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Updated: Aug 10, 2026
11:22
Three-dimensional Imaging of Nociceptive Intraepidermal Nerve Fibers in Human Skin Biopsies
Published on: April 29, 2013
Neurological involvement in the epidermal naevus syndrome
Summary
This case study details an 18-year-old female with epidermal nevus syndrome experiencing stroke-like symptoms due to carotid artery occlusion. Novel findings include arterial occlusion, retinal abnormalities, and Raynaud's phenomenon in this context.
Area of Science:
- Neurology
- Vascular Medicine
- Genetics
Background:
- Epidermal nevus syndrome is a rare congenital disorder.
- Neurological complications are known but not fully characterized.
- Carotid artery occlusion is an uncommon presentation.
Purpose of the Study:
- To describe a unique case of epidermal nevus syndrome.
- To highlight unusual neurological and vascular manifestations.
- To discuss potential underlying mechanisms.
Main Methods:
- Case report of an 18-year-old female.
- Clinical presentation analysis.
- Review of relevant literature.
Main Results:
- The patient presented with dysphasia, hemiparesis, and sensory deficits.
- Right internal carotid artery occlusion was identified.
- Associated findings included abnormal retinal vessels and Raynaud's phenomenon.
- These vascular findings are novel for this syndrome.
Conclusions:
- Epidermal nevus syndrome can present with severe vascular complications.
- Dysplastic arteries may be the cause of arterial occlusion.
- Further research is needed to understand the vascular aspects of this syndrome.
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