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泛发性家族性良性天疱疮

Fang WANG,Li MA,Xia DOU,Zhong LU,Wei SU,Jin-hua XU,Lian-jun CHEN

Journal of Clinical Dermatology（2009）

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Abstract
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枉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.
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Key words
Family characteristics,Pemphigus, benign, familial, generalized
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