Meurer M
Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus der Technischen Universität Dresden, Dresden.
Hautarzt. 2009 Mar;60(3):208-16. doi: 10.1007/s00105-009-1733-1.
The pemphigus diseases, which include some of the most severe bullous autoimmune skin reactions, are seen predominantly in middle-aged and elderly individuals. Only endemic pemphigus foliaceus in South America most frequently affects juveniles and children. All non-endemic pemphigus diseases, including paraneoplastic pemphigus, have been reported to occur in adolescents and even very rarely in children younger than 10 years. Pemphigus vulgaris in pregnancy represents a frequently overseen medical problem and may result in fetal growth retardation, intrauterine death, premature delivery and - in about 30% - in neonatal pemphigus vulgaris of the newborn. Contrary to pemphigus vulgaris, the transplacental crossing of autoantibodies against desmoglein1 in pregnant women with pemphigus foliaceus hardly ever leads to neonatal skin lesions in the offspring. This phenomenon can be explained by differences in the distribution and cross-compensation of the pemphigus antigens desmoglein3 and 1 in neonatal and adult skin or mucosa, respectively.
天疱疮疾病包括一些最严重的大疱性自身免疫性皮肤反应,主要见于中年人和老年人。只有南美洲的地方性落叶型天疱疮最常影响青少年和儿童。据报道,所有非地方性天疱疮疾病,包括副肿瘤性天疱疮,都可发生于青少年,甚至10岁以下儿童极为罕见。妊娠期寻常型天疱疮是一个常被忽视的医学问题,可能导致胎儿生长受限、宫内死亡、早产,约30%的新生儿会出现新生儿寻常型天疱疮。与寻常型天疱疮相反,患有落叶型天疱疮的孕妇体内抗桥粒芯糖蛋白1自身抗体经胎盘传递几乎不会导致子代出现新生儿皮肤病变。这种现象可以用新生儿和成人皮肤或黏膜中,天疱疮抗原桥粒芯糖蛋白3和1的分布及交叉补偿差异来解释。