Darier-White disease (keratosis follicularis) is a rare disorder of keratinization involving the epidermis, mucous membranes, and nails. It is said to occur as a result of mutation in the ATP2A2 gene located on chromosome 12q23-24.1. In this article we present the case of two brothers with exacerbations of Darier-White disease who responded very well to systemic retinoids without any side effects within 2 weeks of commencing treatment.
Keywords: Darier-White disease; isotretinoin; keratosis follicularis.