Brunsting-Perry型获得性大疱性表皮松解症1例

Brunsting-Perry Type Epidermolysis Bullosa Acquisita:A Case Report

患者女,73岁,面颈部丘疹水疱2年。皮损组织病理示:表皮下水疱,疱液内中性粒细胞浸润。皮肤直接免疫荧光:基底膜带IgG、C3(+)。盐裂皮肤直接免疫荧光示:真皮侧IgG、C3(+)。酶联免疫吸附试验检测血清抗Ⅶ型胶原抗体弱阳性。诊断:Brunsting-Perry型获得性大疱性表皮松解症。予口服醋酸泼尼松片和甲氨蝶呤片,治疗2周后皮损明显好转。随访2年,泼尼松逐渐减量,未见复发。

A 73-year-old female presented with papules and blisters localized to the face and neck for 2 years.Histopathology of the lesions showed subepidermal blisters with neutrophil infiltration.Direct immunofluorescence of skin showed linear IgG and C3 deposited at the basement membrane zone.Direct immunofluorescence of salt-split skin showed linear IgG and C3 deposited on the dermal side.Enzyme-linked immunosorbent assay showed that serum anti-typeⅦcollagen antibody was weakly positive.Brunsting-Perry type epidermolysis bullosa acquisita was diagnosed.The patient was treated with prednisone and methotrexate,the skin lesions improved significantly after two weeks of treatment.Follow-up for two years,the dose of prednisone was gradually reduced,and no recurrence was observed.