Angiofibroma (overview) D23.0

A group of benign tumours (hamartomas) on the skin and/or mucous membranes close to the skin that are neither clinically nor histologically detectable as a clear entity. In addition to connective tissue proliferation, the tumours show a prominent vascular richness. Angiofibromas are usually a random histological finding.

Angiofibromas as hamarous malformations:

• In the context of Bourneville-Pringle-phacomatosis, angiofibromas occur systematically (face, periungual fibromas) (the term Adenoma sebaceum for the acne-like angiofibromas of this genodermatosis is a misnomer).
• Tumour-associated genodermatoses include the angiofibromas and connective tissue nevi in MEN-I syndrome and Cowden syndrome.

A distinction is made between:

• Angiofibroma (Adenoma sebaceum)
• Angiofibroma, cell-rich
• Nasal papule, fibrous
• Fibroma, periunguales (Koenen tumor)
• Fibrokeratome, acquired, digital
• Wermer syndrome (multiple endocrine neoplasia type 1)
• Cowden's syndrome.

See below the respective clinical pictures.

The previously described "angiofibromas" are to be separated from the rare cell-rich angiofibroma, which occurs mainly as a tumour of the female (or more rarely male) genitals (especially the vulva).