Epidermal nevus is benign skin growth that is often present at birth or within the first year of life. Rarely, it can develop later in childhood or adulthood. It typically appears as one or more oblong or linear growths that are skin colored, brown or gray in color. The surface can be wart-like or velvety with sharp borders. Epidermal nevi present at birth tend not to grow; however, those that appear after birth often enlarge in proportion with the child until puberty. Common locations for epidermal nevi include the trunk, limbs and neck.

Epidermal nevi are seen in 1 in 1000 live births and typically occur sporadically, although some familial cases have been noted. The prevalence of epidermal nevus is equal in males and females.

While most cases of epidermal nevi are uncomplicated, rare reports of basal and squamous cell carcinomas arising within epidermal nevi have been reported. This malignant transformation typically occurs in middle aged or elderly. In addition, the presence of multiple or extensive epidermal nevi can be associated with abnormalities of other organ systems including the bones, eyes and brain. When epidermal nevi are associated with these types of abnormalities it is called epidermal nevus syndrome.

Infants and children with extensive or multiple epidermal nevi should have a thorough work-up for systemic abnormalities and should be followed closely for attainment of developmental milestones. Available treatment options for epidermal nevi include surgical excision, laser ablation, topical therapies, and oral therapies. While surgical excision and laser ablation can be effective, scarring is inevitable. Topical and oral therapies do not usually result in resolution and only help decrease the thickness of the epidermal nevus.

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