English

Abstract

INTRODUCTION: Darier disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare congenital acantholytic disorder which is transmitted as an autosomal dominant inheritance with variable expressivity but complete penetrance. Darier disease was initially described by Prince Marrow in 1886 and simultaneously by Darier and White in 1889, independently. The prevalence of this disorder in the population is 1:100,000, most often affecting males.1-3 Its manifestations appear in childhood and adolescence. It is characterized by persistent eruption of hyperkeratotic papules primarily over seborrheic areas, nail changes like pathognomonic triangular nicking at the free edges and histologically suprabasal acantholysis with dyskeratosis. Associated anomalies have been described in the literature, including mental retardation and psychosis.4