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大疱性类天疱疮和黏膜类天疱疮的流行病学与发病机制

Author
Kristin M Leiferman, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD
Translators
李丽, 副主任医师，副教授

引言

大疱性类天疱疮和黏膜类天疱疮(mucous membrane pemphigoid, MMP)是不常见的自身免疫性上皮下疱性疾病，最常见于较年长成人，其特点是存在皮肤大疱和糜烂性黏膜病损。目前对这些疾病发病机制的理解已取得了重大进展。多个事件很可能促成大疱性类天疱疮和MMP的临床表现，包括：免疫球蛋白与基底膜带成分的结合，后续的补体活化，以及炎症细胞迁移进入上皮下组织。

大疱性类天疱疮和MMP的流行病学和发病机制将总结在此。这些疾病的临床特征、诊断和治疗，以及有关MMP眼型(眼瘢痕性类天疱疮)的更详细内容参见其他专题。(参见“Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid”和“大疱性类天疱疮的处理与预后”和“黏膜类天疱疮的治疗”和“眼部瘢痕性类天疱疮”)

分类

类天疱疮疾病包括：大疱性类天疱疮、MMP、抗层粘连蛋白332型类天疱疮(也称为抗表皮整联配体蛋白型瘢痕性类天疱疮)、妊娠性类天疱疮、Brunsting-Perry型类天疱疮，以及抗层粘连蛋白γ1(抗p200)型类天疱疮，其临床特点为存在炎症性、疱性和/或糜烂性皮肤黏膜病损，组织学特点为上皮下裂隙、表皮基底膜带的IgG和/或补体沉积(图片 1A-B)。水疱和免疫球蛋白沉积的部位可以区分类天疱疮疾病与天疱疮。天疱疮的水疱形成和抗体沉积发生于表皮/上皮内(图片 2A-B)。 (参见“Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid”和“天疱疮的发病机制、临床表现与诊断”)

MMP不是一种疾病，而是一组主要累及黏膜表面的异质性慢性上皮下疱性疾病[1]。由于该疾病在临床上可能发生瘢痕后遗症，过去将这类现在划分为MMP的疾病称为瘢痕性类天疱疮。

一些作者还将线型IgA大疱性皮肤病和获得性大疱性表皮松解症的黏膜为主型也视为MMP[1]。然而，我们倾向于将这些视为不同的疾病。(参见“线状IgA大疱性皮肤病”和“获得性大疱性表皮松解症”)

                

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Literature review current through: 2017-06 . | This topic last updated: 2017-02-07.
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