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primary cutis verticis gyrata

^ http://purl.obolibrary.org/obo/MONDO_0019033


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

database cross reference
  • UMLS:C0263417 (Orphanet:671/e)
  • SCTID:51603000 (MONDO:equivalentTo)
  • Orphanet:671 (MONDO:equivalentTo)
  • ICD9:757.39 (MONDO:relatedTo)
  • GARD:0001643 (MONDO:equivalentTo)
Subsets

gard_rare, disease_grouping, ordo_group_of_disorders

definition

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).

exactMatch

http://identifiers.org/snomedct/51603000, http://purl.obolibrary.org/obo/Orphanet_671, http://linkedlifedata.com/resource/umls/id/C0263417

has exact synonym

cutis verticis gyrata

id

MONDO:0019033

seeAlso

https://rarediseases.info.nih.gov/diseases/1643/cutis-verticis-gyrata

Term relations

Subclass of: