Mucous membrane pemphigoid (MMP) is a clinically and immunopathologically heterogenous disease with an incidence of about 2/million inhabitants/year in central Europe. Pemphigoid diseases are characterized by autoantibodies against structural proteins of the epidermis and/or surface-close epithelia. MMP has been defined as pemphigoid disease with predominant mucosal lesions. Most frequently, the oral cavity and the conjunctivae are affected. Lesions outside the mouth tend to heal with scarring leading to visual impairment and finally blindness, as well as, more rarely, impairment of breathing and food intake. Autoantibodies target BP180 (collagen type XVII), laminin 332, BP230 (nearly always in conjunction with other antigens), and type VII collagen in about 75%, 10–20%, 10–30%, and <5% of MMP patients, respectively. While the main autoantibody isotype is IgG, additional, and less frequently exclusive, IgA autoantibodies can be detected in the majority of patients. Assaying for anti-laminin 332 reactivity is pivotal, since in about a quarter of patients with anti-laminin 332 MMP, a malignancy, mainly solid cancers, is associated. The pathophysiology of MMP is yet incompletely understood. A recent mouse model of anti-laminin 332 MMP replicating characteristic clinical and immunopathological findings of the human disease may be helpful to close this knowledge gap. Diagnosis is established by the clinical picture with predominant mucosal lesions and visualization of tissue-bound anti-basement membrane zone antibodies by direct immunofluorescence microscopy. In recent S3 guidelines initiated by the European Academy of Dermatology and Venereology, the clinical spectrum and diagnostic strategies are detailed. In addition, treatment regimens for different clinical situations including patients with exclusive oral or ocular involvement are outlined. Future studies are needed to better understand the clinical complexity and associations as well as to establish widely available diagnostic assays and evidence-based therapeutic strategies.

粘膜类天疱疮(MMP) 是一种临床和免疫病理学异质性疾病，在中欧的发病率约为 2/百万居民/年。类天疱疮疾病的特征在于针对表皮和/或表面紧密上皮的结构蛋白的自身抗体。MMP 被定义为以黏膜病变为主的类天疱疮疾病。最常见的是口腔和结膜受到影响。口腔外的病变往往会随着疤痕愈合而导致视力障碍并最终失明，以及更罕见的呼吸和食物摄入障碍。自身抗体靶向 BP180（XVII 型胶原蛋白）、层粘连蛋白 332、BP230（几乎总是与其他抗原结合），以及VII 型胶原蛋白分别在约 75%、10-20%、10-30% 和 <5% 的 MMP 患者中出现。虽然主要的自身抗体同种型是 IgG，但在大多数患者中可以检测到额外的且不太常见的 IgA 自身抗体。抗层粘连蛋白 332 反应性的测定至关重要，因为在大约四分之一的抗层粘连蛋白 332 MMP 患者中，与恶性肿瘤（主要是实体癌）有关。MMP 的病理生理学尚不完全清楚。最近的抗层粘连蛋白 332 MMP 小鼠模型复制了人类疾病的特征性临床和免疫病理学发现，可能有助于缩小这一知识差距。诊断依据以黏膜病变为主的临床表现和直接观察到组织结合的抗基底膜区抗体免疫荧光显微镜。在最近由欧洲皮肤病学和性病学会发起的 S3 指南中，详细介绍了临床谱和诊断策略。此外，还概述了针对不同临床情况的治疗方案，包括仅受口腔或眼部受累的患者。未来的研究需要更好地了解临床复杂性和相关性，以及建立广泛可用的诊断分析和循证治疗策略。