Pemphigoïde bulleuse

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I-Bullous pemphigoid yisifo sesikhumba (i-dermatosis).

Lesi sakamuva sibonakala ngokuthuthukiswa kwamabhamuza amakhulu kuma-erythematous plaque (ama-plaque abomvu esikhumbeni). Ukubonakala kwala magwebu kuholela ezilonda futhi ngokuvamile kuyimbangela yokulunywa. (1)

Kuyisifo se-autoimmune, umphumela wokuphazamiseka kwamasosha omzimba kumuntu othintekile. Lo mthethonqubo wamasosha omzimba uqukethe ukukhiqizwa kwamasosha omzimba athile ngokumelene nomzimba wawo.

Le pathology ayivamile kodwa ingaba yingozi. Kudinga ukwelashwa isikhathi eside. (1)

Nakuba kuyisifo esiyivelakancane, siphinde sibe yi-autoimmune bullous dermatoses. (2)

Ukuvama kwayo kungu-1/40 (inani lamacala kumuntu ngamunye) futhi kuthinta kakhulu abantu asebekhulile (ngokwesilinganiso cishe eminyakeni engu-000 ubudala, ngengozi eyanda kancane kwabesifazane).

Ifomu losana nalo likhona futhi lithinta ingane phakathi nonyaka wokuqala wokuphila kwayo. (3)

Izimpawu

I-Bullous pemphigoid i-dermatosis yemvelaphi ye-autoimmune. Ngakho-ke umuntu ophethwe yilesi sifo ukhiqiza amasosha omzimba alwa nomzimba wakhe (autoantibodies). Lezi zihlasela izinhlobo ezimbili zamaprotheni: I-AgPB230 ne-AgPB180 etholakala phakathi kwezingqimba ezimbili zokuqala zesikhumba (phakathi kwedermis ne-epidermis). Ngokudala ukuhlukana phakathi kwalezi zingxenye ezimbili zesikhumba, lawa ma-auto-antibodies aholela ekwakhekeni kwamagwebu uphawu lwesifo. (1)

Izimpawu ze-atypical ze-bullous pemphigoid ukubukeka kwamabhamuza amakhulu (phakathi kuka-3 no-4 mm) nombala okhanyayo. Lawa mabhamuza avela ikakhulukazi lapho isikhumba sibomvu (i-erythematous), kodwa futhi angavela esikhumbeni esinempilo.

Izilonda ze-epidermal zivame ukutholakala endaweni yesiqu kanye nemilenze. Ubuso buvame ukugcinwa. (1)

Ukulunywa kwesikhumba (ukuluma), ngezinye izikhathi kusenesikhathi lapho kuvela amagwebu, nakho kubalulekile kulesi sifo.


Izinhlobo eziningana zesifo zibonisiwe: (1)

- ifomu elijwayelekile, izimpawu zalo ukubukeka kwamabhamuza amakhulu amhlophe nokuluma. Leli fomu livame kakhulu.

- ifomu le-vesicular, elichazwa ukubonakala kwamabhamuza amancane kakhulu ezandleni ngokuluma okukhulu. Leli fomu nokho alivamile kangako.

-ifomu le-urticaria: njengoba igama layo libonisa, kubangela ama-hives namabala abangela ukulunywa okukhulu.

- i-prurigo-like form, ukulunywa kwayo kusabalele kakhulu kepha kushubile. Lolu hlobo lwesifo lungabangela nokuqwasha esihlokweni esithintekile. Ngaphezu kwalokho, akuwona amabhamuza abonakala ngendlela yohlobo lwe-prurigo kodwa ama-crusts.


Ezinye iziguli zingase zingabi nazo izimpawu. Abanye baba bomvu, ukulunywa, noma ukucasuka. Ekugcineni, izimo ezivame kakhulu ziba bomvu kanye nokulunywa okukhulu.

Amabhamuza angaqhuma enze izilonda noma avuleke izilonda. (4)

Imvelaphi yalesi sifo

I-Bullous pemphigoid i-autoimmune dermatosis.

Le mvelaphi yalesi sifo iphumela ekukhiqizweni kwamasosha omzimba (amaprotheni amasosha omzimba) ngumzimba ngokumelene namaseli awo. Lokhu kukhiqizwa kwama-autoantibodies kuholela ekubhujisweni kwezicubu kanye / noma izitho kanye nokusabela kokuvuvukala.

Incazelo yangempela yalesi simo ayaziwa okwamanje. Noma kunjalo, izici ezithile zingaba nesixhumanisi esiqondile noma esingaqondile nokuthuthukiswa kwama-autoantibodies. Lezi yizici zemvelo, i-hormonal, imithi noma izakhi zofuzo. (1)

Lawa ma-autoantibodies akhiqizwa isihloko esithintekile aqondiswe kumaprotheni amabili: i-BPAG1 (noma i-AgPB230) ne-BPAG2 (noma i-AgPB180). Lawa maprotheni anendima yesakhiwo ekuhlanganeni phakathi kwedermis (ungqimba olungezansi) kanye ne-epidermis (ungqimba olungaphezulu). Lawa ma-macromolecules ahlaselwa ama-autoantibodies, isikhumba siyaxebuka bese kubangela ukuba kuvele amagwebu. (2)


Ngaphezu kwalokho, akukho ukutheleleka okufanele kuhlotshaniswe nalesi sifo. (1)

Ngaphezu kwalokho, izimpawu ngokuvamile zivela ngokuzenzakalelayo futhi kungalindelekile.

I-Bullous pemphigoid ayikho, nokho: (3)

- ukutheleleka;

- i-allergies;

- isimo esihlobene nendlela yokuphila noma ukudla.

Izici zengozi

I-Bullous pemphigoid yisifo esizimele, ngaleyo ndlela akusona isifo esitholakala ngofuzo.

Noma kunjalo, ukuba khona kwezakhi zofuzo ezithile kungaba yingozi yokuthola lesi sifo kubantu abanalezi zakhi zofuzo. Kuphakathi kokuthi kukhona ukuthambekela okuthile kofuzo.

Le ngozi ye-predisposition, nokho, iphansi kakhulu. (1)

Njengoba isilinganiso seminyaka yokuthuthukiswa kwesifo siseduze kwe-70, ubudala bomuntu bungase bube yingozi eyengeziwe yokuthuthukisa i-bullous pemphigoid.

Ngaphezu kwalokho, akumele sikushaye indiva iqiniso lokuthi le pathology ibuye ichazwe ngefomu losana. (3)

Ngaphezu kwalokho, ukutholakala okuncane kwalesi sifo kubonakala kwabesifazane. Ngakho-ke ubulili besifazane benza kube yisici esihlobene nengozi. (3)

Ukuvimbela nokwelashwa

Ukuxilongwa okuhlukile kwalesi sifo kubonakala ngokuyinhloko: ukubonakala kwama-bubbles acacile esikhumbeni.

Lokhu kuxilongwa kungaqinisekiswa yi-biopsy yesikhumba (ukuthatha isampula esikhumbeni esilimele ukuze sihlaziywe).

Ukusetshenziswa kwe-immunofluorescence kungasetshenziswa ekuboniseni amasosha omzimba ngemva kokuhlolwa kwegazi. (3)

Imithi yokwelapha enqunyiwe esimweni sokuba khona kwe-bullous pemphigoid ihlose ukunciphisa ukukhula kwamabhamuza kanye nokuphulukisa amabhamuza asevele ekhona esikhumbeni. (3)

Ukwelashwa okuvame kakhulu okuhambisana nalesi sifo i-systemic corticosteroid therapy.

Kodwa-ke, ezinhlotsheni zendawo ze-bullous pemphigoid, i-topical corticosteroid therapy (esebenza kuphela lapho umuthi usetshenziswa khona), kuhlanganiswe ne-dermatocorticoids yekilasi I (isidakamizwa esisetshenziswa ekwelashweni kwesikhumba kwendawo). (2)

Umuthi kadokotela wama-antibiotics womndeni we-tetracycline (ngezinye izikhathi ohlotshaniswa nokudla kukavithamini B) ungase usebenze ngodokotela.

Ukwelashwa kuvame ukunikezwa isikhathi eside futhi kusebenza ngempumelelo. Ngaphezu kwalokho, ukuphindeka kwalesi sifo ngezinye izikhathi kubonakala ngemva kokuyeka ukwelashwa. (4)

Ngemuva kokuxilongwa kokuba khona kwe-bullous pemphigoid, ukubonisana nodokotela wesikhumba kunconywa kakhulu. (3)

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