Epidermal Nevus Syndrome Associated with Brain Malformations and Medulloblastoma

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A severe form of epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma.

Here we report a boy with epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma. The patient had epidermal nevi and complicated brain malformations including macrocephaly with polymicrogyria, dysmorphic and enlarged midbrain tectum, enlarged cerebellar hemispheres with small and maloriented folia. The patient died after surgical resection o...

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Epidermal nevus syndrome: Report of a giant linear epidermal nevus

Linear epidermal nevi frequently follow the lines of Blaschko and may be confused with the verrucous stage of incontinentia pigmenti. Inflammatory epidermal nevus appears as a line array of pink to red papules usually on the lower limb (Buttock and hip) clinically resembling psoriasis or eczematous dermatitis. But inflammatory epidermal nevus has distinct histopathologic features. We report a c...

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Epidermal nevus associated with Lymphangioma: A case report

Epidermal nevi are cutaneous hamartomas developing from ectoderm and one of their types is verrucous epidermal nevus. During the neonatal period, they are pink or slightly pigmented and velvety. Later, they darken and the surface become more warty. When lesions are distributed on one side of body, it is termed nevus unis lateralis. Lymphangioma is a lymphatic malformation which manifests with f...

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Epidermal nevus syndrome associated with ocular symblepharon and gum hypertrophy

Epidermal nevi are congenital hamartomas of embryonal ectodermal origin classified on the basis of their main component. The compone keratinocytic. An estimated 1/3 of individuals with epidermal nevi have involvement of other organ systems; hence this condition is considered to be epidermal nevus syndrome. Solomon defined epidermal nevus syndrome as a sporadic neuro in the skin, brain, eyes and...

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epidermal nevus syndrome and dysplatic kidney disease.

epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. multicystic kidney disease has been very rarely reported in this syndrome. here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with wilms' tumor. according to this ...

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ژورنال

عنوان ژورنال: Pediatric Neurology Briefs

سال: 2013

ISSN: 2166-6482,1043-3155

DOI: 10.15844/pedneurbriefs-27-1-11