Pemphigus vulgaris

ʻO Pemphigus vulgaris (PV) kahi maʻi autoimmune o ka ʻili. Hoʻopili ia i ka bliting a me nā ʻeha (erosions) o ka ʻili a me nā membrane mucous.

Hoʻopuka ka ʻōnaehana pale i nā mea pale kūʻē i nā protein ponoʻī i ka ʻili a me nā membrane mucous. ʻO kēia mau mea ʻino e wāwahi i nā pilina ma waena o nā hunaola ʻili. Ke alakaʻi nei kēia i ka hoʻokumu ʻia ʻana o kahi blister. ʻAʻole ʻike ʻia ke kumu kūpono.

I nā hihia maʻamau, pili ʻia ka pemphigus e kekahi mau lāʻau, e like me:

• ʻO kahi lāʻau i kapa ʻia penicillamine, kahi e hemo ai i kekahi mau mea mai ke koko (chelating agent)
• ʻO nā lāʻau lapaʻau kaomi koko i kapa ʻia nā mea kāohi ACE
• Nā lāʻau anti-inflammatory nonsteroidal (NSAIDs)

ʻAʻole maʻamau ʻo Pemphigus. Kū pinepine ia i waena o ka poʻe waena a ʻelemakule paha.

Ma kahi o 50% o ka poʻe me kēia ʻano e hoʻomohala mua i nā maʻi ʻeha a me nā ʻeha ma ka waha. Hahai ʻia kēia e nā ʻōpala ʻili. Hele paha a ʻeha ka ʻili.

ʻO ka ʻeha ʻili e wehewehe ʻia ma ke ʻano:

• Draining
• Oozy
• ʻO Crusting
• ʻO ka ʻili a hemo maʻalahi paha

Aia paha lākou:

• I ka waha a i ka ʻāʻī
• Ma ke poʻo, kumu, a i ʻole nā ​​ʻili ʻē aʻe

Hoʻokaʻawale maʻalahi ka ʻili ke ʻānai ʻia ka ʻili o ka ʻili i hoʻopili ʻole ʻia i ka ʻaoʻao me ka pulupulu a i ʻole ka manamana lima. Kāhea ʻia kēia he hōʻailona Nikolsky maikaʻi.

Hana pinepine ʻia kahi ʻili biopsy a me nā hoʻokolohua koko e hōʻoia i ka ʻike.

Pono paha nā hihia koʻikoʻi o pemphigus i ka mālama ʻana i ka ʻeha, e like me ka mālama ʻana no ka wela nui. Pono paha ka poʻe me PV e noho i ka haukapila a loaʻa ka mālama i loko o kahi hale puhi a i ʻole kahi mālama mālama nui.

Hoʻolālā ʻia ka mālama ʻana i ka hōʻemi ʻana i nā ʻōuli, e like me ka ʻeha. Hoʻopaʻa pū ʻia ia i ka pale ʻana i nā pilikia, nā maʻi hoʻi.

Hoʻopili paha ka mālama ʻana:

• Nā lāʻau Antibiotics a me nā lāʻau antifungal e kāohi a pale ai i nā maʻi
• Hāʻawi ʻia nā wai a me nā electrolytes ma o ke aa (IV) inā nui nā maʻi puʻupuʻu waha
• IV ka hānai ʻana inā nui nā maʻi maʻi ʻeha o ka waha
• Lohe ka waha nūhou (anesthetic) e hoʻoliʻiliʻi i ka ʻeha ulcer waha
• ʻO nā lāʻau ʻeha inā ʻaʻole lawa ka hōʻoluʻolu ʻeha kūloko

Pono ka laulā ākea (ʻōnaehana) e kāohi i ka pemphigus a pono e hoʻomaka i ka wā hiki. ʻO ka mālama ʻōnaehana pū kekahi:

• ʻO kahi lāʻau anti-inflammatory i kapa ʻia dapsone
• Corticosteroids
• Nā lāʻau i loaʻa i ke gula
• Nā lāʻau e kāohi i ka ʻōnaehana pale (e like me azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, a rituximab)

Hiki ke hoʻohana ʻia nā lāʻau antibiotic e mālama a pale ai i ka maʻi. Hoʻohana ʻia ʻo immunoglobulin intravenous (IVIg) i kekahi manawa.

Hiki ke hoʻohana ʻia ka Plasmapheresis me nā lāʻau lapaʻau ʻōnaehana e hōʻemi i ka nui o nā mea ʻino i loko o ke koko. ʻO Plasmapheresis kahi hana e hoʻoneʻe ʻia ai ka plasma-antody i loko o ke koko a hoʻololi ʻia me nā wai kahe a i ʻole ka plasma i hāʻawi ʻia.

ʻO nā hoʻomaʻamaʻa Ulcer a me ka blister me ka hoʻoluʻolu a hoʻomaloʻo ʻana i nā wai ʻono, nā lole pulu, a me nā ana like.

Me ka mālama ʻole ʻia, hiki i kēia ʻano ke hoʻoweliweli i ke ola. ʻO ka maʻi nui ke kumu pinepine o ka make.

Me ka mālama ʻana, mālama mau ʻia ka maʻi maʻi. ʻO nā ʻaoʻao ʻē aʻe o ka mālama ʻana he koʻikoʻi a hoʻopau ʻole paha.

Komo nā pilikia o PV i:

• Nā maʻi ʻili ʻelua
• Ka make nui loa ʻana
• Nā hopena o nā lāʻau
• Ka hohola ʻana o ka maʻi ma o ke kahe o ke koko (sepsis)

Pono kāu mea mālama ola e nānā i nā palapū i hōʻike ʻole ʻia.

Kāhea i kāu mea hāʻawi inā ua mālama ʻia ʻoe no PV a hoʻomōhala ʻoe i kekahi o nā ʻōuli aʻe.

• ʻO Chills
• Fiva
• Manaʻo maʻi laulā
• ʻEha pū
• ʻEha ka ʻili
• Pāhū a ulcer hou paha

• Pemphigus vulgaris ma ke kua
• Pemphigus vulgaris - nā liona i ka waha

Amagai M. Pemphigus. I: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. ʻEhā ed. Piladelapia, PA: Elsevier; 2018: chap 29.

ʻO Dinulos JGH. Nā maʻi Vesicular a me bullous. I: Dinulos JGH, hoʻoponopono. ʻO Dermatology Lapaʻau ʻo Habif. ʻEpekema 7 Piladelapia, PA: Elsevier; 2021: chap 16.

James WD, Elston DM, Hana iā JR, Rosenbach MA, Neuhaus IM. Nā maʻi dermatoses maʻi. I: James WD, Elston DM, mālama iā JR, Rosenbach MA, Neuhaus IM, eds. Nā maʻi o Andrew o ka ʻili. 13th ed. Piladelapia, PA: Elsevier; 2020: chap 21.

Patterson JW. ʻO ke kumu hoʻohālikelike vesiculobullous. I: Patterson JW, hoʻoponopono. ʻO Weedon's Skin Pathology. 5th lā. Piladelapia, PA: Elsevier; 2021: chap 7.