Angiofibroma (overview) D23.0

A group of benign tumors (hamartomas) on the skin and/or mucous membranes adjacent to the skin that cannot be clearly defined as a distinct entity, either clinically or histologically.  In addition to connective tissue proliferation, these tumors are characterized by a prominent vascular network. Angiofibromas are usually discovered incidentally during histological examination. 

Angiofibromas as hamartomatous malformations:

• In the context ofBourneville-Pringle phakomatosis, angiofibromas occur in a systematic pattern (face, periungual fibromas) (the term“adenoma sebaceum” for the acne-like angiofibromas of this genodermatosis is a misnomer).
• Tumor-associated genodermatoses include 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.

In case of systematized or multiple occurrence, removal by dermabrasion or laser ( _{CO2 laser}). Individual lesions can be removed surgically, e.g. by punch biopsy.

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).