NEONATAL PEMPHIGUS FOLIACEUS

Background: Pemphigus refers to a group of autoimmune blistering disea ses of the skin. Of the two major types of pemphigus, pemphigus vulgar is 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 als o been shown to cross the placenta, never before has a newborn been cl inically affected. We report the first case of neonatal pemphigus foli aceus confirmed by both cal presentation and immunofluorescence studie s. Observation: The distinguishing factors in this case were the high antibody titers by indirect immunofluorescence present in both the mot her and her fetus (1:640 and 1:80, respectively). Conclusions: A thres hold 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 mat ernal antibody titers. Thus, strict control of maternal pemphigus foli aceus should lower the incidence of placental antibody transfer and im prove neonatal outcome.