Abstract: Darier's disease (DD) is an autosomal dominant genodermatosis, with manifestations of brown keratotic papules and plaques distributed in the seborrhoeic regions, covered with greasy scales. Histopathology features are dyskeratosis and acantholysis. The gene mutation of ATP2A2 is associated with DD. The new therapy methods of DD include new drugs, radiation therapy, allograft skin and gene therapy.

Key words: Darier's disease, pathogenesis, treatment progress