Observations on Keratosis Follicularis

Abstract
Keratosis follicularis, commonly referred to as Darier's disease, is a rare, persistent, cutaneous disorder characterized by malodorous, greasy, crusted, sometimes pustular, hyperkeratotic brownish-red papules located primarily on the scalp, face, retroauricular areas and the trunk. The disease occurs most commonly in males and usually begins in childhood. Sporadic cases are common but many instances of familial occurrence have been noted which were transmitted as a simple Mendelian dominant.1 Observations A few months ago three patients with keratosis follicularis were seen almost simultaneously at the University of Oklahoma Medical Center. At that time the observation was made that there were lesions, which appeared to be verruca plana, on the hands of all three of these patients with keratosis follicularis (Fig. 1). It was thought that this association was probably not coincidental. Biopsies were performed of several of these verruca-plana-like lesions. The histopathologic characteristics include hyperkeratosis without parakeratosis or vacuolization of