Warty dyskeratoma of the vulva.

Suprabasal acantholysis of the epidermis is a histopathologic common denominator of diverse conditions such as pemphigus vulgaris, benign familial pemphigus (Hailey-Hailey), transient acantholytic dermatosis (Grover's disease), some actinic keratoses and cutaneous squamous carcinomas, keratosis follicularis (Darier's disease [DD]), and warty dyskeratoma (WD). Of these, WD rarely occurs in the vulva and is indistinguishable histologically from DD. However, DD is an inherited dermatosis (autosomal dominant) consisting of multiple keratotic papules on the face, trunk, and extremities, while WD occurs as an isolated, noninherited, single keratotic nodule mainly confined to the head and neck. We had the opportunity to study three patients who presented with isolated WD in the vulva. These patients had no other skin lesions and had no family history of DD or multiple dermatologic papulonodules. Histologically, all three vulvar lesions were indistinguishable from classic head and neck WD. WD must be clinically and histologically differentiated from vulvar dysplasia, bowenoid papulosis, squamous carcinoma, condyloma, and other viral-induced squamous lesions. WD should be added to the list of recognizable vulvar squamous lesions.