Preferred Label : Bazex-Dupré-Christol syndrome;
ICD-11 definition : Bazex-Dupré-Christol syndrome is a rare genodermatosis with a predisposition to early-onset
basal cell carcinomas. It manifests during the neonatal period or during infancy with
hypotrichosis, hypohidrosis, and milia. Follicular atrophoderma is frequent and most
common on the dorsum of the hands and feet, the extensor surfaces of the elbows and
knees, and the face. Hypotrichosis affects the scalp and sometimes the eyebrows. The
milia papules and basal cell carcinomas are located predominantly on the face.;
ICD-11 synonym : Follicular atrophoderma and basal call carcinoma syndrome [Bazex];
Origin ID : 403754879;
Automatic exact mappings (from CISMeF team)
Bazex-Dupré-Christol syndrome is a rare genodermatosis with a predisposition to early-onset
basal cell carcinomas. It manifests during the neonatal period or during infancy with
hypotrichosis, hypohidrosis, and milia. Follicular atrophoderma is frequent and most
common on the dorsum of the hands and feet, the extensor surfaces of the elbows and
knees, and the face. Hypotrichosis affects the scalp and sometimes the eyebrows. The
milia papules and basal cell carcinomas are located predominantly on the face.
