A 6-year-old girl presented with multiple, brownish-black, well-demarcated papillomatous plaques over face, neck, trunk, and arm, arranged in a linear configuration along Blaschko’s lines alongwith asymptomatic nodule of size 2cm x 1cm in her left eye, over limbus, since 6 months of age (Fig. 1). Radiological investigations showed no abnormality. Histopathology of skin lesion was consistent with the diagnosis of verrucous epidermal nevus and excised tissue from limbus showed features consistent with dermoid.

Fig. 1 Plaques over face, arm, and neck.

Epidermal nevus syndrome is a disease complex consisting of the association of an epidermal nevus with developmental abnormalities of the skin, eyes, nervous, skeletal, cardiovascular and urogenital systems. Around 9 to 30% patients have ocular abnormalities, the commonest of which are colobomas and choriostomas. Choriostomas include dermoid, lipodermoid, single tissue and complex choriostoma.

Linear verrucous epidermal nevus should be differentiated from other dermatoses presenting as linear hyperkeratotic or verrucous lesions: lichen planus, psoriasis, lichen straitus, and porokeratosis. Linear lichen planus is severely itchy and violaceous in color with slight scaling, psoriasis presents with thick silvery white scales. Lichen straitus is an asymptomatic and self-limiting disease, which usually resolves in 1 year. Linear porokeratosis can be differentiated by its pathognomonic cornoid lamellae.