儿童获得性大疱性表皮松解症一例

A case of epidermolysis bullosa acquisita in childhood

报道1例儿童获得性大疱性表皮松解症.患者女,12岁,因全身反复水疱、大疱和糜烂面3个月入院.患者弱智,父母非近亲结婚,无家族史.入院时表现全身大片糜烂面,包括手足、膝关节周围和股臀部,同时躯干、四肢正常或水肿性红斑基础上紧张性水疱、大疱.治疗过程中突然发生躯干、四肢大量紧张性水疱.组织病理提示,表皮下水疱,免疫荧光结果见表皮基底膜带IgG和C3线状沉积,盐裂后沉积限于真皮侧.ELISA检测BP180和BP230阴性.采用大剂量糖皮质激素联合四环素口服取得满意疗效.

A case of epidermolysis bullosa acquisita （EBA） in childhood is reported. A 12-year-old girl was hospitalized for a 3-month history of recurrent blisters, bullae and erosions on the trunk and limbs. The girl had mental retardation but no family history of similar disorders. The marriage between her parents was not consanguineous. Physical examination on admission revealed large erosions with moderate oozing on the hands and feet, around the knees and on the buttock and thighs. There were scattered tense blisters and bullae arising in normal skin or edematous erythema on the trunk and limbs. During the treatment course the patient suddenly developed a number of tense blisters over the whole integument on the trunk and limbs. Skin biopsy showed subepidermal bullae with moderate perivascular infiltration of neutrophils and eosinophils. Direct immunofluorescence （DIF） revealed linear IgG and C3 deposition along the basal membrane zone, which was on the dermal side of salt-split skin on indirect immunofluorescence （IIF）. ELISA detected no serum antiBP180 or -BP230 antibodies in the patient. A diagnosis of EBA was made. The patient was successfully controlled by intravenous steroids combined with oral tetracycline.