I-Pemphigus vulgaris

I-Pemphigus vulgaris (PV) ukuphazamiseka okuzenzakalelayo kwesikhumba. Kubandakanya ukugqabhuka nezilonda (ukuguguleka) kwesikhumba nolwelwesi lwamafinyila.

Amasosha omzimba akhiqiza amasosha omzimba aphikisana namaprotheni athile esikhunjeni nasezikhungweni zomzimba. La masosha omzimba aphula izibopho eziphakathi kwamaseli esikhumba. Lokhu kuholela ekwakhekeni kweblister. Imbangela ngqo ayikaziwa.

Ezimweni ezingavamile, i-pemphigus ibangelwa eminye imithi, kufaka phakathi:

• Umuthi obizwa nge-penicillamine, osusa izinto ezithile egazini (i-chelating agent)
• Imithi yengcindezi yegazi ebizwa nge-ACE inhibitors
• Izidakamizwa ezingezona ukuvuvukala (ama-NSAID)

I-Pemphigus ayijwayelekile. Ngokuvamile kwenzeka kubantu abaneminyaka ephakathi nendawo noma asebekhulile.

Cishe ama-50% abantu abanalesi simo baqala babe namabhamuza abuhlungu nezilonda emlonyeni. Lokhu kulandelwa ngamabhamuza esikhumba. Izilonda zesikhumba zingahle zidlule.

Izilonda zesikhumba zingachazwa ngokuthi:

• Ukukhipha amanzi
• Iyasondeza
• Ukuqhekeka
• Ukuxebuka noma ukuhlukaniswa kalula

Zingatholakala:

• Emlonyeni nasemphinjeni
• Esikhumbeni, esiqwini, noma kwezinye izindawo zesikhumba

Isikhumba sihlukana kalula lapho ingaphezulu lesikhumba esingathintekile ligcotshwa eceleni nge-swab kakotini noma ngomunwe. Lokhu kubizwa ngokuthi uphawu oluhle lukaNikolsky.

Ukuhlolwa kwesikhumba nokuhlolwa kwegazi kuvame ukwenziwa ukuqinisekisa ukuxilongwa.

Izimo ezinzima zepemphigus zingadinga ukuphathwa kwamanxeba, okufana nokwelashwa kokusha okukhulu. Abantu abane-PV bangadinga ukuhlala esibhedlela futhi bathole ukunakekelwa egumbini lokusha noma egumbini labagula kakhulu.

Ukwelashwa kuhloswe ukunciphisa izimpawu, kufaka phakathi ubuhlungu. Iphinde ihlose ukuvimbela izinkinga, ikakhulukazi izifo.

Ukwelashwa kungafaka:

• Imithi elwa namagciwane nemithi yokulwa namagciwane yokulawula noma yokuvimbela ukutheleleka
• Uketshezi nama-electrolyte anikezwa ngomthambo (IV) uma kunezilonda zomlomo ezinzima
• Ukondliwa kwe-IV uma kunezilonda zomlomo ezinzima
• I-lozenges yomlomo we-numbing (anesthetic) ukunciphisa izinhlungu zezilonda zomlomo
• Imithi yezinhlungu uma ukukhululeka kobuhlungu bendawo kunganele

Ukwelashwa komzimba wonke (systemic) kuyadingeka ukulawula i-pemphigus futhi kufanele kuqalwe ngokushesha ngangokunokwenzeka. Ukwelashwa okuhlelekile kufaka phakathi:

• Umuthi olwa nokuvuvukala obizwa nge-dapsone
• Ama-Corticosteroids
• Imithi equkethe igolide
• Imithi ecindezela amasosha omzimba (njenge-azathioprine, methotrexate, cyclosporine, cyclophosphamide, mycophenolate mofetil, noma i-rituximab)

Ama-antibiotic angasetshenziswa ukwelapha noma ukuvimbela ukutheleleka. I-immunoglobulin efakwa ngaphakathi (IVIg) isetshenziswa ngezikhathi ezithile.

I-Plasmapheresis ingasetshenziswa kanye nemithi ye-systemic ukunciphisa inani lama-antibodies egazini. I-Plasmapheresis inqubo lapho i-plasma equkethe ama-antibody isuswa egazini bese ithathelwa indawo ngamanzi afakwa emithanjeni noma i-plasma enikelwe.

Ukwelashwa kwe-ulcer ne-blister kufaka phakathi ama-lotion athobisayo noma omile, ukugqoka okumanzi, noma izinyathelo ezifanayo.

Ngaphandle kokwelashwa, lesi simo singasongela impilo. Ukutheleleka okukhulu kuyimbangela yokufa ejwayelekile kakhulu.

Ngokwelashwa, lesi sifo sihlala singamahlalakhona. Imiphumela emibi yokwelashwa ingaba nzima noma ikhubaze.

Izinkinga ze-PV zifaka:

• Izifo zesikhumba zesibili
• Ukuphela kwamanzi emzimbeni
• Imiphumela emibi yemithi
• Ukusabalala kokutheleleka ngegazi (sepsis)

Umhlinzeki wakho wezokunakekelwa kwempilo kufanele ahlole noma yimaphi amabhamuza angachazeki.

Shayela umhlinzeki wakho uma welashelwe i-PV futhi uba nezimpawu ezilandelayo:

• Amakhaza
• Imfiva
• Ukuzizwa okuvamile
• Izinhlungu ezihlangene
• Ubuhlungu bemisipha
• Amabhamuza amasha noma izilonda

• I-Pemphigus vulgaris ngemuva
• I-Pemphigus vulgaris - izilonda emlonyeni

Amagai M. Pemphigus. Ku: Bolognia JL, Schaffer JV, Cerroni L, ama-eds. Isikhumba. Umhla wesi-4. IPhiladelphia, PA: Elsevier; 2018: isahluko 29.

I-Dinulos JGH. Izifo ezibangelwa i-veesicular and bullous. Ku: I-Dinulos JGH, ed. IHabif's Clinical Dermatology. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2021: isahluko 16.

UJames WD, u-Elston DM, Phatha i-JR, i-Rosenbach MA, i-IM ye-Neuhaus. Ama-dermatoses angapheli. Ku: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Izifo zika-Andrew Zesikhumba. Umhlaka 13. IPhiladelphia, PA: Elsevier; 2020: isahluko 21.

IPatterson JW. Iphethini yokuphendula ye-vesiculobullous. Ku: Patterson JW, ed. I-Weedon's Skin Pathology. Umhlaka 5. IPhiladelphia, PA: Elsevier; 2021: isahluko 7.