Angiofibroma - Angiofibrome
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ReferencesLes angiofibromes cutanés peuvent apparaître sur différentes parties du corps. Sur le visage, on les désigne souvent « papules fibreuses » ou « adénome sébacé » ; sur le pénis, ils sont appelés pearly penile papules ; sous l’ongle, on parle d’angiofibromes périunguéaux ou de tumeurs de Koenen ; et dans la bouche, on les nomme fibromes buccaux. Les angiofibromes faciaux constituent une caractéristique importante de la tuberous sclerosis (TS), une maladie héréditaire qui affecte la peau, les reins, le cœur, le cerveau et les poumons. Ils se développent généralement sur le visage pendant l’enfance ou au début de l’âge adulte chez les personnes atteintes de TS. La présence de trois angiofibromes faciaux ou plus, ou de deux angiofibromes péri‑unguéaux ou plus, est considérée comme significative pour le diagnostic du TS. De plus, plusieurs angiofibromes faciaux peuvent être observés dans le cadre de certaines affections, notamment le multiple endocrine neoplasia type 1 (MEN‑1) et le Birt‑Hogg‑Dubé syndrome. Les pearly penile papules se situent le long de la marge coronale et du sillon du pénis. Ce sont des bosses persistantes, indolores, plus fréquemment observées chez les hommes non circoncis.
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) Visage – Fibrous papule
2) Pénis – Pearly penile papule
3) Sous l’ongle ou l’ongle d’orteil – Periungual angiofibroma