Brenner S, Dorfman B, Himelfarb M
Dermatologica. 1985;171(1):38-40. doi: 10.1159/000249384.
A case of familial pemphigus vulgaris is described in an uncle and his niece who developed the disease 18 years apart. The man died from pemphigus in 1964. The diagnosis was confirmed histopathologically in both cases, but immunofluorescent microscopic studies were performed only in the woman because the technique was not available in 1964. HLA typing in the woman and her daughter and sister showed A 26, BW 38 and DRW 4 in all of them. Clinical disease did not develop in the other family members.
本文描述了一例寻常型家族性天疱疮,患者为一位叔叔及其侄女,两人发病间隔18年。该男子于1964年死于天疱疮。两例均经组织病理学确诊,但仅对该女性患者进行了免疫荧光显微镜检查,因为1964年时尚无此项技术。对该女性患者及其女儿和妹妹进行的HLA分型显示,她们均具有A26、BW38和DRW4。其他家庭成员未患临床疾病。