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Epidermal nevus syndrome: MR of intracranial involvement
S Lazzeri1, M Mascalchi, M Cellerini
1Dipartimento di Fisiopatologia Clinica, Universitá di Firenze, Italy.
AJNR. American Journal of Neuroradiology
|September 1, 1993
Abstract:
Focal thickening of the calvarium, hypoplasia of the white matter, cortical calcifications, and a leptomeningeal drape that enhanced after contrast injection were demonstrated by MR in the parietooccipital region ipsilateral to a facial sebaceous nevus in a patient with epidermal nevus syndrome.
Insights
Epidermal nevus syndrome can manifest with brain abnormalities, including focal calvarial thickening and white matter hypoplasia, alongside characteristic skin findings like facial sebaceous nevi.
Area of Science:
- Neuroimaging
- Dermatology
- Genetics
Background:
- Epidermal nevus syndrome (ENS) is a rare congenital disorder characterized by the development of epidermal nevi.
- ENS can be associated with extracutaneous manifestations, including central nervous system (CNS) abnormalities.