Angiofibroma
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References
Cutaneous angiofibromas pot aparèixer en diverses parts del cos. A la cara, sovint es coneixen com a pàpules fibroses o adenoma sebaceum; al penis, es diuen pearly penile papules ; sota l'ungla, s'anomenen angiofibromes periungueals o tumors de Koenen; i a la boca, s'anomenen fibromes orals. Els angiofibromes facials són una característica destacada de tuberous sclerosis (TS) , un trastorn hereditari que afecta la pell, els ronyons, el cor, el cervell i els pulmons. Normalment es desenvolupen a la cara durant la infància o la primera edat adulta en persones amb ST. Tenir tres o més angiofibromes facials o dos o més angiofibromes periungueals es considera significatiu en el diagnòstic de TS. A més, poden estar presents múltiples angiofibromes facials en condicions (multiple endocrine neoplasia type 1 (MEN-1) i Birt-Hogg-Dubé syndrome) . Pearly penile papules es troben al llarg del marge coronal i del solc del penis. Són cops persistents i indolors que es veuen amb més freqüència en homes no circumciss.
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) cara
Fibrous papule
2) penis
Pearly penile papule
3) sota d'una ungla o ungla del peu
Periungual angiofibroma