Epidermal nevus syndrome with development of a mandibular ameloblastoma

Epidermal nevus syndrome (ENS) is a hamartoneoplastic syndrome characterize d by the association of epidermal nevi with abnormalities in other organ sy stems. We report a 32-year-old woman with ENS that, in addition to cutaneou s manifestations, showed red plaques on the maxillary and mandibular labial alveolar mucosa and a papillomatous lesion of the midline posterior hard p alate. Radiographic examination of the jaws was noncontributory. Approximat ely 5 years later, a follicular ameloblastoma developed in the mandible. Th e tumor showed duct-like cystic spaces, continuity with the overlying epith elium, and globular myxomatous areas in the connective tissue. The palatal lesion was diagnosed as papilloma, whereas the maxillary plaques showed non specific mucositis. The association of ameloblastoma with ENS is discussed. This is the second case of ENS associated with ameloblastoma reported in t he medical literature.