I-Pemphigus

Uhlolojikelele lwe-pemphigus

I-Pemphigus yisifo esenza ukuba amabhamuza akhe esikhumbeni nangaphakathi emlonyeni, ekhaleni, emphinjeni, emehlweni nasezithweni zangasese. Lesi sifo asivamile e-United States.

I-Pemphigus yisifo esizimele lapho amasosha omzimba ehlasela ngephutha amangqamuzana angaphezulu kwesikhumba (epidermis) kanye nolwelwesi lwamafinyila. Abantu abanalesi simo bakhiqiza amasosha omzimba ngokumelene nama-desmoglein, amaprotheni ahlanganisa amangqamuzana esikhumba komunye nomunye. Lapho lezi zibopho ziphukile, isikhumba siba brittle futhi uketshezi lunganqwabelana phakathi kwezingqimba zalo, kwakheka amabhamuza.

Kunezinhlobo eziningana ze-pemphigus, kodwa ezimbili eziyinhloko yilezi:

• I-Pemphigus vulgaris, ngokuvamile ethinta isikhumba nolwelwesi lwamafinyila, njengangaphakathi emlonyeni.
• I-Pemphigus foliaceus, ethinta isikhumba kuphela.

Alikho ikhambi le-pemphigus, kodwa ezimweni eziningi lingalawulwa ngemithi.

Ubani othola i-pemphigus?

Kungenzeka ukuthi uthole i-pemphigus uma unezici ezithile zobungozi. Lokhu kuhlanganisa:

• Isizinda sobuhlanga. Nakuba i-pemphigus yenzeka phakathi kwamaqembu ezinhlanga nezinhlanga, abantu abathile basengozini enkulu yezinhlobo ezithile zesifo. Abantu bamaJuda (ikakhulukazi i-Ashkenazi), amaNdiya, aseNingizimu-mpumalanga yeYurophu, noma okhokho baseMpumalanga Ephakathi basengozini yokuthola i-pemphigus vulgaris.
• Isimo sendawo. I-Pemphigus vulgaris iwuhlobo oluvame kakhulu emhlabeni wonke, kodwa i-pemphigus foliaceus ivame kakhulu kwezinye izindawo, njengezindawo zasemaphandleni eBrazil naseTunisia.
• Ubulili neminyaka. Abesifazane bathola i-pemphigus vulgaris kaningi kunamadoda, futhi iminyaka yobudala ngokuvamile iphakathi kweminyaka engama-50 nengama-60 ubudala. I-Pemphigus foliaceus ngokuvamile ihlasela amadoda nabesifazane ngokulinganayo, kodwa kwezinye imiphakathi, abesifazane bathinteka kakhulu kunabesilisa. Nakuba ubudala bokuqala kwe-pemphigus foliaceus ngokuvamile buphakathi kweminyaka engu-40 no-60 ubudala, kwezinye izindawo, izimpawu zingase zivele phakathi nengane.
• Ufuzo. Ososayensi bakholelwa ukuthi ukwanda kwalesi sifo emiphakathini ethile kungenxa yezakhi zofuzo. Isibonelo, idatha ibonisa ukuthi okuhlukile okuthile emndenini wezakhi zofuzo zamasosha omzimba okuthiwa i-HLA zihlotshaniswa nengcuphe ephezulu ye-pemphigus vulgaris ne-pemphigus foliaceus.
• Imithi. Akuvamile, i-pemphigus yenzeka ngenxa yokuthatha imithi ethile, njengama-antibiotic athile kanye nemithi yomfutho wegazi. Imithi equkethe iqembu lamakhemikhali elibizwa ngokuthi i-thiol nayo iye yaxhunyaniswa ne-pemphigus.
• Umdlavuza. Ezimweni ezingavamile, ukuthuthukiswa kwesimila, ikakhulukazi ukukhula kwe-lymph node, i-tonsil noma i-thymus gland, kungavusa lesi sifo.

Izinhlobo ze-pemphigus

Kunezinhlobo ezimbili eziyinhloko ze-pemphigus futhi zihlukaniswa ngokongqimba lwesikhumba lapho amabhamuza akheka khona nalapho amabhamuza atholakala khona emzimbeni. Uhlobo lwamasosha omzimba ahlasela amangqamuzana esikhumba lusiza nokunquma uhlobo lwe-pemphigus.

Izinhlobo ezimbili eziyinhloko ze-pemphigus yilezi:

• I-Pemphigus vulgaris uhlobo oluvame kakhulu e-United States. Amabhamuza akheka emlonyeni nakweminye i-mucosal surface, kanye nasesikhumbeni. Zikhula ezingxenyeni ezijulile ze-epidermis futhi ngokuvamile zibuhlungu. Kunohlobo oluncane lwalesi sifo olubizwa nge-pemphigus autonomicus, lapho amabhamuza akheka ngokuyinhloko embeleni nangaphansi kwamakhwapha.
• I-Leaf pemphigus okungajwayelekile futhi kuthinta isikhumba kuphela. Amabhamuza akheka ezingxenyeni ezingenhla ze-epidermis futhi angase alume noma abe buhlungu.

Ezinye izinhlobo ezingavamile ze-pemphigus zihlanganisa:

• I-Paraneoplastic pemphigus. Lolu hlobo lubonakala ngezilonda zomlomo nezindebe, kodwa ngokuvamile luphinde lube namabhamuza noma izilonda ezivuvukalayo esikhumbeni nakwezinye izinhlaka zamafinyila. Ngalolu hlobo, izinkinga ezinzima zamaphaphu zingenzeka. Abantu abanalolu hlobo lwesifo bavame ukuba nesimila, futhi lesi sifo singathuthuka uma isimila sisuswa ngokuhlinzwa.
• I-pemphigus. Leli fomu libangelwa uhlobo lwe-antibody olubizwa nge-IgA. Amabhamuza noma amaqhubu ngokuvamile avela ngamaqembu noma amasongo esikhumbeni.
• i-pemphigus yokwelapha. Eminye imithi, njengamanye ama-antibiotics kanye nemithi yomfutho wegazi, nezidakamizwa eziqukethe iqembu lamakhemikhali okuthiwa i-thiol, zingabangela amabhamuza noma izilonda ezinjenge-pemphigus. Amabhamuza nezilonda kuvame ukunyamalala uma uyeka ukuphuza umuthi.

I-Pemphigoid yisifo esihlukile kune-pemphigus kodwa esinezici ezithile ezivamile. I-Pemphigoid ibangela ukuhlukana lapho kuhlangana khona i-epidermis kanye ne-dermis engaphansi, okuholela kumabhamuza aqinile ajulile anganqamuki kalula.

Izimpawu ze-pemphigus

Uphawu oluyinhloko lwe-pemphigus ukuqhuma kwesikhumba futhi, kwezinye izimo, ulwelwesi lwamafinyila njengomlomo, ikhala, umphimbo, amehlo, nezitho zangasese. Amabhamuza ayaphuka futhi avame ukuqhuma, abangele izilonda ezinzima. Amabhamuza esikhumbeni angase ahlangane, akhe amabala abukhali avame ukungenwa izifo futhi akhiphe uketshezi oluningi. Izimpawu ziyahlukahluka kuye ngohlobo lwe-pemphigus.

• I-Pemphigus vulgaris amabhamuza ngokuvamile aqala emlonyeni, kodwa angase avele kamuva esikhumbeni. Isikhumba singaba brittle kangangokuthi siyaxebuka lapho sihlikihlwa ngomunwe. Ulwelwesi lwamafinyila njengekhala, umphimbo, amehlo, nezitho zangasese nazo zingase zithinteke.

  Amabhamuza akha ungqimba olujulile lwe-epidermis futhi ngokuvamile abuhlungu.

• I-Leaf pemphigus kuthinta isikhumba kuphela. Amabhamuza avame ukuvela ebusweni, ekhanda, esifubeni, noma emhlane ongenhla, kodwa angasakazekela kwezinye izindawo zomzimba ngokuhamba kwesikhathi. Izindawo ezithintekile zesikhumba zingavuvuka futhi zibe namaqebelengwane noma izikali. Amabhamuza akheka ezingxenyeni ezingaphezulu ze-epidermis futhi angase alume noma abe buhlungu.

Izinkinga ze-pemphigus

I-Pemphigus yisifo se-autoimmune esenzeka lapho amasosha omzimba ehlasela isikhumba esinempilo. Amangqamuzana amasosha omzimba abizwa ngokuthi amasosha omzimba aqondisa amaprotheni abizwa ngokuthi ama-desmogleins, asiza ukuhlanganisa amangqamuzana esikhumba angomakhelwane elinye nelinye. Lapho lezi zibopho ziphulwa, isikhumba siba brittle futhi uketshezi lungahlangana phakathi kwezingqimba zamangqamuzana, kwakhe amabhamuza.

Ngokuvamile, amasosha omzimba avikela umzimba ezifweni nasezifweni. Abacwaningi abazi ukuthi yini ebangela ukuthi amasosha omzimba avule amaprotheni omzimba, kodwa bakholelwa ukuthi kokubili izici zofuzo nezemvelo zihilelekile. Okuthile endaweni ezungezile kungabangela i-pemphigus kubantu abasengozini ngenxa yezakhi zabo zofuzo. Akuvamile, i-pemphigus ingabangelwa isimila noma imithi ethile.