Cutaneous Angiofibroma

Excerpt

Cutaneous angiofibroma is a benign skin tumor characterized by fibrovascular tissue and presents as a group of lesions with varied clinical appearances but consistent histological features. These benign fibrous neoplasms exhibit a proliferation of stellate and spindled cells, thin-walled blood vessels with dilated lumina in the dermis, and concentric collagen bundles. These growths typically manifest as small, firm, reddish, or flesh-colored papules, most commonly on the face (often referred to as fibrous papules or adenoma sebaceum), particularly around the nose and cheeks. However, they can also appear on other parts of the body, including the penis (as pearly penile papules), under the nails (as periungual angiofibromas or Koenen tumors), and in the mouth (as oral fibromas).

Facial angiofibromas are one of the most prominent clinical signs of tuberous sclerosis—an autosomal dominant disorder that affects the skin, kidneys, heart, brain, and lungs. In tuberous sclerosis, these angiofibromas typically emerge on the face during childhood or early adulthood (see Image. Facial Angiofibromas Observed in Tuberous Sclerosis). The presence of 3 or more facial angiofibromas or 2 or more periungual angiofibromas is major diagnostic criteria for the condition. Multiple facial angiofibromas are also observed in multiple endocrine neoplasia type 1 (MEN-1) and Birt–Hogg–Dubé syndrome.

Pearly penile papules are chronic, asymptomatic papules found on the coronal margin and sulcus of the penis and are more commonly observed in uncircumcised men.