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...

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 ...

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...

1. Sugarman JL. Epidermal nevus syndromes. Semin Cutan Med Surg. 2007;26:221-30. 2. Solomon LM, Fretzin DF, Dewald RL. The epidermal nevus syndrome. Arch Dermatol. 1968;97:273-85. 3. Solomon LM, Esterly NB. Epidermal and other congenital organoid nevi. Curr Provi Pediatr. 1975;6:1-56. 4. Singal A, Dhaliwal U, Bhattacharyya SN , Rohatgi J, Singh N. Complex ocular choristomas in linear nevus seba...

The epidermal nevus syndrome (ENS) is an uncommon neurocutaneous disorder in which epidermal nevi are found in association with congenital abnormalities of the brain, eye, and/or skeleton. The association of epidermal nevi and neurologic abnormalities was comprehensively described by Schimmelpenning in 1957. Pavone et al. (1991) identified a homogeneous variant of ENS with hemimegalencephaly, g...

Becker nevus syndrome is a phenotype characterized by the fundamental presence of Becker's nevus with ipsilateral hypoplasia of the breast or other skin, skeletal and/or muscular disorders. This syndrome generally originates at birth, intensifies significantly in adolescence and is one of the syndromes that constitute epidermal nevus syndrome. To the best of our knowledge, this is the first cas...

A malignant transformation is known to occur in many nevi such as a sebaceous nevus or a basal cell nevus, but a verrucous epidermal nevus has only rarely been associated with neoplastic changes. Keratoacanthoma, multifocal papillary apocrine adenoma, multiple malignant eccrine poroma, basal cell carcinoma and cutaneous squamous cell carcinoma (CSCC) have all been reported to develop from a ver...

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...

Epidermal nevus syndrome (ENS) is a term used to describe the occurrence of an epidermal nevus in association with other extra-cutaneous developmental anomalies, most commonly involving the nervous and musculoskeletal systems. The nevus is classified on the basis of the main component which may be keratinocytic, sebaceous, follicular, apocrine, or eccrine. Most patients who present with ENS is ...

Epidermal nevus syndrome (ENS) is a rare condition characterized by congenital epidermal nevi (EN) associated abnormalities of other organs including central nervous system (CNS), skeletal system, eyes, and oral cavity (1). ENS is divided into a group of distinct disorders, based on the associated epidermal nevus and genetic inheritance pattern. Organoid nevus could be seen in some ENS, includi...