线状IgA大疱性皮病三例临床分析及文献复习.docVIP

精品论文 参考文献 线状IgA大疱性皮病三例临床分析及文献复习 芦桂青　毕志刚　刘艳　康莉　储小燕　富志良　王羿婷　蒋洁瑶　苏彤 南京医科大学附属南京明基医院皮肤科　江苏　南京　２１００１９１．南京市卫生局医学科技发展项目基金资助(ZKX１２０５０):２．南京市卫生青年人才培养工程第三层次培养对象(QRX１１２７８) 　　【摘要】　目的　分析线状IgA 大疱性皮病的临床特点、治疗、预后,提高临床医生对本病的认识.方法　回顾性分析本科近２年诊治的３例线状IgA 大疱性皮病的临床资料,并对相关文献进行分析.结果　男２例,女１例,年龄３－１８岁.临床上表现为红斑基础上的水疱,或外观正常的皮肤上出现的水泡,尼氏征阴性,瘙痒剧烈,伴有口腔黏膜的损害.组织病理示表皮下水疱,直接免疫荧光示基底膜带有线状IgA 沉积.治疗上我们予以柳氮磺胺吡啶、糖皮质激素、免疫抑制剂,加用重组人II型肿瘤坏死因子受体－抗体融合蛋白和/或人血丙种免疫球蛋白治疗,均被成功治疗,随访至今,未见复发.结论　在缺乏氨苯砜的前提下,我们的病例显示柳氮磺胺吡啶、糖皮质激素、免疫抑制剂,加用重组人II型肿瘤坏死因子受体－抗体融合蛋白和/或人血丙种免疫球蛋白能有效治疗线状IgA 大疱性皮病. 【关键词】　线状IgA 大疱性皮病;柳氮磺胺吡啶;糖皮质激素;免疫抑制剂;重组人II型肿瘤坏死因子受体－抗体融合蛋白;人血丙种免疫球蛋白.Analysisof３CasesofLinerIgABullousDermatosisandReviewofLiteratureLuGui－qing,BiZhi－gang,YanLiu,etal??Departmentofdermatology, BenQ MedicalCenter,Nanjing MedicalUniversity,Nanjing,２１００１９[Correspondingauthor]ZhiGang－bi,E－mail:Elton??bi＠benqhospital??com【Abstract】ObjectiveToanalyzetheclinicalfeaturesandtherapeuticactivityoflinerIgAbullousdermatosis(LAD)andimprovethedermatologistrsquo;sunderＧstandingofLAD?? MethodsClinicaldataof３caseswithLADwereanalyzedretrospectively??ResultsAllof３patientspresentedwithisolatedandgroupedtenseserＧum－filledvesiclesandbullaewithnegativeNikolskyrsquo;ssignonhealthyskinoronanblistersin３cases??Directimmunofluorescence(DIF)showedcontinuouslinＧeardepositionofIgAatthebasementmembranezone(BMZ)??Theyweretreatedwithsalazosulfapyridine(SASP),glucocorticosteroid,immunodepressant,somewerecombinedwithrecombinanthumantumornecrosisfactor－alpha;receptorII:IgGFcfusionproteinorintravenousimmunoglobulin??ConclusionWithoutdapsone,theywerecuredwithSASP,glucocorticosteroid,immunodepressant,somewerecombinedwithrecombinanthumantumornecrosisfactor－alpha;receptorII:IgGFcfusion【Kperyotweionrdosr】IVIg?? LinerIgABullousDermatosis;Glucocorticosteroid;Recombinanthumantumornecrosisfactor－alpha;receptorII:IgGFcfusionproteinforinjecＧtion;Intravenousimmunoglobulin 【中图分类号】R７５８．６__________ 【文献标识码】B 　　【文章编号】１００８－６３１５(２０１５)１２－００３８－０２　　 　　线状IgA 大疱性皮病(LinerIgABullousDermatosis,LAD)是一种累及皮肤和粘膜