线状IgA大疱性皮病26例临床及免疫血清学特征回顾分析  

Clinical and immunoserological characteristics of 26 cases of linear IgA bullous dermatosis: a retrospective analysis

作　　者：荆可 李锁 冯素英 Jing Ke;Li Suo;Feng Suying(Department of Dermatology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China;Department of Venereology,Hospital of Dermatology,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,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皮肤内科,南京210042 [2]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

出　　处：《中华皮肤科杂志》2024年第6期503-509,共7页Chinese Journal of Dermatology

基　　金：江苏省卫生健康委科研项目(ZD2021035)。

摘　　要：目的总结线状IgA大疱性皮病(LABD)患者的临床、免疫血清学和治疗特点。方法回顾性收集2016—2023年就诊于中国医学科学院皮肤病医院诊断为LABD的患者资料,分析其临床、免疫血清学和治疗特征。结果共纳入LABD患者26例,女14例、男12例,年龄[M(Q1,Q3)]为32(11,48)岁。26例患者中12例(46.2%)表现为典型的花环样红斑、水疱,14例(53.8%)表现不典型,即非花环样排列的红斑、水疱。直接免疫荧光检查阳性率为73.7%(14/19)。盐裂皮肤-间接免疫荧光(ss-IIF)检查阳性率为53.8%(14/26),均表现为IgA伴或不伴IgG沉积在表皮侧。免疫印迹实验检测到IgA抗体阳性率为88.5%(23/26),18例(69.2%)患者血清IgA可识别相对分子质量120000的线状IgA抗原1(LAD-1),其中4例IgA同时识别BP180;1例(3.8%)IgA识别表皮提取物中相对分子质量约170000的蛋白;1例(3.8%)IgA识别表皮提取物中BP230和相对分子质量为140000的蛋白;1例(3.8%)IgA可识别相对分子质量为290000的Ⅶ型胶原。21例患者对氨苯砜治疗反应良好,1例疗效欠佳,1例不耐受,此2例使用托法替布亦不能达到完全缓解;3例使用米诺环素、秋水仙碱、柳氮磺吡啶等治疗有效。结论本组LABD患者中青年多见,LAD-1是最常见的靶抗原,免疫印迹实验检测IgA抗体的阳性率较免疫荧光检测法高,是鉴别诊断的重要手段。虽然LABD对氨苯砜反应良好,但仍需探索其他安全有效的药物。Objective To investigate clinical,immunoserological,and therapeutic characteristics of patients with linear IgA bullous dermatosis(LABD).Methods Clinical data were collected from patients with LABD in Hospital of Dermatology,Chinese Academy of Medical Sciences from 2016 to 2023,and their clinical,immunoserological,and therapeutic characteristics were retrospectively analyzed.Results Twenty-six patients were included,comprising 12 males and 14 females,with a median age(Q1,Q3)of 32(11,48)years.Among the 26 patients,12(46.2%)presented with annular erythema and blisters,while 14(53.8%)with atypical lesions(erythema and blisters not in an annular arrangement).Direct immunofluorescence assay yielded positive results in 14 out of 19 patients(73.7%).Indirect immunofluorescence on salt-split skin showed IgA with or without IgG deposited in the epidermis of the salt-split skin in 53.8%(14/26)of the patients.The positive rate of IgA antibody detected by Western blot analysis was 88.5%(23/26).Western blot analysis with epidermal extracts as substrates showed that 18 patients(69.2%)had serum IgA recognizing the linear IgA bullous dermatosis autoantigen LAD-1 with a relative molecular weight of 120000,of whom 4(15.4%)also had IgA recognizing BP180;in 1 case(3.8%),the serum IgA could recognize a protein with a relative molecular weight of about 170000 in the epidermal extracts;another 1(3.8%)had IgA recognizing BP230 and a protein with a relative molecular weight of 140000 in the epidermal extracts at the same time;additionally,the serum IgA recognizing typeⅦcollagen with a relative molecular weight of 290000 in the dermal extracts was detected in 1 case(3.8%).Among 23 patients receiving dapsone treatment,21 well responded,1 showed poor response,and 1 was intolerant;in addition,the latter two patients could not achieve complete remission by tofacitinib.Minocycline,colchicine,and sulfasalazine were effective in another 3 patients.Conclusions In this study,LABD mainly occurred in middle-age individuals,and LAD-1 was determined

关 键 词：皮肤疾病 水疱大疱性 免疫球蛋白A 印迹法 蛋白质 氨苯砜 线状IGA大疱性皮病 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]