ABSTRACT

Darier’s disease also known as keratosis follicularis, dyskeratosis follicularis or benign dyskeratosis, is a rare autosomal dominant genodermatosis that primarily affects the skin and, to lesser extent, the oral mucosa. Lesions characterized by dirty, warty papul that commonly settled in face, trunk and flexural area of extremities. It is characterized by loss of adhesion between suprabasal epidermal keratinocytes (acantholysis) and abnormal keratinization (dyskeratosis). In this study we reported four cases of Darier’s disease of same family for the rarety of condition. By means of this case, data about this disorder will be able to review.