DR. RESHMA RAVIKANT SHAH

Abstract

Epidermal nevus syndrome (ENS) is a group of rare congenital neurocutaneous disorders characterized by presence of skin lesions known as epidermal nevi in association with extracutaneous abnormalities affecting brain, eyes and skeletal system most commonly. We, hereby, report a rare case of 3-month-old baby with epidermal nevus syndrome presented with multiple, linear verrucous plaques present over left hemibody along the blaschko lines, involving left side of face extending from chin, neck, abdomen, back and left upper and lower limbs along with fleshy overgrowth over left eye, lid coloboma and severe maldevelopment of same with associated frontal bossing.