Cooccurrence of Darier’s Disease and Epilepsy: A Pediatric Case Report and Review of the Literature

Abstract

Darier's disease is a skin disorder characterized by multiple eruptions of hyperkeratosis or crusted papules at seborrheic areas with histologic acantholysis and dyskeratosis. It is caused by mutations in a single gene, being ATP2A2 and that is expressed in the skin and brain. The cooccurrence of various neurologic and psychiatric diseases with Darier's disease has been reported frequently in literature. They include mood disorders, epilepsy, encephalopathy, and schizophrenia. In this study, we report a pediatric case with the cooccurrence of Darier's disease and epilepsy. We also revised current English literature on this topic.

Unilateral segmental Darier disease following Blaschko lines: A case report

“…Penetrance is high but expressivity is variable so, if a patient is greatly affected it does not necessarily mean that other members will get this severe disease [2,3]. The gene is expressed also in the brain [4]. DD begins in childhood and affects men and women the same.…”