Angiofibroma - 혈관 섬유종
relevance score : -100.0%
ReferencesCutaneous angiofibromas는 신체의 다양한 부위에 나타날 수 있습니다. 얼굴에서는 주로 섬유성 구진이나 피지선종으로 알려져 있습니다. 음경에서는 pearly penile papules이라고 부릅니다. 손톱 아래에 발생한 경우에는 조갑주위 혈관섬유종 또는 Koenen tumor이라고 합니다. 구강 내에서는 구강 섬유종이라고 합니다. 안면 혈관섬유종은 피부, 신장, 심장, 뇌 및 폐에 영향을 미치는 유전 질환인 tuberous sclerosis (TS)의 대표적인 특징입니다. TS 환자는 일반적으로 아동기 또는 성인 초기 단계에서 얼굴에 병변이 나타납니다. 안면 혈관섬유종이 3개 이상이거나 조갑주위 혈관섬유종이 2개 이상인 경우 TS 진단에 중요한 근거가 됩니다. 또한, 다양한 안면 혈관섬유종은 여러 질환—multiple endocrine neoplasia type 1 (MEN‑1) 및 Birt‑Hogg‑Dubé syndrome—에서도 나타날 수 있습니다. pearly penile papules은 음경의 관상 가장자리와 고랑을 따라 발견되며, 포경수술을 하지 않은 남성에게서 더 흔히 보이는 지속적이고 통증이 없는 돌기입니다.
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) 얼굴 – Fibrous papule
2) 음경 – Pearly penile papule
3) 손톱·발톱 주변 – Periungual angiofibroma