Development of hemangioma in a tongue harboring long-standing angiokeratoma circumscriptum

October 31, 2012
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Angiokeratoma is a very rare vascular lesion of the papillary dermis. It is characterized by vascular ectasia with overlying epidermal hyperkeratosis. The systemic form of angiokeratoma is associated with Fabry disease and fucosidosis. There are several localized forms. Tongue involvement is uncommon. Hemangiomas are tumors made up of capillaries; they grow by active endothelial proliferation as opposed to expansion of vascular spaces in vascular malformations. Lingual hemangiomas are usually indolent, but they can cause cosmetic deformities, recurrent hemorrhage, and functional problems with speaking, mastication, and deglutition. We report a case of angiokeratoma of the tongue with an underlying hemangioma in a 30-year-old woman. Angiokeratomas have been reported to develop over arteriovenous malformations and in the area of lymphangioma circumscriptum following repeated local trauma. To the best of our knowledge, the development of a lingual hemangioma in a patient with long-standing angiokeratomatous lesions has not been previously reported in the literature.


Angiokeratoma is an uncommon vascular lesion of the papillary dermis that is characterized by vascular ectasia with overlying epidermal hyperkeratosis.1-3 It can be either systemic or localized. The systemic form is associated with metabolic disorders, the most common of which are Fabry disease and fucosidosis. Angiokeratoma circumscriptum is a localized form that is usually found in the lower limbs. Tongue involvement is rare; when it does occur, it often presents as part of a systemic disorder.

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