Angiofibroma
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relevance score : -100.0%
ReferencesCutaneous angiofibromas var parādīties dažādās ķermeņa daļās. Uz sejas tās bieži sauc par šķiedru papulām vai sebaceous adenoma; uz dzimumlocekļa tās sauc par pearly penile papules; zem nagiem tās sauc par periungual angiofibromas vai Koenen tumors; un mutē tās tiek sauktas par mutes fibromām. Sejas angiofibromas ir bieža pazīme tuberous sclerosis (TS) – iedzimta slimība, kas ietekmē ādu, nieres, sirdi, smadzenes un plaušas. Tās parasti parādās uz sejas bērnībā vai agrīnā pieaugušo vecumā cilvēkiem ar TS. Trīs vai vairāk sejas angiofibromas vai divas vai vairākas periungālas angiofibromas tiek uzskatītas par nozīmīgu pazīmi TS diagnostikā. Turklāt apstākļos, piemēram, multiple endocrine neoplasia type 1 (MEN‑1) un Birt‑Hogg‑Dubé syndrome, var būt vairākas sejas angiofibromas. Pearly penile papules atrodas gar dzimumlocekļa koronālo malu un vaginu. Tie ir pastāvīgi, nesāpīgi izciļņi, ko biežāk novēro neapgraizītiem vīriešiem.
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) seja – Fibrous papule
2) dzimumloceklis – Pearly penile papule
3) zem rokas vai kājas nagas – Periungual angiofibroma