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Images in Dermatology
December 2, 2020

Neonatal Pemphigus Vulgaris

Author Affiliations
  • 1University of Louisville School of Medicine, Louisville, Kentucky
  • 2Division of Dermatology, University of Louisville, Louisville, Kentucky
JAMA Dermatol. 2021;157(2):220. doi:10.1001/jamadermatol.2020.3990

A 33-year old woman delivered a boy at 38 weeks via cesarean delivery. The neonate had diffuse erosions present at birth. Further evaluation revealed superficial annular erosions with central granulation tissue and desquamating borders on the occipital scalp, postauricular region, nape, and right calf (Figure 1). The mother had a 5-year history of pemphigus vulgaris and originally presented with erosions of the buccal mucosa, palate, nare, and conjunctival injection (Figure 2). Indirect immunofluorescence was positive for IgG cell surface antibodies (1:1260) and anti–desmoglein-3 antibodies on enzyme-linked immunosorbent assay (196 units). At the time of delivery, the mother exhibited erosions at the proximal nail folds of the feet. Neonatal pemphigus vulgaris was suspected. Serum testing of the neonate supported the diagnosis with positive IgG cell surface antibodies (1:320) and anti–desmoglein-3 antibodies (146 units).

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