患者男,69岁.全身起红斑、水疱、血疱、结痂伴剧烈瘙痒20余天,左上臂起瘀斑伴肿胀3天.结合组织病理、疱病特异性自身抗体BP180(+),及凝血机制异常和凝血因子活性Ⅷ重度缺乏,诊断大疱性类天疱疮并获得性血友病A.本病凶险,死亡率高,应引起临床医生高度重视,及时诊断.“,”A 69-year-old male presented with erythematous plaques,blisters,blood blisters and crusts with severe itching all over the body for about 20 days,and swollen with ecchymosis on his left upper arm for 3 days.According to histopathological features,BP180(+),the abnormal blood coagulation,and deficiency of factor Ⅷ clotting activity,a diagnosis of bullous pemphigoid with acquired hemophilia A disease was made.Because of the high mortality rate,clinicians should pay more attention to this disease and make correct diagnosis in time.