Walker D C, Kolar K A, Hebert A A, Jordon R E
Department of Dermatology, University of Texas Medical School at Houston, USA.
Arch Dermatol. 1995 Nov;131(11):1308-11.
Pemphigus refers to a group of autoimmune blistering diseases of the skin. Of the two major types of pemphigus, pemphigus vulgaris and pemphigus foliaceus, only pemphigus vulgaris has been known to affect newborn infants via passive transfer of maternal IgG antibodies across the placenta. Although pemphigus foliaceus antibodies have also been shown to cross the placenta, never before has a newborn been clinically affected. We report the first of neonatal pemphigus foliaceus confirmed by both clinical presentation and immunofluorescence studies.
The distinguishing factors in this case were high antibody titers by indirect immunofluorescence present in both the mother and her fetus (1:640 and 1:80, respectively).
A threshold of fetal antibody titer ( > 1:40) may need to be surpassed before neonatal disease can occur in pemphigus foliaceus. The likelihood of reaching this threshold has been shown to be increased with higher maternal antibody titers. Thus, strict control of maternal pemphigus foliaceus should lower the incidence of placental antibody transfer and improve neonatal outcome.
天疱疮是指一组皮肤自身免疫性水疱病。在天疱疮的两种主要类型中，寻常型天疱疮和落叶型天疱疮，只有寻常型天疱疮已知可通过母体IgG抗体经胎盘被动转移影响新生儿。虽然落叶型天疱疮抗体也已显示可穿过胎盘，但此前从未有新生儿出现临床受累情况。我们报告首例经临床表现和免疫荧光研究确诊的新生儿落叶型天疱疮。
该病例的鉴别因素是母亲及其胎儿间接免疫荧光检测均显示高抗体滴度（分别为1:640和1:80）。
在落叶型天疱疮中，可能需要超过胎儿抗体滴度阈值（>1:40）才会发生新生儿疾病。已表明母体抗体滴度越高，达到该阈值的可能性就越大。因此，严格控制母体落叶型天疱疮应可降低胎盘抗体转移的发生率并改善新生儿结局。
