Epidermal nevi (ILVEN)

Published on 11/06/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

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Red, warty, curvilinear, unilateral plaque on toddler’s scrotum, adjacent abdomen and thigh.

Linear epidermal nevus on the plantar surface of child’s foot.

CLINICAL FEATURES

Epidermal nevi are linear or curvilinear areas of red papules with an irregular, rough surface. The majority of epidermal nevi are present at birth and 95% by age 7 years. Initially, an epidermal nevus may be barely palpable and appear as a confluence of smooth-topped papules. With time, the surface becomes warty.

Most epidermal nevi are small, 2 to 5 cm in length, but occasionally appear as long unilateral warty streaks covering one-half of the body (nevus unius lateris). They may rarely be widespread over the entire body (icthyosis hystrix). Epidermal nevi may become very red and itchy, with recurrent episodes of inflammation. This variant is called inflammatory linear verrucous epidermal nevus (ILVEN).

A majority of children with epidermal nevi have no other findings but up to one-third will have a variety of associated abnormalities. A number of cutaneous findings including café-au-lait spots, hypopigmented macules and congenital pigmented nevi can be seen. Skeletal defects, seizure disorders, mental retardation and ocular anomalies have been associated. All children with epidermal nevi should have a detailed history and physical examination including a careful neurologic and ophthalmologic examination.

Children with widespread skin involvement have a higher risk of associated abnormalities and should have referral to a children’s genetic center or multidiscipline clinic. The pattern of skin involvement follows the embryonic skin lines of Blaschko, indicating genetic mosaicism. Affected skin areas may have mutations of the FGFR 3 gene or keratin 1 or 10 genes.

Small epidermal nevi may be confused with warts.

TREATMENT

Small lesions may be excised. For the child with widespread skin involvement, topical keratolytics such as retinoid creams may be useful when combined with topical lubrication. The affected areas will revert to their previous state when topical therapy is discontinued. Oral retinoids have been helpful in severe involvement.