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Primary non-essential cutis verticis gyrata

MedGen UID:
1653253
Concept ID:
C4755295
Disease or Syndrome
Synonym: primary non-essential cutis verticis gyrata
SNOMED CT: Primary non-essential cutis verticis gyrata (778044004)
 
Monarch Initiative: MONDO:0018168
Orphanet: ORPHA357225

Definition

A rare genetic dermis disorder with characteristics of slowly progressive thickening of the scalp, which becomes raised and forms ridges and furrows with symmetrical distribution resembling the cerebral gyri and cannot be flattened by traction or pressure, associated with ophthalmologic (for example congenital cataract) and/or neurological abnormalities (for example intellectual disability, epilepsy, microcephaly, encephalopathy). [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary non-essential cutis verticis gyrata

Recent clinical studies

Etiology

Zhu H, Feng ST, Zhang X, Ke Z, Zeng R, Yang X, Han J, Zhou H
Curr Med Imaging Rev 2019;15(9):906-910. doi: 10.2174/1573405614666181005113448. PMID: 32008538
Rahman A, Mahmood A
J Coll Physicians Surg Pak 2012 Feb;22(2):120-2. PMID: 22313654

Diagnosis

Teresa-Palacio M, Akpoke MA, Bah AB, Aldecoa-Bilbao V
BMJ Case Rep 2024 Feb 15;17(2) doi: 10.1136/bcr-2023-256735. PMID: 38359953Free PMC Article
Zeng W, Guo L
BMC Surg 2021 May 4;21(1):234. doi: 10.1186/s12893-021-01229-9. PMID: 33947392Free PMC Article
Zhu H, Feng ST, Zhang X, Ke Z, Zeng R, Yang X, Han J, Zhou H
Curr Med Imaging Rev 2019;15(9):906-910. doi: 10.2174/1573405614666181005113448. PMID: 32008538
Radwanski HN, Almeida MW, Pitanguy I
J Plast Reconstr Aesthet Surg 2009 Nov;62(11):e430-3. Epub 2008 Oct 23 doi: 10.1016/j.bjps.2008.06.062. PMID: 18951076
Larsen F, Birchall N
Australas J Dermatol 2007 May;48(2):91-4. doi: 10.1111/j.1440-0960.2007.00343.x. PMID: 17535195

Therapy

Nachinolcar SN, Pai V, Shukla P, Muthuprabhakaran K
Indian J Dermatol Venereol Leprol 2021 Sep-Oct;87(5):748. doi: 10.25259/IJDVL_1050_19. PMID: 34245526
Rahman A, Mahmood A
J Coll Physicians Surg Pak 2012 Feb;22(2):120-2. PMID: 22313654

Prognosis

Zeng W, Guo L
BMC Surg 2021 May 4;21(1):234. doi: 10.1186/s12893-021-01229-9. PMID: 33947392Free PMC Article
Zhu H, Feng ST, Zhang X, Ke Z, Zeng R, Yang X, Han J, Zhou H
Curr Med Imaging Rev 2019;15(9):906-910. doi: 10.2174/1573405614666181005113448. PMID: 32008538
Rahman A, Mahmood A
J Coll Physicians Surg Pak 2012 Feb;22(2):120-2. PMID: 22313654

Clinical prediction guides

Zeng W, Guo L
BMC Surg 2021 May 4;21(1):234. doi: 10.1186/s12893-021-01229-9. PMID: 33947392Free PMC Article
Zhu H, Feng ST, Zhang X, Ke Z, Zeng R, Yang X, Han J, Zhou H
Curr Med Imaging Rev 2019;15(9):906-910. doi: 10.2174/1573405614666181005113448. PMID: 32008538

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