抗p200类天疱疮35例临床及免疫血清学特征回顾性分析  

Clinical and immunoserological features of 35 cases of anti-p200 pemphigoid: a retrospective analysis

作　　者：李锁 荆可 王媛[3] 李志量 赵晨静 张寒梅 梁桂熔 冯素英 Li Suo;Jing Ke;Wang Yuan;Li Zhiliang;Zhao Chenjing;Zhang Hanmei;Liang Guirong;Feng Suying(Department of Venereology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China;Department of Dermatology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China;Department of Dermatology and Venereology,the Second Affiliated Hospital of Kunming Medical University,Kunming 650101,China)

机构地区：[1]中国医学科学院、北京协和医学院皮肤病医院性病科,南京210042 [2]中国医学科学院、北京协和医学院皮肤病医院皮肤内科,南京210042 [3]昆明医科大学第二附属医院皮肤性病科,昆明650101

出　　处：《中华皮肤科杂志》2024年第10期897-903,共7页Chinese Journal of Dermatology

基　　金：江苏省卫生健康委医学科研项目(ZD2021035);中国医学科学院临床与转化医学研究专项项目(2023-I2M-C&T-B-112)。

摘　　要：目的总结抗p200类天疱疮患者的临床、免疫血清学和治疗特点。方法回顾分析2015年1月至2024年2月间就诊于中国医学科学院皮肤病医院的抗p200类天疱疮患者资料,分析其临床、免疫血清学特征和治疗情况。结果共纳入患者35例,包括男25例,女10例,男女比例为2.5∶1,年龄(57.74±17.12)岁,2例(5.71%)合并银屑病。患者皮损表现具有多样性,其中20例(57.14%)表现为典型BP样损害,8例(22.86%)表现为线状IgA大疱性皮病样损害,4例(11.43%)表现为湿疹样损害。直接免疫荧光(DIF)、盐裂皮肤-间接免疫荧光(ss-IIF)、以真皮侧提取物为底物和层黏连蛋白γ1-C端(Lnγ1C)为底物的免疫印迹阳性率分别为100%(24/24)、82.86%(29/35)、100%(35/35)、80.64%(25/31)。35例中33例有治疗及随访信息,6例(18.18%)接受非糖皮质激素(简称激素)系统治疗和外用激素治疗,随访时间[M(Q1,Q3),下同]为19.50(6.50,69.25)个月,其中1例已经停药;16例接受激素联合传统抗炎药物系统治疗,随访时间为13.50(4.25,18.00)个月,激素起始剂量相当于泼尼松0.30~0.50 mg·kg-1·d-1,疾病控制时间为(15.31±5.23)d,其中3例病情波动加用氨苯砜能够控制病情,1例已停用激素;5例(15.15%)接受激素联合免疫抑制剂系统治疗,随访时间为26.00(14.00,90.00)个月,激素起始剂量相当于泼尼松0.50~0.75 mg·kg-1·d-1,疾病控制时间为(10.20±3.27)d,其中2例以相当于泼尼松5~10 mg/d维持治疗,2例患者停药,1例激素停用后复发;1例(3.03%)接受激素联合利妥昔单抗系统治疗,随访时间为53个月,目前激素已停用;1例(3.03%)接受激素联合度普利尤单抗治疗且有效;4例(12.12%)接受激素联合JAK抑制剂治疗,其中3例有效。结论抗p200类天疱疮临床表现具有多形性,但很少出现瘢痕和粟丘疹,部分患者存在Lnγ1C为底物的免疫印迹阴性现象,预后较好,多数患者最终可以达到完全缓解并最终停药。Objective To analyze clinical,immunoserological,and therapeutic features of patients with anti-p200 pemphigoid.Methods Clinical data were collected from patients with confirmed anti-p200 pemphigoid at the Hospital of Dermatology,Chinese Academy of Medical Sciences from January 2015 to February 2024.Their clinical,immunoserological,and therapeutic characteristics were retrospectively analyzed.Results A total of 35 patients were included,with a male-to-female ratio of 2.5∶1(25 males and 10 females)and ages of 57.74±17.12 years.Two(5.71%)patients were accompanied by psoriasis.In these patients,anti-p200 pemphigoid exhibited heterogeneous clinical phenotypes,mimicking classic bullous pemphigoid(20 cases,57.14%),linear IgA bullous dermatosis(8 cases,22.86%),or eczema(4 cases,11.43%).The positive rates of direct immunofluorescence(DIF),indirect immunofluorescence on salt-split skin(ss-IIF),Western blot analysis with dermal extracts as substrates,and Western blot analysis with lamininγ1 C-terminal region(Lnγ1C)as substrates were 100%(24/24),82.86%(29/35),100%(35/35),and 80.64%(25/31),respectively.Among the 35 patients,treatment and follow-up information was available for analysis in 33.Six patients(18.18%)received non-glucocorticoid systemic therapy and topical glucocorticoid therapy,with a follow-up period(M[Q1,Q3])of 19.50(6.50,69.25)months,and 1 withdrew the drugs.Sixteen patients received systemic glucocorticoids combined with traditional anti-inflammatory drugs,with a follow-up period of 13.50(4.25,18.00)months,the initial dose of glucocorticoids was equivalent to 0.30-0.50 mg·kg-1·d-1 of prednisone,and the time to disease control was 15.31±5.23 days;among the 16 patients,3 experienced fluctuations in disease condition which were alleviated by adding dapsone,and 1 discontinued glucocorticoids.Five patients(15.15%)received systemic glucocorticoids combined with immunosuppressants,with a follow-up period of 26.00(14.00,90.00)months,the initial dose of glucocorticoids was equivalent to 0.50-0.75 mg·kg-1·d-1

关 键 词：皮肤疾病 水疱大疱性 抗p200类天疱疮 类天疱疮 大疱性 皮肤表现 荧光抗体技术 间接 盐裂皮肤 印迹法 蛋白质 治疗 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]