中华皮肤科杂志 ›› 2025, Vol. 58 ›› Issue (5): 446-452.doi: 10.35541/cjd.20240185

• 论著 • 上一篇    下一篇

联合血清学诊断方法在抗BP180型和抗层粘连蛋白332型黏膜类天疱疮中的临床应用

王媛    李锁    李志量    荆可    孙超    梁桂熔    张寒梅    冯素英   

  1. 中国医学科学院  北京协和医学院皮肤病医院， 南京  210042
    王媛现在昆明医科大学第二附属医院皮肤性病科，昆明  650101

  • 收稿日期:2024-04-10 修回日期:2024-08-15 发布日期:2025-04-30
  • 通讯作者: 冯素英 E-mail:fengsy@pumcderm.cams.cn
  • 基金资助:
    江苏省卫生健康委科研项目（ZD2021035）；中国医学科学院临床与转化医学研究专项（2023-I2M-C&T-B-112）

Clinical application of combined serological diagnostic methods in anti-BP180-type and anti-laminin 332-type mucous membrane pemphigoid

Wang Yuan, Li Suo, Li Zhiliang, Jing Ke, Sun Chao, Liang Guirong, Zhang Hanmei, Feng Suying   

  1. Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China
  • Received:2024-04-10 Revised:2024-08-15 Published:2025-04-30
  • Contact: Feng Suying E-mail:fengsy@pumcderm.cams.cn
  • Supported by:
    Scientific Research Project of Jiangsu Provincial Health Commission （ZD2021035）; Clinical and Translational Medicine Research Project of Chinese Academy of Medical Sciences （2023-I2M-C&T-B-112）

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摘要： 【摘要】 目的 应用联合血清学诊断方法，总结抗BP180型和抗层粘连蛋白332（Ln332）型黏膜类天疱疮（MMP）患者的临床和免疫血清学特征。方法 回顾性分析2017年1月至2022年2月中国医学科学院皮肤病医院拟诊为MMP的52例患者资料并收集血清样本，应用盐裂皮肤-间接免疫荧光、酶联免疫吸附试验、免疫印迹联合血清学试验分析患者的免疫血清学和临床特征。两组皮损发生率的比较采用Fisher确切概率检验。结果 52例拟诊为MMP的患者中，32例（61.5%）确诊为抗BP180型MMP；10例（19.2%）为抗Ln332型MMP；4例（7.7%）同时检测到抗BP180和抗Ln332抗体，诊断为抗BP180和抗Ln332型MMP；2例盐裂皮肤IgG表皮侧阳性，但血清学检查未发现靶抗原，诊断为MMP（待分型）；4例免疫血清学检查阴性。对比分析发现，10例抗Ln332型MMP中有6例出现眼睛受累，32例抗BP180型MMP中仅有 6 例（18.8%），两型发生率经Fisher确切概率检验，差异有统计学意义（P = 0.02）。10例抗Ln332型MMP患者中，有5例鼻部受累，而32例抗BP180型患者无鼻部累及（P < 0.001）；抗Ln332型MMP患者中有9例出现多个黏膜部位受累，而抗BP180型患者中仅有8例（25.0%）（P < 0.001）。6例抗Ln332型MMP有瘢痕形成，而抗BP180型患者仅有6例（18.8%）（P = 0.02）。结论 盐裂皮肤-间接免疫荧光、酶联免疫吸附试验、免疫印迹联合血清学试验能很好识别MMP中抗BP180和抗Ln332自身抗体，BP180是最常见的靶抗原；抗Ln332型比抗BP180型MMP更常出现眼睛、鼻部受累，且常与多个黏膜部位受累相关，形成瘢痕风险更高。

关键词: 类天疱疮, 良性黏膜, 荧光抗体技术, 间接, 酶联免疫吸附测定, 免疫印迹法, BP180, 层粘连蛋白332, 盐裂皮肤

Abstract: 【Abstract】 Objective To apply combined serological diagnostic methods for anti-BP180-type and anti-laminin 332 （Ln332）-type mucous membrane pemphigoid （MMP）, and to summarize their clinical and immunoserological characteristics. Methods A retrospective analysis was conducted on the data from 52 patients clinically suspected of having MMP at the Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2017 to February 2022. Serum samples were collected, and combined serological tests, including indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting, were performed to analyze the immunoserological and clinical characteristics of the patients. The Fisher′s exact test was used to compare the lesion occurrence rates between groups. Results Among the 52 patients clinically suspected of MMP, 32 （61.5%） were diagnosed with anti-BP180-type MMP, 10 （19.2%） with anti-Ln332-type MMP, and 4 （7.7%） with anti-BP180- and anti-Ln332-type MMP due to the presence of both anti-BP180 and anti-Ln332 antibodies; 2 tested positive for IgG on the epidermal side of salt-split skin, but no target antigens were identified by serological tests, and they were diagnosed with MMP （subtype pending）; 4 tested negative by immunoserological tests. Ocular involvement was observed in 6 out of 10 patients with anti-Ln332-type MMP, whereas only 6 out of 32 anti-BP180-type MMP patients （18.8%） had ocular symptoms, and there was a significant difference in the occurrence rate of ocular involvement between the two groups （P = 0.02）; the occurrence rates of nasal involvement and multi-mucosal involvement were significantly higher in the anti-Ln332-type MMP patients （50%［5/10］, 90%［9/10］, respectively） than in the anti-BP180-type MMP patients （0, 25%［8/32］, respectively, both P < 0.001）. Scar formation occurred in 6 out of 10 anti-Ln332-type MMP patients, but occurred in only 6 out of 32 anti-BP180-type patients （18.8%, P = 0.02）. Conclusion The combination of indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting could effectively identify anti-BP180 and anti-Ln332 autoantibodies in MMP patients, with BP180 being the most common target antigen; compared with anti-BP180-type MMP, anti-Ln332-type MMP appeared to be more frequently involve the ocular and nasal mucosae, associated with the involvement of multiple mucosal sites, carrying a higher risk of scar formation.

Key words: Pemphigoid, benign mucous membrane, Fluorescent antibody technique, indirect, Enzyme-linked immunosorbent assay, Immunoblotting, BP180, Laminin332, Salt-split skin

引用本文

王媛 李锁 李志量 荆可 孙超 梁桂熔 张寒梅 冯素英. 联合血清学诊断方法在抗BP180型和抗层粘连蛋白332型黏膜类天疱疮中的临床应用[J]. 中华皮肤科杂志, 2025,58(5):446-452. doi:10.35541/cjd.20240185

Wang Yuan, Li Suo, Li Zhiliang, Jing Ke, Sun Chao, Liang Guirong, Zhang Hanmei, Feng SuyingHospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing , China. Clinical application of combined serological diagnostic methods in anti-BP180-type and anti-laminin 332-type mucous membrane pemphigoid[J]. Chinese Journal of Dermatology, 2025, 58(5): 446-452.doi:10.35541/cjd.20240185