Epidermal nevus and ameloblastoma: A rare association

Epidermal nevi are clinical manifestations of a malformation affecting the embryonic ectoderm. They appear as linear verrucous lesions and are more often located in the skin of the trunk or the extremities. Rarely, verrucous lesions affect the oral mucosa. Epidermal nevi can be a component of epidermal nevus syndrome (ENS), which is characterized by malformations involving several organs, such as the central nervous system. ENS is rarely associated with other solid tumors. We present the case of a patient affected by ENS who developed maxillary and mandibular ameloblastomas. Epidermal nevi were located in the left middle and lower third of the face and in the left cervical region, whereas the ameloblastomas arose within the left maxillary mucosa and within the left mandibular ramus. Features of the syndrome are described and the relevant literature is reviewed.