Maternal Pemphigus Foliaceus With Cell Surface Antibody Bound in Neonatal Epidermis

Neonatal pemphigus vulgaris associated with mild oral pemphigus vulgaris in the mother during pregnancy

“…In previous studies, babies of mothers with pemphigus foliaceus have all had lower neonatal titres of antibody, with none being affected clinically 20 ,. 21 …”

Protection against Pemphigus Foliaceus by Desmoglein 3 in Neonates

“…2,3 In pregnant women with pemphigus foliaceus, autoantibodies cross the placenta and bind to the fetal epidermis, but they rarely cause blisters in neonates. [4][5][6] We hypothesized that the coexpression of desmoglein 3 in the superficial epidermis in neonates protects their skin from blistering caused by passively transferred maternal antibodies against desmoglein 1, with the presence of desmoglein 3 compensating for the antibody-induced loss of desmoglein 1. To confirm this hypothesis, we determined the distribution of desmoglein 3 in neonatal and adult skin and evaluated blistering after the injection of pemphigus P foliaceus antibodies in transgenic mice with desmoglein 3 expressed in both the superficial and deep layers of the epidermis.…”

Pemphigus vulgaris in a neonate

“…5,18 This is supported by at least 18 cases of pregnant women with PF who delivered babies free of the disease. 29,30 There is no correlation between the titer of maternal PV antibodies and the presentai i tion of the disease in neonates. 2,5,6 This is supported by cases of neonatal PV from mothers with no active disi i ease 5,9,31 and cases of healthy neonates born to mothers with highly active disease.…”