Acantholytic dermatosis of the vagina: the diagnostic challenge of acantholytic disease in the genital region

Summary We report the case of a 24-year-old woman with an 8-month history of deep pelvic pain and postcoital bleeding. Examination revealed desquamation of the vaginal epithelium with tender fissured plaques in the vagina, initially thought to be vaginal intraepithelial neoplasia. Histology showed squamous mucosa with suprabasal acantholysis and hyperkeratosis, and no evidence of viral infection, dysplasia or malignancy. These findings were consistent with acantholytic dermatosis (AD), a rare lesion that resembles Hailey–Hailey and Darier disease histopathologically, but can be distinguished on a clinical basis. Vulval cases of AD are well recognized, but to our knowledge, this is the first reported case involving the vaginal epithelium alone.