Primary essential cutis verticis gyrata

Orpha code: 357220OMIM code:

Definition

Primary essential cutis verticis gyrata is a rare, progressive dermis disorder characterized by thickening of the scalp resulting in redundancy of the skin which gives rise to folds and grooves that give the scalp a cerebriform appearance. Folds cannot be corrected by pressure or traction and typically are symmetric and extend anteroposteriorly from vertex to occiput and/or transversely in occipital region. Additional features may include mild subungual hyperkeratosis and distal onycholysis of the nail plates of the great toes. It is not associated with neurological and ophthalmological changes, nor with secondary causes.

Disease data
Classification

Disease

ORPHA code
357220
OMIM code
-
ICD10 code
Q82.8
ICD11 code
-

No additional description.

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