Darier's disease (DD), also known as keratosis follicularis, is a rare hereditary acantholytic dermatosis, inherited in an autosomal dominant pattern, caused by mutations in the ATP2A2 [ATPase, Ca(++) transporting, cardiac muscle, slow-twitch 2] gene, with high penetrance but variable expressivity (1). This evidence concerns the gene ATP2A2 and dentin dysplasia.