Solitary angiokeratoma of the oral cavity

Angiokeratoma is a rare, cutaneous disorder that typically occurs at multiple sites and is often associated with a number of metabolic disorders. Although solitary cutaneous forms have been reported, to date localized lesions within the oral cavity have not been described. We report on an 82-year-old man with a history of squamous cell carcinoma of the ear and scalp who also had a solitary pigmented lesion of the oral cavity. Excisional biopsy of the buccal mucosal lesion showed features consistent with angiokeratoma. We report the first intra-oral solitary angiokeratoma and review the clinical and pathologic features of this unusual condition.