Pemphigus vulgaris presenting as chronic vulvovaginal erosions: The importance of autoantibody testing: A case report

BACKGROUND: 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. CASE: 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. CONCLUSION: 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.