中华皮肤科杂志 ›› 2022, Vol. 55 ›› Issue (7): 557-561.doi: 10.35541/cjd.20210896

• 论著 • 上一篇    下一篇

抗p200类天疱疮7例临床及免疫血清学特征分析

王媛    李志量    李锁    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皮肤科，南京  210042
  • 收稿日期:2021-12-10 修回日期:2022-04-22 发布日期:2022-07-05
  • 通讯作者: 冯素英 E-mail:fengsy@pumcderm.cams.cn
  • 基金资助:
    中国医学科学院医学与健康科技创新工程项目（2021-I2M-1-059）； 江苏省卫生健康委科研项目（ZD2021035）

Clinical and immunoserological features of 7 cases of anti-p200 pemphigoid

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

  1. Department of Dermatology, Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China
  • Received:2021-12-10 Revised:2022-04-22 Published:2022-07-05
  • Contact: Feng Suying E-mail:fengsy@pumcderm.cams.cn
  • Supported by:
    CAMS Innovation Fund for Medical Sciences （2021-I2M-1-059）; Scientific Research Project of Jiangsu Provincial Health Commission （ZD2021035）

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摘要： 【摘要】 目的 总结抗p200类天疱疮患者的临床和免疫血清学特征。方法 收集2015年1月至2021年10月在中国医学科学院皮肤病医院确诊为抗p200类天疱疮的患者资料，回顾性分析其临床和免疫血清学特征。结果 纳入7例抗p200类天疱疮患者，盐裂皮肤-间接免疫荧光实验显示，7例患者血清IgG抗体均结合于盐裂皮肤的真皮侧，以真皮提取物为底物的免疫印迹显示，在相对分子质量200 000蛋白处有条带。4例呈经典型大疱性类天疱疮样损害，2例初起呈湿疹样损害，1例类似线状IgA大疱性皮病。6例循环IgG抗体可识别层粘连蛋白γ1-C端重组蛋白。4例患者接受不同剂量系统糖皮质激素治疗，其中1例对高剂量系统糖皮质激素（相当于泼尼松1.4 mg·kg-1·d-1）治疗抵抗；2例对米诺环素、氨苯砜治疗反应好；1例失访。4例患者在随访平均22.5个月时达到完全缓解停药；2例在随访平均8个月时达到最小剂量治疗保持完全缓解。结论 抗p200类天疱疮临床表现多样，重组Lnγ1-C端可作为可靠的抗原底物用于检测抗p200类天疱疮患者自身抗体；部分患者最终可达到完全缓解并停药。

关键词: 类天疱疮, 大疱性, 皮肤表现, 荧光抗体技术, 间接, 印迹法, 蛋白质, 治疗, 抗p200类天疱疮, 层粘连蛋白γ1, C端, 盐裂皮肤

Abstract: 【Abstract】 Objective To analyze clinical and immunoserological features of patients with anti-p200 pemphigoid. Methods Clinical data were collected from patients with confirmed anti-p200 pemphigoid in Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2015 to October 2021, and their clinical and immunoserological characteristics were retrospectively analyzed. Results Seven patients with anti-p200 pemphigoid were included. Indirect immunofluorescence on salt-split skin （IIF-SSS） showed that serum IgG antibodies of the 7 patients were located in the dermis of the salt-split skin, and Western blot analysis with dermal extracts as substrates revealed a protein band with a relative molecular mass of 200 000. Four patients presented with classic bullous pemphigoid-like skin lesions, 2 initially presented with eczematous lesions, and 1 presented with linear IgA bullous dermatosis-like skin lesions. Circulating IgG antibodies could recognize the recombinant laminin γ1 C-terminal region in 6 cases. Four patients received different doses of systemic glucocorticoids, 1 of whom was resistant to high-dose systemic glucocorticoids （equivalent to 1.4 mg·kg-1·d-1 prednisone）; 2 responded well to minocycline and dapsone; 1 was lost to follow-up. Four patients achieved complete remission and discontinued the treatment at a mean follow-up of 22.5 months; 2 received complete remissiona on minimal therapy at a mean follow-up of 8 months. Conclusion Patients with anti-p200 pemphigoid presented with heterogeneous clinical manifestations, and the recombinant C-terminal fragment of laminin γ1 can serve as a reliable antigen substrate for the detection of autoantibodies in patients with anti-p200 pemphigoid; some patients can eventually achieve complete remission off treatment.

Key words: Pemphigoid, bullous, Skin manifestations, Fluorescent antibody technique, indirect, Blotting, Western, Therapy, Anti-p200 pemphigoid, Laminin γ1, C-terminus, Salt-split skin