ʻO Bullous pemphigoid kahi maʻi ʻili (dermatosis).

Hoʻohālikelike ʻia ka hope e ka hoʻomohala ʻana o nā huʻa nui ma nā papa erythematous (nā ʻulaʻula i ka ʻili). ʻO ke helehelena o kēia mau huʻa e alakaʻi i nā leona a ʻo ke kumu pinepine ia o ka aniani. (1)

He maʻi maʻi autoimmune ia, ka hopena o ka hoʻohaunaele ʻana o ka ʻōnaehana pale maʻi i ka mea i hoʻopilikia ʻia. Aia kēia rula o ka ʻōnaehana pale i ka hana ʻana i nā antibodies kūʻē i kona kino ponoʻī.

Kākaʻikahi kēia maʻi pilikino akā hiki ke lilo i mea koʻikoʻi. Koi ʻia ka lapaʻau lōʻihi. (1)

ʻOiai he maʻi maʻamau ia, ʻo ia nō ka mea maʻamau o nā dermatoses autoimmune bullous. (2)

ʻO kāna laha ka 1/40 (ka helu o nā hihia no kēlā me kēia kamaʻāina) a pili nui i ka ʻelemakule (ma ka awelika ma kahi o 000 mau makahiki, me ka piʻi iki o ka makaʻu no nā wahine).

Aia kekahi ʻano pēpē a pili i ke keiki i ka makahiki mua o kona ola. (3)

ʻO Bullous pemphigoid kahi dermatosis o ke kumu autoimmune. No laila ke kumuhana e ʻeha nei i kēia maʻi e hana i nā mea pale kūʻē i kāna mea ponoʻī (autoantibodies). Hoʻouka kēia i nā ʻano protein ʻelua: AgPB230 a me AgPB180 aia ma waena o nā papa ʻelua o ka ʻili (ma waena o nā dermis a me nā epidermis). Ma ke kumu o ka hoʻokaʻawale ma waena o kēia ʻelua ʻāpana o ka ʻili, alakaʻi kēia mau anti-auto-antibodies i ka hoʻokumu ʻia ʻana o nā huʻa o ka maʻi. (1)

ʻO nā ʻōuli atypical o bullous pemphigoid ke ʻano o nā huʻa nui (ma waena o 3 a me 4 mm) a me ke kala māmā. Hana ʻia kēia mau huʻa ma kahi e ʻulaʻula ai ka ʻili (erythematous), akā hiki ke ʻike ʻia ma ka ʻili olakino.

Hoʻonohonoho ʻia nā liona Epidermal i ka pahu a me nā lālā. Hoʻopakele pinepine ʻia ka maka. (1)

ʻO kahi pruritus o ka ʻili (ʻili), i kekahi manawa i ka wanaʻao ke puka mai nā huʻa, he mea nui nō hoʻi ia o kēia maʻi.

Ua hōʻike ʻia kekahi ʻano o nā maʻi: (1)

- ke ʻano laulā, nā ʻōuli o ia mau ʻano ke ʻano o nā huʻa keʻokeʻo nui a me ka aniani. ʻO kēia palapala ka mea maʻamau.

- ke ʻano vesicular, ka mea i wehewehe ʻia e ke ʻano o nā pōpō liʻiliʻi loa i nā lima me ka makaʻu ikaika. ʻAʻole emi kēia palapala.

- ke ʻano urticarial: e like me ka hōʻike a kona inoa, nā hopena i nā pā o ka hives e hoʻoulu nui ana i ka aniani.

- ke ʻano prurigo-like, ʻoi aku ka nui o ka pehu ʻana akā ka ikaika. Hiki i kēia ʻano maʻi ke hana i ka hiamoe ʻole i ke kumuhana pili. Eia hou, ʻaʻole nā ​​huʻa e ʻike ʻia i ke ʻano o ka prurigo ʻano akā ʻo nā pāpaʻa.

ʻAʻohe o kekahi mau maʻi i loaʻa i kekahi mau maʻi. Hoʻokumu kekahi i kahi ʻulaʻula iki, ʻili, a i ʻole huhū. ʻO ka mea hope loa, hoʻomohala nā hihia maʻamau i ka ʻulaʻula a me ka makaʻu nui.

Hiki i nā pālū ke haki a hana i nā ulcer a i ʻole nā ​​ʻeha hāmama. (4)

ʻO Bullous pemphigoid kahi dermatosis autoimmune.

ʻO kēia kumu o ka maʻi i hopena i ka hana ʻana o nā mea ʻohola (proteins of the immune system) e ke kino e kūʻē i kāna mau huna ponoʻī. Ke alakaʻi nei kēia hana o autoantibodies i ka luku ʻia ʻana o nā aʻa a me / a i ʻole nā ​​ʻokena a me nā hopena hoʻomāinoino.

ʻO ka wehewehe maoli no kēia hanana ʻaʻole i ʻike ʻia. Eia nō naʻe, he loulou a pololei ʻole paha kekahi mau mea me ka hoʻomohala ʻana o nā autoantibodies. ʻO kēia nā wahi, ka hormonal, nā lāʻau lapaʻau a i ʻole nā ​​kumu kūlohelohe. (1)

Kuhi ʻia kēia mau autoantibodies e ka mea i hoʻopilikia ʻia e kūʻē i nā protein ʻelua: BPAG1 (a i ʻole AgPB230) a me BPAG2 (a i ʻole AgPB180). He kuleana ko kēia mau protein i ka hoʻohui ma waena o dermis (papa lalo) a me nā epidermis (ʻāpana kiʻekiʻe). Ke hoʻouka ʻia nei kēia mau macromolecules e nā autoantibodies, hemo ka ʻili a hoʻomaka i nā ʻōhū. (2)

Eia hou, ʻaʻohe maʻi lele e hoʻopili ʻia me kēia pathology. (1)

Hoʻohui, ʻike pinepine ʻia nā ʻōuli a me ka manaʻo ʻole ʻia.

ʻAʻole ʻo Bullous pemphigoid: (3)

- kahi maʻi maʻi;

- kahi maʻi āpau;

- kahi kūlana e pili ana i ka nohona a i ʻole ka papaʻai.

ʻO Bullous pemphigoid kahi maʻi autoimmune, ma kēlā ʻano ʻaʻole ia he maʻi hoʻoilina.

Eia nō naʻe, he mea weliweli ke kū ʻana o kekahi mau gen. Loaʻa kekahi predisposition genetical.

ʻO kēia makaʻu o ka predisposition he, haʻahaʻa loa. (1)

ʻOiai ʻo ka awelika o ka ulu ʻana o ka maʻi ma kahi o 70, ʻo ka makahiki o ke kanaka he kumu pilikia hou aʻe paha no ka hoʻomohala ʻana i ka pemphigoid bullous.

Eia hou, ʻaʻole pono mākou e nānā i ka ʻoiaʻiʻo o kēia pathology i wehewehe ʻia ma o kahi ʻano pēpē. (3)

Hoʻohui ʻia, ʻike ʻia kahi predominance liʻiliʻi o ka maʻi i nā wahine. No laila ke kāne wahine e lilo ia i mea pilikia pili. (3)

ʻO ka ʻike ʻokoʻa o ka maʻi ka nānā nui ʻia: ka helehelena o nā huʻa maopopo i ka ʻili.

Hiki ke hōʻoia i kēia hōʻoia e kahi biopsy o ka ʻili (e lawe ana i kahi laʻana mai ka ʻili i hōʻino ʻia no ka loiloi).

Hiki ke hoʻohana ʻia ka pale o ka pale o ke kino i ka hōʻike o nā mea pale ma hope o ka hoʻāʻo ʻana i ke koko. (3)

ʻO nā kuʻina i kuhikuhi ʻia i ka pōʻaiapili o ke alo o ka bullous pemphigoid i mea e kaupalena ai i ka hoʻomohala ʻana o nā huʻa a e hoʻōla i nā huʻa i loaʻa i ka ʻili. (3)

ʻO ka lapaʻau maʻamau e pili ana i ka maʻi he ʻōnaehana corticosteroid ʻōnaehana.

Eia nō naʻe, no nā ʻano kūloko o bullous pemphigoid, topical corticosteroid therapy (e hana wale ana i kahi e hoʻohana ʻia ai ka lāʻau), hui ʻia me ka papa I dermatocorticoids (lāʻau i hoʻohana ʻia i ka mālama ʻili kūloko). (2)

ʻO ke kauka no nā lāʻau ʻalopika o ka ʻohana tetracycline (pili i kekahi manawa me ka ʻai ʻana o ka wikamina B) e kauka paha.

Hoʻomaʻa pinepine ʻia ka lāʻau no ka wā lōʻihi a maikaʻi. Eia hou, ʻike ʻia kahi hoʻi hou ʻana o ka maʻi i kekahi manawa ma hope o ka hoʻōki ʻana i ka lāʻau. (4)

Ma hope o ka hōʻoia ʻana o ke alo o ka bullous pemphigoid, koi nui ʻia ke kūkākūkā ʻana o kahi dermatologist. (3)