Introduction: Linear epidermal nevi are sporadic hamartomatous alterations of the epidermis and superficial dermis that clinically appear as verrucous papules and plaques distributed in a linear pattern following Blaschko's lines. Their extent varies from unilateral involvement (nevus unius lateris) to extensive bilateral involvement (ichthyosis hystrix). Oral mucosal lesions have rarely been described.
Aims: We review the literature, focusing on the rare intraoral manifestations of linear epidermal nevus.
Case series: We present a series of five new cases with oral mucosal involvement. Four cases had associated cutaneous lesions and one case had oral lesions exclusively. Histopathologic evaluation of lesional tissue in four cases showed hyperkeratosis, acanthosis, epithelial hyperplasia, and papillomatosis. Dental abnormalities, consisting of enamel hypoplasia and congenitally missing teeth, were noted in one patient adjacent to the oral lesions.