Unusual papillary lesion of the ventral tongue: case report of solitary angiokeratoma of the oral cavity.

Many lesions in the oral cavity may present with a papillary or pebbly clinical appearance. Although the great majority of these papillary lesions are histologically diagnosed as squamous or viral papillomas, there are occasional cases of other more unusual possibilities. Angiokeratomas are uncommon vascular lesions that often present clinically as papillomas. They may also present with other varying clinical appearances that range from pigmented lesions to hemangiomas. However, all forms demonstrate a characteristic microscopic appearance consisting of hyperkeratotic, hyperplastic epithelium covering connective tissue with abundant blood vessels that are sharply confined to the connective tissue papillae. Angiokeratomas generally involve the skin and are often associated with an underlying systemic metabolic disease such as Fabry's disease or fucosidosis. Patients with these systemic diseases may have multiple lesions, with possible involvement of the oral mucosa. However, solitary lesions involving only the oral cavity are rare; only eight previous cases have been documented. In this paper, we describe a case of solitary angiokeratoma presenting as a papillary lesion on the ventral tongue of an 18-year-old male. The lesion was surgically excised and no recurrence has been reported to date. Although this patient had no other lesions or systemic issues, we stress the importance of evaluating a patient with a diagnosis of angiokeratoma of the oral cavity for underlying systemic metabolic disease.