- Facial Angiofibromas are benign cutaneous tumors. When they are multiple and have a symmetrical distribution on face, involving the cheeks, nasolabial folds and chin, angiofibromas are a hallmark of tuberous sclerosis complex (TSC or Bourneville disease) .
- Multiple facial angiofibromas are also found a sign of Birt-Hogg-Dubé syndrome (BHD) and multiple endocrine neoplasia type 1.
- Facial angiofibromas affect equally both men and women.
Clinical Presentation
- Angiofibromas are small, solitary, dome-shaped, skin-colored or red papules is the classic presentation of facial angiofibromas. On the surface of the papule, we can see little telangiectatic vessels.
- They're most commonly found on the central face, around the nose, and on the malar eminences. Lesions may bleed, alter the vision, and cause emotional stress
- Pearly penile papules are little papules that are pearly white, dome-shaped, and densely aggregated on the head of the penis in like a corona.
- Periungual Angiofibromas appear in early adulthood, on the lateral and the proximal nail fold. They can be painful. They cause a nail to be distorted.
- Oral fibromas occur on the gingiva, buccal and labial mucosa and the tongue (rare).
Diagnosis
- Angiofibroma is diagnosed using a combination of history, physical examination, and skin biopsy. If you suspect that your patients have Tuberous sclerosis complex, MEN-1, or Birt-Hogg-Dube, you should get genetic testing done as well as a thorough workup to look for malignancies related to those illnesses.
Treatment of Facial Angiofibromas
- Multiple Facial angiofibromas don't improve spontaneously.
- For the disfiguring, patients demand usually to be treated. There are many options for the treatment of facial angiofibroma
- Destructive approaches such as dermabrasion, surgical excision, and laser therapy.
- Recentely, several studies cited that topical rapamycin can be an effective treatment for facial angiofibroma associated with Tuberous Sclerosis.
Conclusion
Facial angiofibromas are benign tumors presenting as dome-shaped, firm, pink papules on the nose and adjoining central face.
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