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Epidermal nevus syndrome associated with unusual neurological, ocular, and skeletal features
- Source :
- Indian Journal of Dermatology, Venereology and Leprology, Vol 78, Iss 4, Pp 480-483 (2012)
- Publication Year :
- 2012
- Publisher :
- Wolters Kluwer Medknow Publications, 2012.
-
Abstract
- 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.
- Subjects :
- Adult
Male
Pathology
medicine.medical_specialty
Skin Neoplasms
Choristoma
Eye Diseases
genetic structures
Bone Neoplasms
Dermatology
Middle cranial fossa
Arachnoid cyst
scleral osteoma
Young Adult
Seizures
medicine
epidermal nevus syndrome
lcsh:Dermatology
Nevus
Humans
Abnormalities, Multiple
Kyphosis
choristoma
Osteoma
Nevus, Pigmented
deformed calvarium
medicine.diagnostic_test
business.industry
Eye Neoplasms
Skull
Lacrimal Apparatus
Anatomy
Syndrome
lcsh:RL1-803
Epidermal nevus syndrome
medicine.disease
Arachnoid Cysts
Radiography
Infectious Diseases
medicine.anatomical_structure
Adipose Tissue
Scoliosis
Connective Tissue
Head and Neck Neoplasms
Skin biopsy
business
Subjects
Details
- Language :
- English
- ISSN :
- 03786323
- Volume :
- 78
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- Indian Journal of Dermatology, Venereology and Leprology
- Accession number :
- edsair.doi.dedup.....e3038d93b305c7a1d23012ff139b9f45