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Darier disease

Disease definition

A rare, genetic keratinization disorder which is classically characterized by keratotic papules, acral pits, and acral wart-like lesions that can be associated with a trigger, and may occur anywhere on the body (including mucosal surfaces). Extracutaneous manifestations may include, nail anomalies, blepharitis, dry eye, neuropsychiatric illness and, recurrent parotid gland obstruction and xerostomia.


Classification level: Disorder
  • Synonym(s):
    • Darier-White disease
    • Keratosis follicularis
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult, Childhood, Adolescent
  • ICD-10: Q82.8
  • ICD-11: EC20.2
  • OMIM: 124200
  • UMLS: C0022595
  • MeSH: D007644
  • GARD: 6243
  • MedDRA: 10023369

Detailed information

General public

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.