primary cutis verticis gyrata
Cutis verticis gyrata (CVG) is a progressive cutaneous disorder predominantly affecting males and characterized by hypertrophy and thickening of the skin of the scalp forming convoluted furrows with deep, tender, and cerebriform cutaneous folds. Hair is usually normal in the furrows and sparse on the folds. CG can be isolated (essential CVG) or associated with other abnormalities such as intellectual deficit, epilepsy, cataract, blindness, and deafness (non essential CVG). [ Orphanet:671 ]
Term info
- UMLS:C0263417 (Orphanet:671/e)
- SCTID:51603000 (MONDO:equivalentTo)
- Orphanet:671 (MONDO:equivalentTo)
- ICD9:757.39 (MONDO:relatedTo)
- GARD:0001643 (MONDO:equivalentTo)
gard_rare, disease_grouping, ordo_group_of_disorders
Cutis verticis gyrata (CVG) is a progressive cutaneous disorder predominantly affecting males and characterized by hypertrophy and thickening of the skin of the scalp forming convoluted furrows with deep, tender, and cerebriform cutaneous folds. Hair is usually normal in the furrows and sparse on the folds. CG can be isolated (essential CVG) or associated with other abnormalities such as intellectual deficit, epilepsy, cataract, blindness, and deafness (non essential CVG).
http://identifiers.org/snomedct/51603000, http://purl.obolibrary.org/obo/Orphanet_671, http://linkedlifedata.com/resource/umls/id/C0263417
cutis verticis gyrata
MONDO:0019033
https://rarediseases.info.nih.gov/diseases/1643/cutis-verticis-gyrata