的AIBDs的病理生理机制、临床表现、诊断方法和治疗。

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