Angiofibroma
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ReferencesCutaneous angiofibromas dapat muncul di berbagai bagian tubuh. Di wajah, sering disebut papula berserat atau adenoma sebaceum; di penis disebut pearly penile papules; di bawah kuku, disebut angiofibroma periungual atau tumor Koenen; dan di mulut, disebut fibroma mulut. Angiofibroma wajah merupakan ciri utama tuberous sclerosis (TS), kelainan bawaan yang menyerang kulit, ginjal, jantung, otak, dan paru-paru. Lesi ini biasanya muncul di wajah pada masa kanak‑kanak atau awal dewasa pada individu dengan TS. Kehadiran tiga atau lebih angiofibroma wajah atau dua atau lebih angiofibroma periungual dianggap signifikan untuk diagnosis TS. Selain itu, beberapa angiofibroma wajah dapat muncul pada kondisi multiple endocrine neoplasia type 1 (MEN-1) dan Birt‑Hogg‑Dubé syndrome. Pearly penile papules ditemukan di sepanjang tepi koronal dan sulkus penis. Benjolan ini bersifat persisten, tidak menimbulkan rasa sakit, dan lebih sering terlihat pada pria yang tidak disunat.
Cutaneous angiofibromas can be located on different areas of the body including the face where they are 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 angiofibromas or Koenen tumors, and in the mouth where they are called oral fibromas. Facial angiofibromas are considered one of the most obvious clinical presentations of tuberous sclerosis (TS), an autosomal dominant hamartomatous disorder that affects the skin, kidneys, heart, brain, and lungs. With TS, angiofibromas typically arise on the face in childhood and early adulthood. Both facial angiofibromas (greater than or equal to 3 needed) and periungual angiofibroma (greater than or equal to 2 needed) are 2 of the major criteria for TS. 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.
Cutaneous angiofibromas can be located on different areas of the body including the face where they are 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 angiofibromas or Koenen tumors, and in the mouth where they are called oral fibromas. Facial angiofibromas are considered one of the most obvious clinical presentations of tuberous sclerosis (TS), an autosomal dominant hamartomatous disorder that affects the skin, kidneys, heart, brain, and lungs. With TS, angiofibromas typically arise on the face in childhood and early adulthood. Both facial angiofibromas (greater than or equal to 3 needed) and periungual angiofibroma (greater than or equal to 2 needed) are 2 of the major criteria for TS. 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.
1) Wajah – Fibrous papule
2) Penis – Pearly penile papule
3) Di bawah kuku jari tangan atau kaki – Periungual angiofibroma