Department of Dermatology, University of Lübeck, Lübeck, Germany.
Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
JAMA Dermatol. 2022 Jan 1;158(1):84-89. doi: 10.1001/jamadermatol.2021.4773.
Mucous membrane pemphigoid (MMP) is a rare, heterogeneous subepithelial autoimmune bullous disease. The association between its clinical and immunological features is yet to be fully evaluated.
To characterize the clinical, immunoserological, and immunopathological characteristics of patients with MMP and to identify site- and autoantigen-specific characteristics.
DESIGN, SETTING, AND PARTICIPANTS: A retrospective cohort study encompassing all consecutive patients diagnosed with MMP from January 2007 through February 2020 in 2 tertiary referral centers in Germany.
The clinical, immunoserological, and immunopathological features of eligible patients were evaluated. Associations of different anatomical sites and autoantigens were assessed using a multivariable logistic regression model.
The study encompassed 154 patients (96 [62.3%] women and 58 [37.7%] men; mean [SD] age at diagnosis, 66.2 [13.8] years) with MMP, of whom 125 (81.2%), 61 (39.6%), 34 (22.1%), and 16 (10.4%) presented with lesions involving the oral, ocular, nasal, and genital mucosae, respectively, and 35 (22.7%) presented with cutaneous involvement. Among the 154 patients, the most frequently targeted antigen was BP180 (90 patients [58.4%]), followed by laminin 332 (13 patients [8.4%]) and BP230 (3 patients [1.9%]). Ocular disease was inversely associated with oral (adjusted odds ratio [aOR], 0.02; 95% CI, 0.01-0.13) and nasal (aOR, 0.20; 95% CI, 0.04-0.91) involvement and was associated with a 13-fold increased risk of malignant neoplasm (aOR, 13.07; 95% CI, 1.56-109.36). Anti-laminin 332 reactivity was associated with malignant neoplasm (aOR, 23.27; 95% CI, 1.83-296.68), whereas anti-BP180 NC16A immunoglobulin G seropositivity was associated with absence of ocular lesions (aOR, 0.09; 95% CI, 0.01-0.99).
In this cohort study of patients with MMP, malignant neoplasms were associated with ocular disease and anti-laminin 332 reactivity, suggesting potential benefit of malignant neoplasm screening in these patients.
黏膜类天疱疮（Mucous membrane pemphigoid，MMP）是一种罕见的、异质性的亚上皮自身免疫性大疱病。其临床和免疫特征之间的关联尚未得到充分评估。
描述 MMP 患者的临床、免疫血清学和免疫病理学特征，并确定与特定部位和自身抗原相关的特征。
设计、地点和参与者：这是一项回顾性队列研究，纳入了 2007 年 1 月至 2020 年 2 月期间在德国 2 家三级转诊中心确诊为 MMP 的所有连续患者。
评估了合格患者的临床、免疫血清学和免疫病理学特征。使用多变量逻辑回归模型评估了不同解剖部位和自身抗原之间的关联。
该研究共纳入 154 例 MMP 患者（96 例[62.3%]为女性，58 例[37.7%]为男性；诊断时的平均[标准差]年龄为 66.2[13.8]岁），其中 125 例（81.2%）、61 例（39.6%）、34 例（22.1%）和 16 例（10.4%）分别出现口腔、眼部、鼻腔和生殖器黏膜受累，35 例（22.7%）出现皮肤受累。在这 154 例患者中，最常被靶向的抗原是 BP180（90 例[58.4%]），其次是层粘连蛋白 332（13 例[8.4%]）和 BP230（3 例[1.9%]）。眼部疾病与口腔（校正优势比[aOR]，0.02；95%CI，0.01-0.13）和鼻腔（aOR，0.20；95%CI，0.04-0.91）受累呈负相关，且与恶性肿瘤风险增加 13 倍相关（aOR，13.07；95%CI，1.56-109.36）。抗层粘连蛋白 332 反应性与恶性肿瘤相关（aOR，23.27；95%CI，1.83-296.68），而抗 BP180 NC16A 免疫球蛋白 G 血清阳性与无眼部病变相关（aOR，0.09；95%CI，0.01-0.99）。
在这项 MMP 患者队列研究中，恶性肿瘤与眼部疾病和抗层粘连蛋白 332 反应性相关，提示这些患者可能需要进行恶性肿瘤筛查。
