Neonatal Pemphigus Vulgaris

Abstract

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).Pemphigus vulgaris is a blistering disease of the mucous membranes and skin secondary to autoantibodies directed towards desmoglein-1anddesmoglein-3. 1 Desmogleinsaretransmembraneproteins that function as a structural component of the desmosome and facilitate cell-to-cell adhesion. 1,2 Neonatal pemphigus vulgaris is a transient blistering disorder caused by passive transfer of maternal IgG autoantibodies across the placenta during pregnancy. 1 The risk of an infant developing neonatal pemphigus exists independently of levels of maternal antibody titers and/or mucocutaneous presentation at time ofgestation. [2][3][4] Thedistributionofulcerativelesionsintheneonatefailed to reflect the pattern visible in the mother. [1][2][3][4] Widespread disease in the neonate was a result of desmoglein-3 expression in the neonatal mucosal and cutaneous surfaces. Despite the severity of symptoms, the prognosis was favorable.Immunoglobulin levels transferred from mother to fetus are reduced by age 3 to 6 months. 2 Often, undetectable levels of antibody titers are seen in the infant shortly postdelivery; resolution of symptoms can be seen by age 3 weeks. 3 Therapy aims to support reepithelization of the skin and mucous membranes and prevent concomitant infection. 2,4 Treatment comprises emollients, topical corticosteroids, and anitbiotics. 4 Follow-up with monitoring of symptoms and levels of antibodies is recommended until age 1 year. 1 Although the literature fails to associate the degree of disease in the mother with that of the infant, efforts to reduce antibody levels in pregnant women is recommended. 3,4 This can be achieved with oral prednisone, intravenous immunoglobulin, or plasmapheresis. 1,4

Pregnancy in pemphigus vulgaris: A systematic review

“…Except for neonatal pemphigus vulgaris, pemphigus vulgaris in pregnancy was thought to be related to prematurity and stillbirth 2 . The high prevalence of neonates exhibiting symptoms associated with pemphigus vulgaris could be attributed to placental transmission 8 . In this study, 16 (25.8%) neonates had other abnormalities including stillbirth, premature rupture, premature delivery, placental insufficiency, placenta previa, fetal distress, fetal heart‐rate anomaly, or reported “small for date.”…”

“…These complications include pregnancy‐related hypertension, pregnancy‐related hyperglycemia, greater indication for cesarean section, prematurity, stillborn, intrauterine growth restriction, and other potential risks that endanger both mother and child 6,7 . Furthermore, maternal antibodies can be transmitted to neonates, causing neonatal pemphigus vulgaris, although the antibody titer usually decreases by several months 8 . The relationship between pemphigus vulgaris and pregnancy remains unclear, and the outcomes and treatments of pemphigus vulgaris during pregnancy have not been extensively discussed.…”

Pemphigus vulgaris in a neonate born to a mother with pemphigus vulgaris: A case report

“…However, long‐term follow‐up is required in these patients to detect relapses. No specific treatment is necessary for neonatal PV, as the condition is self‐limiting in 2 to 3 weeks 9,13 . Hence, local wound care with topical emollients is sufficient.…”

“…In addition, the focus should be on promoting early healing and preventing associated infections by using emollients, topical corticosteroids, and antibiotics. Also, a one‐year follow‐up to assess the disease status is recommended 9 …”

Autoimmune Pemphigus: Latest Advances and Emerging Therapies

“…Neonatal pemphigus vulgaris (NPV) is a transient immunobullous disease induced by transplacental transfer of maternal autoantibodies from a mother with PV to her newborn (Fenniche et al, 2006;Gushi et al, 2008;Turrentine et al, 2014;Carvalho et al, 2019;Fenner et al, 2020;Foster et al, 2021). First reported in 1975(Salihbegović-Opalić et al, 1975Zhao et al, 2016), few cases exist in the literature, as female patients with pemphigus can experience infertility, premature delivery and stillbirth (Ouahes et al, 1997).…”