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 CASE REPORT
Year : 2012  |  Volume : 78  |  Issue : 4  |  Page : 480--483

Epidermal nevus syndrome associated with unusual neurological, ocular, and skeletal features


1 Department of Dermatology and STD, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Delhi, India
2 Department of Ophthalmology, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Delhi, India
3 Department of Pathology, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Delhi, India

Correspondence Address:
Archana Singal
B-14, Law Apartments, Karkardooma, Delhi-110092
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0378-6323.98080

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Epidermal nevus syndrome (ENS) is a rare disease, the pathogenesis of which is largely elusive. We, hereby, report an exclusive case of a 20-year-old man with verrucous ENS presented with dark colored papules and plaques along the Blaschko's lines present over the head and neck area along with fleshy growth in both eyes since birth. Limb length discrepancy and kyphoscoliosis were remarkable. Skin biopsy was compatible with verrucous epidermal nevus while the biopsy of the ocular lesion confirmed complex choristoma. MRI brain revealed calcification in the right temporal lobe. Bilateral arachnoid cyst in the middle cranial fossa, scleral osteoma in the posterior part of the right eyeball, and deformed calvarium were evident on CECT skull and orbit. The present illustration emphasizes the importance of a punctilious work up of the case.






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