Primary cutis verticis gyrata

Orpha code: 671OMIM code:

Definition

A progressive cutaneous disorder predominantly affecting males, 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. It can be isolated or associated with other abnormalities, such as intellectual deficit, epilepsy, cataract, blindness, and deafness.

Disease data
Classification

Clinical group

ORPHA code
671
OMIM code
-
ICD10 code
Q82.8
ICD11 code
EE7Y

No additional description.

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