Darier's disease: Clinical and demographic features of nine cases

Abstract

Background and Design: Darier’s disease is a genetic disorder of keratinization with autosomal dominant inheritance. In the article presented here, demographical, clinical and histopathological findings and treatment outcome of Darier’s disease are discussed with 9 cases. Materials and Methods: We performed a retrospective study of all the patients diagnosed with Darier’s disease at the Department of Dermatology of Istanbul Training and Research Hospital, between 2008 and 2016. Results: During the observation period, we identified 9 patients with Darier’s disease; 6 males and 3 females with a mean age of 32.5 years. Two cases were from the same family (mother and child) and no family history was found in the other seven patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the head-neck and trunk. Four patients had hand involvement, five patients had nail lesions. Conclusion: Darier’s disease is a rare genodermatosis and should be considered in the differential diagnosis of dermatoses with keratotic papular lesions.