Yini i-epidermolysis bullosa, izimpawu nokwelashwa

-Delile

• Izimpawu eziyinhloko
• Isizathu se-bulous epidermiolysis
• Yiziphi izinhlobo
• Yelashwa kanjani
• Lapho kudingeka ukuhlinzwa
• Okufanele ukwenze ukuvimbela ukuvela kwamabhulebhu
• Ungenza kanjani ukugqoka
• Yiziphi izinkinga

I-Bullous epidermolysis yisifo sofuzo esikhunjeni esidala ukwakheka kwamabhamuza esikhunjeni nasezikhaleni zomlomo, ngemuva kwanoma yikuphi ukungqubuzana noma ukuhlukumezeka okuncane okungabangelwa ukucasuka kwelebula lezingubo esikhunjeni noma, ngokumane kususwe i-band aid, Ngokwesibonelo. Lesi simo senzeka ngenxa yezinguquko zofuzo ezidluliselwa zisuka kubazali ziye ezinganeni zabo, okuholela ekuguqulweni kwezendlalelo nezinto ezikhona esikhumbeni, njenge-keratin.

Izimpawu nezimpawu zalesi sifo zixhumene nokubonakala kwamabhaladi abuhlungu esikhunjeni nakunoma iyiphi ingxenye yomzimba, futhi kungavela nasemlonyeni, ezintendeni nasematheni ezinyawo. Lezi zimpawu ziyehluka ngokuya ngohlobo nobukhulu be-epidermolysis bullosa, kepha zivame ukuba zimbi ngokuhamba kwesikhathi.

Ukwelashwa kwe-bulous epidermolysis kuqukethe ikakhulukazi ukunakekelwa okusekela, njengokugcina ukondla okwanele nokugqoka amabhamuza esikhumba. Ngaphezu kwalokho, kwenziwa izifundo zokwenza ukufakelwa umnkantsha wethambo kubantu abakulesi simo.

Izimpawu eziyinhloko

Izimpawu eziyinhloko ze-bullous epidermolysis yilezi:

• Ukuqhuma kwesikhumba ngokungqubuzana okuncane;
• Amabhamuza avela ngaphakathi emlonyeni ngisho nasemehlweni;
• Ukuphulukiswa kwesikhumba ngokubukeka okunamandla namabala amhlophe;
• Ukuyekethisa kwesipikili;
• Izinwele zincipha;
• Ukwehliswa kwezithukuthuku noma ukujuluka okweqile.

Ngokuya ngobukhulu be-bullous epidermolysis, ukusikeka kweminwe nezinzwane nakho kungenzeka, okuholela ekukhubazekeni. Yize kunezibonakaliso zesici se-epidermolysis, ezinye izifo zingaholela ekubonakaleni kwamablisters esikhunjeni, njenge-herpes simplex, i-epidermolytic ichthyosis, i-bullous impetigo kanye ne-pigmentary incontinence. Yazi ukuthi yini i-impuligo enamandla futhi yiluphi ukwelashwa.

Isizathu se-bulous epidermiolysis

I-Bullous epidermolysis idalwa ukuguqulwa kwezakhi zofuzo okudluliselwa kusuka kumzali kuye enganeni, futhi kungahle kube namandla, lapho omunye umzali enofuzo lwesifo, noma eyeqile, lapho ubaba nomama bethwala ufuzo lwesifo kepha kungekho ukubonakaliswa kwezimpawu noma izimpawu lesi sifo.

Izingane ezinezihlobo eziseduze ezinesifo noma ezine-bullous epidermolysis gene kungenzeka kakhulu ukuthi zizalwe zinalolu hlobo lwesimo, ngakho-ke uma abazali bazi ukuthi banofuzo lwesifo ngokuhlolwa kofuzo, kukhonjiswa ukwelulekwa kwezofuzo. Bona ukuthi kuyini ukwelulekwa ngezakhi zofuzo nokuthi kwenziwa kanjani.

Yiziphi izinhlobo

I-Bullous epidermolysis ingahlukaniswa ngezinhlobo ezintathu ngokuya ngongqimba lwesikhumba olwenza amablisters, njenge:

• I-bulid epidermolysis elula: ukushwabana kwenzeka engxenyeni engenhla yesikhumba, ebizwa ngokuthi i-epidermis, futhi kujwayelekile ukuthi zivele ezandleni nasezinyaweni. Kulolu hlobo kungenzeka ukubona izinzipho ziqinile futhi ziwugqinsi kanti amabhamuza awapholi ngokushesha;

• I-Dystrophic epidermolysis bullosa: amabhamuza kulolu hlobo avela ngenxa yokukhubazeka ekukhiqizeni kohlobo V | I collagen futhi kwenzeka engxenyeni engaphezulu kakhulu yesikhumba, eyaziwa njenge-dermis;
• Ukuhlangana kwe-epidermolysis bullosa: esaziwa ngokwakhiwa kwamabhalidi ngenxa yokuhlukaniswa kwesifunda phakathi kongqimba olukha phezulu kakhulu noluphakathi kwesikhumba futhi kulokhu, lesi sifo sivela ngokuguquka kwezakhi zofuzo ezixhunywe ku-dermis kanye ne-epidermis, njengeLaminin 332.

I-Kindler's syndrome nayo iwuhlobo lwe-bulous epidermolysis, kepha ayivamile kakhulu futhi ihilela zonke izingqimba zesikhumba, okuholele ekuthambekeni okukhulu. Kungakhathalekile ukuthi hlobo luni lwalesi sifo, kubalulekile ukuqaphela ukuthi i-bullous epidermolysis ayithelelani, okungukuthi, ayidluli isuke komunye umuntu iye komunye ngokuxhumana nezilonda zesikhumba.

Yelashwa kanjani

Akukho ukwelashwa okuqondile kwe-epidermolysis bullosa, futhi kubaluleke kakhulu ukubonisana njalo nodokotela wesikhumba ukuhlola isimo sesikhumba nokugwema izinkinga, njengezifo, isibonelo.

Ukwelashwa kwalesi sifo kuqukethe izindlela ezisekelayo, njengokugqoka amanxeba nokulawula izinhlungu futhi, kwezinye izimo, ukulaliswa esibhedlela kuyadingeka ukuze kwenziwe ukugqoka okuyinyumba, kungabi nezilwanyana ezincane, ukuze imithi inikezwe ngqo emthanjeni, njengemithi elwa namagciwane icala lokutheleleka, nokukhipha amabhamuza esikhunjeni. Kodwa-ke, kwenziwa ezinye izifundo zokwenza ukufakelwa kwama-stem cell ekwelapheni i-dystrophic bulous epidermolysis.

Ngokungafani namabhamuza abangelwa ukusha, amabhamuza abangelwa i-epidermolysis bullosa kumele abhobozwe ngenaliti ethile, kusetshenziswe ukucindezela okuyinyumba, ukuyivimba ukuthi ingasabalali futhi idale umonakalo omkhulu esikhunjeni. Ngemuva kokukhipha, kubalulekile ukufaka umkhiqizo, njenge fafaza elwa namagciwane, ukuvimbela izifo.

Lapho kudingeka ukuhlinzwa

Ukuhlinzwa kwe-Bullous dermatitis kuvame ukukhonjiswa ecaleni lapho izibazi ezishiywe amagwebu zivimba ukunyakaza komzimba noma zibangele ukukhubazeka okwehlisa izinga lempilo. Kwezinye izimo, ukuhlinzwa kungasetshenziselwa nokwenza izingcaphuno zesikhumba, ikakhulukazi emanxebeni athatha isikhathi eside ukuphola.

Okufanele ukwenze ukuvimbela ukuvela kwamabhulebhu

Njengoba lingekho ikhambi, ukwelashwa kwenziwa kuphela ekunciphiseni izimpawu nasekunciphiseni amathuba amabhamuza amasha. Isinyathelo sokuqala ukunakekelwa ekhaya, njenge:

• Gqoka izingubo zikakotini, ugweme izindwangu zokwenziwa;
• Susa amathegi kuzo zonke izingubo;
• Gqoka izingubo zangaphansi ezibhekiswe phansi ukugwema ukuthintana nesikhumba;
• Gqoka izicathulo ezilula futhi ezibanzi ngokwanele ukugqoka kahle amasokisi angenamthungo;
• Qaphela kakhulu lapho usebenzisa amathawula ngemuva kokugeza, ucindezele kahle isikhumba ngethawula elithambile;
• Faka uVaseline ngobuningi ngaphambi kokukhipha okokugqoka futhi ungaphoqi ukususwa kwawo;
• Uma izingubo ekugcineni zinamathela esikhunjeni, shiya isifunda sifakwe emanzini kuze kube yilapho izingubo ziphuma esikhunjeni sodwa;
• Mboza amanxeba ngokugqoka okungekho okunamathelayo futhi nge-gauze evulekile evulekile;
• Lala ngamasokisi namagilavu ​​ukugwema ukulimala okungenzeka ngesikhathi sokulala.

Ngaphezu kwalokho, uma kunesikhumba esilumayo, udokotela anganquma ukusetshenziswa kwama-corticosteroids, njenge-prednisone noma i-hydrocortisone, ukukhulula ukuvuvukala kwesikhumba nokunciphisa izimpawu, ukugwema ukunwaya isikhumba, ukukhiqiza izilonda ezintsha. Kuyadingeka futhi ukuthi uqaphele lapho ugeza, ugweme ukuthi amanzi ashisa kakhulu.

Ukusetshenziswa kwe botox ezinyaweni kubonakala kusebenza ngempumelelo ekuvikeleni amabhamuza kulesi sifunda, kanti i-gastrostomy ikhonjiswa lapho kungenzeki ukuthi kudliwe kahle ngaphandle kokuvela kwamabhalabha emlonyeni noma emphinjeni.

Ungenza kanjani ukugqoka

Ukugqoka kuyingxenye yenkambiso yalabo abane-bullous epidermolysis futhi lokhu kugqokwa kufanele kwenziwe ngokucophelela ukuze kuthuthukise ukwelashwa, kunciphise ukungqubuzana futhi kuvimbele ukopha esikhunjeni, ngoba lokhu kubalulekile ukusebenzisa imikhiqizo enganamatheli esikhumbeni , okungukuthi, ezingenayo i-glue enamathela kakhulu.

Ukugqoka amanxeba anoketshezi oluningi, kubalulekile ukusebenzisa okokugqoka okwenziwe ngogwebu lwe-polyurethane, njengoba kumunca lezi ziketshezi futhi kunikeze ukuvikelwa kuma-microorganisms.

Ezimweni lapho izilonda sezivele zomile, kunconywa ukusebenzisa okokugqoka kwe-hydrogel, ngoba kusiza ukuqeda izicubu zesikhumba ezifile nokuqeda izinhlungu, ukulunywa nokungahambi kahle endaweni. Ukugqokiswa kufanele kulungiswe ngamamaki we-tubular noma e-elastic, akunconywa ukusebenzisa okunamathiselwe esikhunjeni.

Yiziphi izinkinga

I-Bullous epidermolysis ingadala ezinye izinkinga ezinjengokutheleleka, njengoba ukwakheka kwamabhamuza kushiya isikhumba sithinteke kakhulu ekungcolisweni ngamagciwane nesikhunta, ngokwesibonelo. Kwezinye izimo ezibucayi, lawa mabhaktheriya angena esikhunjeni somuntu one-bulous epidermolysis angafinyelela egazini futhi asakaze kuwo wonke umzimba, abangele i-sepsis.

Abantu abane-epidermolysis bullosa bangaphinde bahlupheke ngokushoda kokudla okunempilo, okuvela kumabhamuza emlonyeni noma ekungeneni kwegazi, okubangelwa ukopha ezilondeni. Ezinye izinkinga zamazinyo, ezinjenge-caries, zingavela, njengoba ulwelwesi lomlomo lubuthaka kakhulu kubantu abanalesi sifo. Futhi, ezinye izinhlobo ze-epidermolysis bullosa zandisa ubungozi bokuthi umuntu abe nomdlavuza wesikhumba.