Angiofibroma - Angiofibróm
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ReferencesCutaneous angiofibromas sa môžu objaviť v rôznych častiach tela. Na tvári sú často známe ako vláknité papuly alebo adenóm sebaceum; na penise sa nazývajú pearly penile papules; pod nechtom sa nazývajú periungválne angiofibromy alebo Koenenove nádory; a v ústach sa označujú ako orálne fibrómy. Tvárové angiofibromy sú prominentným znakom tuberous sclerosis (TS), dedičnej poruchy postihujúcej kožu, obličky, srdce, mozog a pľúca. Typicky sa vyvíjajú na tvári počas detstva alebo ranej dospelosti u jedincov s TS. Mať tri alebo viac angiofibromov tváre alebo dva alebo viac periungválnych angiofibromov sa považuje za významné pri diagnostike TS. Okrem toho môžu byť v podmienkach multiple endocrine neoplasia type 1 (MEN‑1) a Birt‑Hogg‑Dubé syndrome prítomné viaceré angiofibromy tváre. Pearly penile papules sa nachádzajú pozdĺž koronálneho okraja a sulku penisu. Sú to trvalé, bezbolestné hrbole, ktoré sa častejšie vyskytujú u neobrezaných mužov.
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) tvár – Fibrous papule
2) penis – Pearly penile papule
3) pod nechtom na ruke alebo nohe – Periungual angiofibroma