痒疹样营养不良型大疱性表皮松解症误诊1例

A Case of Misdiagnosed Dystrophic Epidermolysis Bullosa Pruriginosa

患者女,46岁。反复双下肢结节、水疱13年,加重伴泛发全身2年。先后在各地多家医院进行了4次组织病理检查,诊断为"痒疹、扁平苔藓"等。躯干背部、肘关节、双下肢胫前对称性分布紫红色丘疹、斑块,部分斑块上可见水疱,Nikolsky征阴性,局部见散在抓痕、结痂。皮肤病理示表皮下裂隙形成,直接免疫荧光结果IgG,IgA,IgM,C1q,C3a均阴性。确诊为痒疹样营养不良型大疱性表皮松解症。

A 46-year-old woman presented a 13-year history of recun＇ent plaques and blisters over both lower limbs,aggravated and expanded to the trunk two years ago. She had t＇nur times of histopathological examination in tbur local different hospitals with diagnosis of prurigo,lichen planus respectively. On examination she was tbund to have a few mauve papules and plaques over back,elbow joint and both lower limbs with vesicles on the surface. Nikolsky＇s sign was negative. Some scratch marks and crusts were seen on the legs. Light microscopic histopathology of skirl lesion showed the formation of sub epidermal blister and the result of I）[F demonstrated lgG （ - ） ,lgA （ - ） ,lgM （ - ） ,Clq （ - ） ,C3a （ - ）. Diagnosis of dystrophic epidennolysis bnl- losa pruriginosa was made.