Darier disease, hypertrophic / vegetating type

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