Angiofibroma
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ReferencesCutaneous angiofibromas podem aparecer em diversas partes do corpo. Na face, são frequentemente chamadas de pápulas fibrosas ou adenoma sebáceo; no pênis, são chamadas de pearly penile papules; sob a unha, são denominadas angiofibromas periungueais ou tumores de Koenen; e na boca são chamadas de fibromas orais. Os angiofibromas faciais são uma característica proeminente da tuberous sclerosis (TS), um distúrbio hereditário que afeta a pele, os rins, o coração, o cérebro e os pulmões. Eles normalmente se desenvolvem na face durante a infância ou no início da idade adulta em indivíduos com TS. Ter três ou mais angiofibromas faciais ou dois ou mais angiofibromas periungueais é considerado significativo para o diagnóstico de TS. Além disso, múltiplos angiofibromas faciais podem estar presentes nas condições multiple endocrine neoplasia type 1 (MEN-1) e Birt-Hogg-Dubé syndrome. Pearly penile papules são encontradas ao longo da margem coronal e do sulco do pênis. São nódulos persistentes e indolores, mais comumente observados em homens não circuncidados.
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) rosto – Fibrous papule
2) pênis – Pearly penile papule
3) ao redor da unha – Periungual angiofibroma