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Definition of Darier disease

Medically reviewed by Dr. Otari Nergadze, Neurosurgeon | Updated: January 2026

Darier disease: A genetic skin disease characterized by an abnormality of the horny layer of the skin around the hair follicles. This disorder is inherited in an autosomal dominant manner and is due to mutation in a gene called ATP2A2 on chromosome 12. Also known as keratosis follicularis.