泛发性家族性良性天疱疮

Generalized familial benign pemphigus:a case report

报告1例泛发性家族性良性天疱疮。患者男,56岁。反复发作全身瘙痒性皮损40余年,皮损表现为以躯干部位为主的泛发性绿豆到黄豆大红斑或斑丘疹,以及在红斑或正常皮肤上出现的水疱。皮损组织病理检查示表皮内广泛的棘层松解,并形成表皮内水疱。直接免疫荧光检查结果阴性。家系调查示该家族66人中共有12人患此病。诊断:泛发性家族性良性天疱疮。机械摩擦、感染和搔抓是该病加重的重要因素。

A case of generalized familial benign pemphigus is reported. A 56-year-old male presented with recurrent pruritic eruptions all over the body for more than 40 years. Generalized erythematous macules, maculopapules and the vesicles on the macules or normal skin were noted, predominantly on the trunk. Histological examination revealed extensive acantholysis and intraepidermal blistering. Direct immunoflouresenee test with IgG, IgM, IgA and C3 showed a negative result. Twelve patients out of total 66 members in the family were revealed by the family survey. The case was finally diagnosed as generalized familial benign pemphigus, and the aggravation by mechanical friction, infection and scratching was noticed.