Woolly hair with systematized epidermal nevus

Abstract

Woolly hair is a hair shaft disorder characterized by fine and tightly curled hair. Woolly hair could be syndromic (associated with systemic disease) or non-syndromic (not associated with any systemic disease). Woolly hair is described in association with many skin, dental, ophthalmic and cardiac anomalies but association of woolly hair with bilateral systematized epidermal nevus described in our case is first of its kind.

Ipsilateral Verrucous Epidermal Nevus with Woolly Hair Nevus

“…Epidermal nevi are nests of epidermal cells which are noted at birth or within the first year as a thin plaque or linear tan patch often along Blaschko lines[ 2 ] and have multiple clinical variants including a verrucous type. VEN occurs as a result of hamartomatous proliferation of the keratinocytes.…”