• Am J Dermatopathol · Aug 2003

    Case Reports

    Acral arteriovenous tumor developed within a nevus flammeus in a patient with Sturge-Weber syndrome.

    • Loreto Carrasco, Antonia Pastor, Carmen Fariña, Lucia Martín, Félix Manzarbeitia, and Luis Requena.
    • Department of Dermatology, Fundacíon Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
    • Am J Dermatopathol. 2003 Aug 1; 25 (4): 341-5.

    AbstractThe Sturge-Weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with Sturge-Weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with Sturge-Weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with Sturge-Weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with Sturge-Weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.

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