LAUSR

Neonatal pemphigus vulgaris (NPV) is a transient immunobullous disease, and its pathogenesis involves the passive transfer of maternal immunoglobulin G (IgG) antibodies through the placenta from a mother to the fetus [1]. Individuals aged 40–60 years have a high risk of pemphigus vulgaris, and female patients with pemphigus might experience stillbirth [2] and even infertility [3]. Thus, NPV is rare in the clinical setting. NPV has various clinical manifestations, including skin/mucosa defects, blisters/erosions at birth, and extensive skin exfoliation. Here, we report a case of NPV characterized by tensive blisters and a negative Nikolsky sign. Two tensive blisters were observed on the back of the baby’s neck approximately 10 h after birth. Approximately 15 hours later, many tensive blisters rapidly appeared on several parts of the body. He had been delivered by eutocia at 37 weeks of gestation without complications. On physical examination, no abnormalities were noted. On dermatological examination, several tensive blisters of different sizes were seen scattered on the back of the neck, the inner thigh, and the perineum (Fig. 1). The oral and genital mucosae were not involved. The findings of the laboratory tests, including routine blood tests, urine tests, and liver and kidney function tests, were normal. In addition, the Treponema pallidum particle agglutination test and rapid plasma reagin test were negative. Since the mother previously had pemphigus vulgaris for 4 years, we considered the possibility of NPV and tested pemphigus titers. The titer of anti-desmoglein 3 in the peripheral blood was 66.5 and that of anti-desmoglein 1 was negative (reference, < 20 U/mL). Histopathological results showed intraepithelial blisters with suprabasal clefting, acantholysis, and a mixed dermal inflammatory infiltrate containing lymphocytes and neutrophils (Fig. 2). Direct immunofluorescence showed epidermal intercellular deposition of IgG and C3 (Fig. 3). The diagnosis of NPV was confirmed according to the clinical manifestations, laboratory examination results, and histopathological and immunopathological results of the baby, along with the mother’s medical history. The baby did