Angiofibroma
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ReferencesCutaneous angiofibromas kin ferskine yn ferskate dielen fan it lichem. Op it gesicht wurde se faak bekend as fibrous papules of adenoma sebaceum; op 'e penis wurden se pearly penile papules neamd; ûnder de nagel wurden se periungual angiofibromas (periungual angiofibromas) of Koenen‑tumors neamd; en yn 'e mûle wurden se oantsjutten as orale fibromas (oral fibromas). Gesichtsangiofibromas binne in prominint skaaimerk fan tuberous sclerosis (TS), in erflike oandwaning dy't ynfloed hat op 'e hûd, nieren, hert, brein en longen. Se ûntwikkelje typysk op it gesicht yn 'e jeugd as iere folwoeksenheid yn persoanen mei TS. It hawwen fan trije of mear facial angiofibromas of twa of mear periungual angiofibromas wurdt beskôge as wichtich yn diagnoaze TS. Derneist kinne meardere angiofibromas fan it gesicht oanwêzich wêze yn syndromen (multiple endocrine neoplasia type 1 (MEN-1) en Birt‑Hogg‑Dubé syndrome). Pearly penile papules wurden fûn lâns de koronale marzje en sulcus fan 'e penis. Se binne oanhâldende, symptomleaze bulten dy't faker sjoen wurde yn ûnbesnien manlju.
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) gesicht
Fibrous papule
2) penis
Pearly penile papule
3) ûnder in fingernagel of toenail
Periungual angiofibroma