Angiofibroma - Anjiyofibrom
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References
Cutaneous angiofibromas vücudun çeşitli yerlerinde ortaya çıkabilir. Yüzde genellikle fibröz papüller veya adenoma sebaseum olarak bilinirler; peniste bunlara pearly penile papules denir; tırnak altında bulunanlara periungual anjiyofibromlar veya Koenen tümörleri denir; ve ağızda bulunanlara oral fibromlar denir. Yüz anjiyofibromları cildi, böbrekleri, kalbi, beyni ve akciğerleri etkileyen kalıtsal bir hastalık olan tuberous sclerosis (TS) hastalığının belirgin bir özelliğidir. Tipik olarak TS'li bireylerde çocukluk veya erken yetişkinlik döneminde yüz bölgesinde gelişirler. Üç veya daha fazla yüz anjiyofibromunun veya iki veya daha fazla periungual anjiyofibromun bulunması TS tanısında anlamlı kabul edilir. Ek olarak, (multiple endocrine neoplasia type 1 (MEN-1) ve Birt-Hogg-Dubé syndrome) durumlarda birden fazla yüz anjiyofibromu mevcut olabilir. Pearly penile papules penisin koronal kenarı ve sulkusu boyunca bulunur. Sünnetsiz erkeklerde daha sık görülen kalıcı, ağrısız şişliklerdir.
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) yüz
Fibrous papule
2) penis
Pearly penile papule
3) bir el veya ayak tırnağının altı
Periungual angiofibroma