Angiofibroma - Angiofibrom
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ReferencesCutaneous angiofibromas se mohou objevit v různých částech těla. Na obličeji jsou často známé jako vláknité papuly nebo adenoma sebaceum; na penisu se jim říká pearly penile papules; pod nehtem se nazývají periunguální angiofibromy nebo Koenenovy nádory; a v ústech se označují jako orální fibromy. Obličejové angiofibromy jsou prominentním znakem tuberous sclerosis (TS), dědičné poruchy postihující kůži, ledviny, srdce, mozek a plíce. Typicky se vyvíjejí na obličeji během dětství nebo rané dospělosti u jedinců s TS. Přítomnost tří nebo více obličejových angiofibromů nebo dvou a více periunguálních angiofibromů se považuje za významné diagnostické kritérium pro TS. Kromě toho mohou být v souvislosti s multiple endocrine neoplasia type 1 (MEN-1) a Birt-Hogg-Dubé syndrome přítomny mnohočetné obličejové angiofibromy. Pearly penile papules se nacházejí podél koronálního okraje a sulku penisu. Jedná se o trvalé, nebolestivé hrboly, které se častěji vyskytují u neobřezaných mužů.
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) obličej – Fibrous papule
2) penis – Pearly penile papule
3) pod nehet na ruce nebo na noze – Periungual angiofibroma