伴食道大疱的获得性大疱性表皮松解症合并获得性血友病1例国内首报

目的报告1例伴食道大疱的获得性大疱性表皮松解症合并获得性血友病。方法对其临床、胃镜、血液病学检查、皮肤组织病理和直接免疫荧光进行研究。结果胃镜检查示食道内多发大小不等水疱。血液病学检查示活化部分凝血活酶时间(APTT)88.7s(正常值:20.90~34.60s);第Ⅷ因子活性6.9%(正常值:50.0%~150.0%);第Ⅷ因子抑制物滴度312B.U./ml(正常:0)。组织病理检查:表皮下水疱。1M氯化钠裂解皮肤直接免疫荧光检查示基底膜带真皮侧IgG呈线状沉积。结论此为一罕见的病例。

Epidermolysis Bullosa Acquisita with Esophageal Blister Associated with Acquired Haemophilia (First Case Report in China)

Objective To report a case of epidermolysis bullosa acquisita with esophageal blister associated with acquired haemophilia.Methods The clinical manifestations,gastroscopy,haematology,histopathology and direct immunofluorescent of the lesion were evaluated.Results Gastroscopy revealed bullae on esophageal mucosa.Haematologica analysis showed activated partial thromboplastin time (APTT) was 88.7 second(normal range:20.90~34.60 second),factor VIII activity was 6.9%(normal range:50.0%~150.0%)and factor VIII inhibitor was present at a titre of 312 Bethesda Units/ml (B.U./ml, normal: 0).Skin biopsy revealed subepidermal blisters.Direct immunofluorescence on 1 M NaCl-split skin revealed linear deposition of IgG line dermal side of the basement membrane zone.Conclusion This is an extremely uncommon case.