Meiri upplýsingar ― Íslenskur

Cutaneous angiofibromas getur birst á ýmsum stöðum líkamans. Á andliti eru þeir oft þekktir sem trefjablöðrur (fibrous papules) eða sebaceous adenoma (adenoma sebaceum); á typpinu heita þau pearly penile papules; undir nöglinni eru þau kölluð periungual angiofibra (periungual angiofibroma) eða Koenen æxli (Koenen tumors); og í munni er vísað til þeirra sem munnhúðar fibromar (oral fibromas). Angiofibras eru áberandi eiginleiki tuberous sclerosis (TS), arfgengs sjúkdóms sem hefur áhrif á húð, nýru, hjarta, heila og lungu. Þeir þróast venjulega í andliti á barnsaldri eða snemma á fullorðinsárum hjá einstaklingum með TS. Að vera með þrjá eða fleiri andlitsangiofibra (facial angiofibromas) eða tvö eða fleiri periungual angiofibra (periungual angiofibromas) er talin mikilvæg við greiningu TS. Þar að auki geta mörg andlitsangiofibra verið til staðar við aðstæður multiple endocrine neoplasia type 1 (MEN-1) og Birt‑Hogg‑Dubé syndrome. Pearly penile papules finnast meðfram kórónujaðrinum og sulcusi penis. Þetta eru smá, sársaukalausir hnökrar sem eru algengari hjá óumskornum körlum.
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.