Cutaneous Angiofibroma

Excerpt

Cutaneous angiofibroma is a term used to define a group of lesions with different clinical presentations but with the same histologic findings. They are benign fibrous neoplasms comprised of a proliferation of stellate and spindled cells, thin-walled blood vessels with dilated lumina in the dermis, and concentric collagen bundles. Cutaneous angiofibroma can be located on different body areas, including the face, commonly called fibrous papules or adenoma sebaceum. On the penis, where they are called pearly penile papules; underneath the nail, where they are called periungual angiofibroma or Koenen tumors, and in the mouth where they are called oral fibromas. Facial angiofibromas are considered 1 of the most obvious clinical presentations of tuberous sclerosis. Tuberous sclerosis is an autosomal dominant hamartomatous disorder that affects the skin, kidneys, heart, brain, and lungs. With tuberous sclerosis, angiofibromas typically arise on the face in childhood and early adulthood. Both facial angiofibroma (greater than or equal to 3 needed) and periungual angiofibroma (greater than or equal to 2 needed) are 2 of the major criteria for tuberous sclerosis. Multiple facial angiofibromas are also found in multiple endocrine neoplasia type 1 (MEN-1) and Birt-Hogg-Dube syndrome. Pearly penile papules are chronic, asymptomatic papules found on the coronal margin and sulcus of the penis. They are more common in uncircumcised men (See Image. Tuberous Sclerosis Angiofibromas).