Darier Disease associated with Cutis Verticis Gyrata

Abstract

Darier disease (DD) is an autosomal dominant genodermatosis characterized by cutaneous manifestations, such as keratotic papules present on the upper trunk and longitudinal erythronychia.

Cutis Verticis Gyrata, also known as pachyderma verticis gyrata, is a rare benign skin disorder characterized by the presence of convoluted folds, hypertrophy and skin hypermobility which generates deep grooves on the scalp of similar aspect to cerebral cortex gyri.

A 34-year-old male patient with no relevant pathological history or neuropsychiatric alterations presents with keratotic papules, developed during childhood, on the thorax (front and back), neck and intertriginous areas. During puberty, longitudinal folds on the head started to grow, extending anteroposteriorly and covering approximately 80% of the surface, without accompanying alterations on the scalp. This case presentation may consist of an association or a rare coincidence.