Macri Angela, Syed Hasnain A., Tanner Laura S.
Sampson Regional Medical Center
Sheikh Zayed Hospital, Lahore
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 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.
皮肤血管纤维瘤是一种以纤维血管组织为特征的良性皮肤肿瘤，表现为一组具有不同临床表现但组织学特征一致的病变。这些良性纤维性肿瘤表现为星状细胞和梭形细胞的增殖、真皮内管腔扩张的薄壁血管以及同心胶原束。这些肿物通常表现为小的、坚实的、红色或肉色丘疹，最常见于面部（通常称为纤维丘疹或皮脂腺瘤），尤其是鼻周和脸颊周围。然而，它们也可出现在身体的其他部位，包括阴茎（如阴茎珍珠状丘疹）、指甲下（如甲周血管纤维瘤或科嫩瘤）以及口腔（如口腔纤维瘤）。面部血管纤维瘤是结节性硬化症最显著的临床体征之一，结节性硬化症是一种常染色体显性疾病，会影响皮肤、肾脏、心脏、大脑和肺部。在结节性硬化症中，这些血管纤维瘤通常在儿童期或成年早期出现在面部（见结节性硬化症中的面部血管纤维瘤）。面部出现3个或更多血管纤维瘤或2个或更多甲周血管纤维瘤是该病的主要诊断标准。在多发性内分泌肿瘤1型（MEN - 1）和Birt - Hogg - Dubé综合征中也可观察到多发性面部血管纤维瘤。阴茎珍珠状丘疹是在阴茎冠状缘和沟处发现的慢性、无症状丘疹，在未行包皮环切术的男性中更常见。
