Preferred Label : Darier disease;
ICD-11 definition : Darier disease is a keratinization disorder characterized by the development of keratotic
papules in seborrhoeic areas and specific nail anomalies. Skin lesions include greasy
and colored (yellow-brown or brown) keratotic papules, which may be isolated or grouped
forming plaques that often become infected and malodorous, and are responsible for
major discomfort. Palms and soles frequently exhibit small pits or punctate keratoses
that are specific for Darier disease.;
ICD-11 synonym : Darier-White disease; Darier disease (MIM 124200); Keratosis follicularis; Dyskeratosis follicularis;
Origin ID : 643994486;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
Darier disease is a keratinization disorder characterized by the development of keratotic
papules in seborrhoeic areas and specific nail anomalies. Skin lesions include greasy
and colored (yellow-brown or brown) keratotic papules, which may be isolated or grouped
forming plaques that often become infected and malodorous, and are responsible for
major discomfort. Palms and soles frequently exhibit small pits or punctate keratoses
that are specific for Darier disease.
