Davis Susan A, Davis Loretta S
J Reprod Med. 2016 Nov-Dec;61(11-12):589-91.
Pemphigus vulgaris is a rare autoimmune bullous disease characterized by mucosal erosions. Pathogenic autoantibodies are directed against desmoglein 3, leading to intraepidermal blister formation. The oral cavity is typically the initial and most common site of involvement, but the female genital tract can also be involved. We present a case of pemphigus vulgaris presenting as chronic vulvovaginal erosions.
A 59-year-old woman presented with a rare sore in her mouth and an 18-month history of vaginal burning, irritation, and white discharge. Previous workup included negative vaginal culture for yeast, nondiagnostic vulvar biopsy, and normal cervical smear. Clobetasol cream had provided minimal relief. Biopsy of an erosion of the vulvar vestibule showing loss of epidermis with acantholysis prompted indirect immunofluorescence testing. High titer immunoglobulin G antibodies directed against epithelial cell surfaces confirmed a diagnosis of pemphigus vulgaris, explaining an 18-month history of isolated chronic vulvovaginal erosions.
Female genital tract involvement by pemphigus vulgaris usually occurs in the setting of extensive involvement at other sites, most notably the oral mucosa. Primary involvement of the genital mucosa is unusual. Confirmatory diagnosis is made with testing for autoantibodies, either with direct or indirect immunofluorescence or enzyme-linked immunosorbent assay.
寻常型天疱疮是一种罕见的自身免疫性大疱性疾病，其特征为黏膜糜烂。致病性自身抗体针对桥粒芯糖蛋白3，导致表皮内水疱形成。口腔通常是最初且最常见的受累部位，但女性生殖道也可受累。我们报告一例以慢性外阴阴道糜烂为表现的寻常型天疱疮病例。
一名59岁女性因口腔出现罕见溃疡以及有18个月的阴道烧灼感、刺激感和白色分泌物病史前来就诊。既往检查包括阴道酵母菌培养阴性、外阴活检未明确诊断以及宫颈涂片正常。氯倍他索乳膏治疗效果甚微。对外阴前庭糜烂处进行活检，显示表皮缺失伴棘层松解，遂进行间接免疫荧光检测。针对上皮细胞表面的高滴度免疫球蛋白G抗体证实为寻常型天疱疮，解释了长达18个月的孤立性慢性外阴阴道糜烂病史。
寻常型天疱疮累及女性生殖道通常发生在其他部位广泛受累的情况下，最显著的是口腔黏膜。生殖器黏膜原发性受累并不常见。通过直接或间接免疫荧光或酶联免疫吸附测定检测自身抗体可确诊。
