Digital Papillary Adenocarcinoma of the Vulva

Digital papillary adenocarcinoma (DPAC) is an uncommon cutaneous adnexal tumor that predominantly involves the skin of the fingers and toes and is associated with human papillomavirus 42 infection. The histologic features can be deceptively bland, but awareness of the entity in typical locations facilitates diagnosis. We describe a case of extradigital DPAC arising in vulvar skin, with the diagnosis confirmed using in situ hybridization for HPV42. Extradigital DPAC is very rare and presents a significant diagnostic challenge, particularly in this anatomical location where a range of other more common tumors with solid, tubular, and papillary architectures are much more frequently recognized.