Darier Disease

**Semantic type:** Disease or Syndrome

**Definition:** An autosomal dominant inherited chronic skin disorder caused by mutations in the ATP2A2 gene. It is characterized by the development of yellow-brown keratotic skin papules in the neck, ears, forehead, chest, back and groin. It is associated with fragility of the free margins of the nails.

**Synonyms:** - Keratosis Follicularis