Enfermedad de Darier

Abstract

Darier's disease is an autosomal dominant keratinization disorder. The clinical signs usually appear during the third decade of life; it is very rarely reported in early childhood. The most effective treatment is etretinate, an oral retinoid that is associated with adverse effects. Thus, its administration should be assessed on the basis of the severity of the clinical picture. We present the case of a boy who began to show signs of the disease at the age of 4 years, with considerable cutaneous involvement, but no familial history of interest.