Angiofibroma - Angiofibrom
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References
Cutaneous angiofibromas kan vises i ulike deler av kroppen. På ansiktet er de ofte kjent som fibrøse papler eller adenom sebaceum; på penis heter de pearly penile papules ; under neglen kalles de periunguale angiofibromer eller Koenen-svulster; og i munnen blir de referert til som orale fibromer. Angiofibromer i ansiktet er et fremtredende trekk ved tuberous sclerosis (TS) , en arvelig lidelse som påvirker hud, nyrer, hjerte, hjerne og lunger. De utvikler seg vanligvis i ansiktet i barndommen eller tidlig voksen alder hos personer med TS. Å ha tre eller flere ansiktsangiofibromer eller to eller flere periunguale angiofibromer anses som viktig for diagnostisering av TS. I tillegg kan flere ansiktsangiofibromer være tilstede under tilstander (multiple endocrine neoplasia type 1 (MEN-1) og Birt-Hogg-Dubé syndrome) . Pearly penile papules finnes langs den koronale kanten og sulcus av penis. De er vedvarende, smertefrie støt som oftere sees hos uomskårne menn.
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) ansikt
Fibrous papule
2) penis
Pearly penile papule
3) under en negl eller tånegl
Periungual angiofibroma