Angiofibroma - Angiofibrom
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References
Cutaneous angiofibromas kan förekomma i olika delar av kroppen. I ansiktet är de ofta kända som fibrösa papler eller adenom sebaceum; på penis kallas de pearly penile papules ; under nageln kallas de periunguala angiofibromas eller Koenen-tumörer; och i munnen kallas de orala fibromer. Angiofibromer i ansiktet är ett framträdande kännetecken för tuberous sclerosis (TS) , en ärftlig sjukdom som påverkar hud, njurar, hjärta, hjärna och lungor. De utvecklas vanligtvis i ansiktet under barndomen eller tidig vuxen ålder hos individer med TS. Att ha tre eller flera angiofibromer i ansiktet eller två eller flera periunguala angiofibromer anses vara av betydelse för diagnostisering av TS. Dessutom kan flera ansiktsangiofibromer förekomma under tillstånd (multiple endocrine neoplasia type 1 (MEN-1) och Birt-Hogg-Dubé syndrome) . Pearly penile papules finns längs penisens kranskant och sulcus. De är ihållande, smärtfria knölar som oftare ses hos oomskurna män.
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) ansikte
Fibrous papule
2) penis
Pearly penile papule
3) under en finger- eller tånagel
Periungual angiofibroma