Darier disease, hypertrophic / vegetating type

Abstract

Background A hypertrophic / vegetating variant of Darier disease causing massive verruciform genital tumors is exceedingly rare. Case presentation A woman in her late 50s underwent wide resection of vulva and inguinal skin due to massive verruciform lesion. She had crusted / keratotic plaques with appearance of follicular keratosis keratosis (with a greasy, warty texture) in the back, neck, face, and extremities for 27 years. Previous biopsies from lesions elsewhere 27 years ago and along the years showed the same verruciform, pseudoepitheliomatous pattern, with no atypia. The diagnosis in those biopsies as well in the resected specimen was Darier disease, hypertrophic / vegetating type. All sampled areas showed the same pattern of parakeratosis, dyskeratosis (corps ronds and grains), suprabasal acantholysis and pseudoepitheliomatous hyperplasia with prominent anastomosing cords of elongated rete ridges (with a syringofibroadenoma silhouette). HPV hybridization situ was negative. Conclusion Awareness of hypertrophic variant of Darier disease is of important to avoid the diagnosis of malignant transformation in long term disease – which is an exceedingly rare event.