Epidermal nevus syndrome with development of a mandibular ameloblastoma

Epidermal nevus syndrome (ENS) is a hamartoneoplastic syndrome characterized by the association of epidermal nevi with abnormalities in other organ systems. We report a 32-year-old woman with ENS that, in addition to cutaneous manifestations, showed red plaques on the maxillary and mandibular labial alveolar mucosa and a papillomatous lesion of the midline posterior hard palate. Radiographic examination of the jaws was noncontributory. Approximately 5 years later, a follicular ameloblastoma developed in the mandible. The tumor showed duct-like cystic spaces, continuity with the overlying epithelium, and globular myxomatous areas in the connective tissue. The palatal lesion was diagnosed as papilloma, whereas the maxillary plaques showed nonspecific mucositis. The association of ameloblastoma with ENS is discussed. This is the second case of ENS associated with ameloblastoma reported in the medical literature.