Angiofibroma
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relevance score : -100.0%
ReferencesA cutaneous angiofibroma a test különböző részein megjelenhet. Az arcon gyakran rostos papulákként vagy adenoma sebaceumként ismerik; a péniszen gyöngyszemes pénisz papulák (pearly penile papules); a köröm alatt ezeket periungual angiofibromáknak vagy Koenen‑daganatoknak nevezik; a szájban pedig oral fibromának (szájfibroma) nevezik őket. Az arc angiofibromái a tuberous sclerosis (TS), a bőrt, a veséket, a szívet, az agyat és a tüdőt érintő örökletes rendellenesség kiemelkedő jellemzői. Jellemzően az arcon alakulnak ki gyermekkorban vagy korai felnőttkorban a TS‑ben szenvedő egyéneknél. Három vagy több arc angiofibroma vagy két vagy több periungual angiofibroma jelenléte a TS diagnózisában jelentős. Ezenkívül több arc angiofibroma is jelen lehet bizonyos körülmények között (multiple endocrine neoplasia type 1 (MEN‑1) és Birt‑Hogg‑Dubé syndrome). Gyöngyszemes pénisz papulák (pearly penile papules) a pénisz koronális peremén és barázdáján találhatók. Ezek tartós, fájdalommentes dudorok, amelyek gyakrabban fordulnak elő körülmetéletlen férfiaknál.
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) arc – Fibrous papule
2) pénisz – Pearly penile papule
3) köröm vagy lábköröm alatt – Periungual angiofibroma