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Wooly hair nevus

MedGen UID:
575391
Concept ID:
C0343114
Neoplastic Process
Synonyms: NEVUS, WOOLLY HAIR, SOMATIC; Woolly hair nevus
SNOMED CT: Wooly hair nevus (239124001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019311
OMIM®: 162900
Orphanet: ORPHA79414

Definition

Woolly hair nevus (WHN) is a rare non-familial hair anomaly characterized by kinky, tightly coiled, and hypopigmented fine hair with an average diameter of 0.5 cm, noted, since birth or during the first two years of life, in a localized circumscribed distribution on the scalp. Occassionally, WHN grows in areas observed to be alopecic in the neonatal period. WHN can be associated with features like ocular defects (persistent pupillary membrane, retinal defects), precocious puberty, and epidermal nevi. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWooly hair nevus
Follow this link to review classifications for Wooly hair nevus in Orphanet.

Recent clinical studies

Diagnosis

Kamat D, Sendhil Kumaran M
Int J Dermatol 2019 Oct;58(10):1197-1198. Epub 2019 Jan 29 doi: 10.1111/ijd.14380. PMID: 30697702

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