Primary cutis verticis gyrata

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Primary cutis verticis gyrata

ORPHA:671Clinical group

What it is

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.

Key facts

Age of onset
All ages
Classified as
Clinical group

Cross-references

GARD 1643MEDDRA 10080996MONDO 0019033MONDO 19033UMLS C0263417

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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