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angiofibromas

Monday 27 April 2009

Definition: Angiofibroma (adenoma sebaceum or fibrous papule) are commonly found on the face in tuberous sclerosis. They are a dermal lesion with dense collagen and stellate and spindled cells surrounding blood vessels.

They can be found in association with Birt-Hogg-Dubé syndrome, and multiple endocrine neoplasia 1 (MEN1).

Tuberous sclerosis is associated with CNS tubers and SEGA, PEComas, cardiac rhabdomyomas and skin tumors (angiofibromas, Shagreen patches - connective tissue nevus, ash-leaf patches and periungual fibromas.)

Types

- cellular angiofibroma
- juvenile angiofibroma
- giant cell angiofibroma

Localization

- cutaneous angiofibroma
- angiofibroma of soft tissue

Predisposition

- tuberous sclerosis