Angiofibroma - Angiofibrooma
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ReferencesCutaneous angiofibromas voi esiintyä kehon eri osissa. Kasvoissa ne tunnetaan usein kuitumaisina papuleina tai adenoma sebaceum (adenoma sebaceum); peniksessä niitä kutsutaan pearly penile papules (pearly penile papules); kynnen alla niitä kutsutaan periungual angiofibroma (periungual angiofibroma) tai Koenen‑kasvaimet (Koenen tumors); ja suussa niitä kutsutaan oral fibroma (oral fibroma). Kasvojen angiofibroomat ovat tuberous sclerosis (TS) -taudin näkyvä piirre, perinnöllinen sairaus, joka vaikuttaa ihoon, munuaisiin, sydämeen, aivoihin ja keuhkoihin. Ne kehittyvät tyypillisesti kasvoille lapsuudessa tai varhaisessa aikuisiässä henkilöillä, joilla on TS. Kolmen tai useamman kasvojen angiofibrooman tai kahden tai useamman periungualisen angiofibroman (periungual angiofibroma) katsotaan olevan merkittävä TS:n diagnosointikriteeri. Lisäksi (multiple endocrine neoplasia type 1 (MEN‑1) ja Birt‑Hogg‑Dubé syndrome) voi esiintyä useita kasvojen angiofibroomeja. Pearly penile papules (pearly penile papules) löytyy peniksen koronaalisesta reunasta ja sulkasta. Ne ovat pysyviä, kivuttomia papuleita, joita esiintyy yleisemmin ympärileikkaamattomilla miehillä.
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) kasvot
Fibrous papule
2) penis
Pearly penile papule
3) kynnen tai varpaankynnen alla
Periungual angiofibroma