Yintoni i-epidermolysis bullosa, iimpawu kunye nonyango

Umxholo

• Iimpawu eziphambili
• Unobangela we-bullous epidermiolysis
• Zithini iindidi
• Unyango lwenziwa njani
• Xa kufuneka utyando
• Kufuneka wenze ntoni ukuthintela ukuvela kwamaqamza
• Indlela yokwenza ukunxiba
• Zithini iingxaki

I-Bullous epidermolysis sisifo semfuza esikhumbeni esibangela ukwenziwa kwamadyungudyungu kulusu kunye neembumba ezinomhlana, emva kwako nakuphi na ukungqubana okanye umothuko omncinci onokubangelwa kukucaphuka kwelebheli yempahla eluswini okanye, ngokulula, ngokususa Into yamabhandeji, umzekelo. Le meko yenzeka ngenxa yotshintsho lwemfuza oluhanjiswa lusuka kubazali luye ebantwaneni babo, olukhokelela kutshintsho kumaleko nakwizinto ezikhoyo kulusu, njengekeratin.

Iimpawu kunye neempawu zesi sifo zinxulunyaniswa nokubonakala kwamadyungudyungu abuhlungu eluswini nakweliphi na ilungu lomzimba, kwaye kunokubonakala nasemlonyeni, ezintendeni nasezinyaweni zeenyawo. Ezi mpawu ziyahluka ngohlobo kunye nobukrakra be-epidermolysis bullosa, kodwa zihlala zisiba mbi ekuhambeni kwexesha.

Unyango lwe-bullous epidermolysis luquka ikakhulu ukhathalelo oluxhasayo, njengokugcina ukutya okwaneleyo kunye nokunxiba iblisters. Ukongeza, kuqhutywa izifundo zokwenza ukufakelwa umongo wethambo kubantu abakule meko.

Iimpawu eziphambili

Iimpawu eziphambili ze-bullous epidermolysis zezi:

• Ukudumba kolusu kububunzima obuncinci;
• Amadyungudyungu avela ngaphakathi emlonyeni nasemehlweni;
• Ukuphilisa ulusu ngokubonakala okurhabaxa kunye namabala amhlophe;
• Ukulalanisa kwinzipho;
• Ukucoca iinwele;
• Ukunciphisa ukubila okanye ukubila okungaphezulu.

Kuxhomekeke kubuzaza besifo se-epidermolysis, ukubola kweminwe neenzwane kunokwenzeka, kukhokelela kwiziphene. Ngaphandle kwempawu ezinesifo se-epidermolysis, ezinye izifo zinokukhokelela ekubonakaleni kwamadyungudyungu kulusu, njenge-herpes simplex, i-epidermolytic ichthyosis, i-bullous impetigo kunye ne-pigmentary incontinence. Yazi ukuba yintoni i-bullous impetigo kwaye luyintoni unyango.

Unobangela we-bullous epidermiolysis

I-Bullous epidermolysis ibangelwa kukutshintshwa kwemfuzo ukusuka kumzali kuye emntwaneni, kwaye inokuba yinto ephambili, apho omnye umzali unesifo sesifo, okanye esixineneyo, apho utata nomama bathwala isifo kodwa kungekho bonakaliso lweempawu esi sifo.

Abantwana abanezihlobo ezisondeleyo abanesifo okanye i-bullous epidermolysis gene kusenokwenzeka ukuba bazalwe benolu hlobo lwesimo, ke ukuba abazali bayazi ukuba banesifo sesifo ngokuvavanywa kwemfuza, ukululekwa ngemfuzo kuyaboniswa. Jonga ukuba yintoni ingcebiso ngemfuzo kwaye yenziwa njani.

Zithini iindidi

I-Bullous epidermolysis inokwahlulahlulwa ibe ziindidi ezintathu kuxhomekeke kubume besikhumba obenza iblisters, ezinje:

• I-epidermolysis elula yenkomo: Ukudumba kwenzeka kumaleko aphezulu esikhumba, abizwa ngokuba yintsholongwane, kwaye kuqhelekile ukuba avele ezandleni nasezinyaweni. Kolu hlobo kunokwenzeka ukubona iinzipho zirhabaxa kwaye zingqindilili kwaye izilonda azipholi ngokukhawuleza;

• I-Dystrophic epidermolysis bullosa: Amadyungudyungu kolu hlobo avela ngenxa yeziphene kwimveliso yohlobo V | I-collagen kwaye yenzeka kwelona hlaza lolusu, olubizwa ngokuba yidermis;
• Ukudibana kwe-epidermolysis bullosa: esaziwa ngokusekwa kwamadyungudyungu ngenxa yomda owomileyo phakathi kolona luhlu luphambili noluphakathi kwesikhumba kwaye kule meko, esi sifo senzeka ngokutshintshwa kwemfuza ehambelana nedermis kunye ne-epidermis, njengeLaminin 332.

I-Kindler's syndrome ikwaluhlobo lwe-bullous epidermolysis, kodwa inqabile kwaye ibandakanya lonke ulusu lolusu, olukhokelela kubuchwephesha obugqithisileyo. Nokuba luhlobo luni lwesi sifo, kubalulekile ukuba uqaphele ukuba i-bullous epidermolysis ayosuleli, oko kukuthi, ayidluli isuka komnye umntu iye komnye ngokuhlangana nezilonda zolusu.

Unyango lwenziwa njani

Akukho lunyango oluthile lwe-epidermolysis bullosa, kwaye kubaluleke kakhulu ukubonisana rhoqo nedermatologist ukuvavanya imeko yolusu kunye nokuphepha iingxaki, ezinje ngezifo, umzekelo.

Unyango lwesi sifo lubandakanya amanyathelo axhasayo, njengokunxiba amanxeba kunye nokulawula iintlungu kwaye, kwezinye iimeko, ukulaliswa esibhedlele kuyimfuneko ukwenza ukunxiba okungenazintsholongwane, kungenazo iintsholongwane, ukuze amayeza afakwe ngqo emthanjeni, imeko yosulelo, kunye nokucoca i-blister kulusu. Nangona kunjalo, ezinye izifundo ziyaphuhliswa ukuze kwenziwe ukufakelwa kweeseli zesitofu kunyango lwe-dystrophic bulous epidermolysis.

Ngokungafaniyo namadyungudyungu abangelwa kukutsha, iblisters ezibangelwa yi-epidermolysis bullosa kufuneka zihlatyiwe ngenaliti ethile, kusetyenziswa uxinizelelo olungenantsholongwane, ukuthintela ukuba ingasasazeki kwaye ibangele ukonakala ngakumbi kulusu. Emva kokutsala, kubalulekile ukufaka imveliso, enje tshiza antibacterial, ukuthintela usulelo.

Xa kufuneka utyando

Utyando lwe-Bullous dermatitis luhlala lubonakalisiwe kwimeko apho amanxeba ashiywe ngamabhamu ethintela ukuhamba komzimba okanye abangela ukukhubazeka okunciphisa umgangatho wobomi. Ngamanye amaxesha, utyando lunokusetyenziselwa ukwenza izicatshulwa zolusu, ngakumbi kumanxeba athatha ixesha elide ukuphola.

Kufuneka wenze ntoni ukuthintela ukuvela kwamaqamza

Kuba akukho lunyango, unyango lwenziwa kuphela ukunciphisa iimpawu kunye nokunciphisa ithuba lamadyungudyungu amatsha. Inyathelo lokuqala kukukhathalelwa ekhaya, njenge:

• Nxiba impahla yomqhaphu, uphephe amalaphu okwenziwa;
• Susa iithegi kuzo zonke iimpahla;
• Nxiba iimpahla zangaphantsi eziguqulwe zangaphezulu ukujongana nokunwebeka kolusu;
• Nxiba izihlangu ezikhaphukhaphu nezibanzi ngokwaneleyo ukuba zinokunxiba iikawusi ezingenamthungo;
• Lumka kakhulu xa usebenzisa iitawuli emva kokuhlamba, ucofe ulusu ngobunono ngetawuli ethambileyo;
• Faka iVaseline ngobuninzi ngaphambi kokuba ususe ukunxiba kwaye unganyanzeli ukususwa kwayo;
• Ukuba iimpahla ekugqibeleni zinamathela eluswini, shiya ummandla ofakwe emanzini de iimpahla zikhululeke kulusu lodwa;
• Gubungela amanxeba ngokunxiba okungahambelaniyo kunye ne-gauze evulekileyo;
• Lala ngeekawusi kunye neiglavu ukunqanda ukwenzakala okunokwenzeka xa ulele.

Ukongeza, ukuba kukho ulusu olurhawuzayo, ugqirha unokuyalela ukusetyenziswa kwe-corticosteroids, enje nge-prednisone okanye i-hydrocortisone, ukunciphisa ukudumba kolusu kunye nokunciphisa iimpawu, ukunqanda ukukrwela ulusu, ukuvelisa izilonda ezintsha. Kuyimfuneko ukuba ulumke xa uhlamba, ukunqanda ukuba amanzi ashushu kakhulu.

Ukusetyenziswa kwe botox ezinyaweni kubonakala ngathi ziyasebenza ekuthinteleni i-blisters kulo mmandla, kwaye i-gastrostomy iboniswa xa kungenakwenzeka ukuba itye ngokufanelekileyo ngaphandle kokuvela kwamadyungudyungu emlonyeni okanye ngomphunga.

Indlela yokwenza ukunxiba

Ukunxiba kuyinxalenye yesiqhelo kwabo bane-bulm epidermolysis kwaye ezi zinxibo mazenziwe ngononophelo ukuze zikhuthaze ukuphilisa, zinciphise ukukhuhlana kwaye zithintele ukopha kulusu, kuba oku kubalulekile ukusebenzisa iimveliso ezinganyanzelekanga kulusu Oko kukuthi, akukho glu ibambelela ngamandla.

Ukunxiba amanxeba anokukhutshwa okuninzi, kubalulekile ukuba kusetyenziswe izinto ezenziwe ngogwebu lwe-polyurethane, njengoko zifunxa la manzi kwaye zikhusela nxamnye neentsholongwane.

Kwiimeko apho amanxeba sele omile, kuyacetyiswa ukuba kusetyenziswe ukunxiba kwehydrogel, kuba zinceda ekupheliseni izicwili zolusu ezifileyo kunye nokuphelisa iintlungu, ukurhawuzelela nokungahambi kakuhle kuloo ndawo. Ukunxitywa kufuneka kulungiswe nge-tubular okanye i-elastic meshes, ayikhuthazwa into yokusebenzisa isikhumba.

Zithini iingxaki

I-Bullous epidermolysis inokubangela ezinye iingxaki ezinje nosulelo, njengoko ukwenziwa kwamadyungudyungu kushiya ulusu luchaphazeleka lula kukungcola ziibhaktheriya kunye nefungi, umzekelo. Kwezinye iimeko ezimandundu ngakumbi, ezi bhakteriya zingena kulusu lomntu one-bulm epidermolysis inokufikelela kwigazi kwaye isasazeke kuwo wonke umzimba, ibangele i-sepsis.

Abantu abane-epidermolysis bullosa banokuthi bahlupheke ngenxa yokunqongophala kwesondlo, okuvela kwi-blisters emlonyeni okanye kwi-anemia, ebangelwa ukuphuma kwegazi kwizilonda. Ezinye iingxaki zamazinyo, ezinje ngee-caries, zinokubonakala, njengoko ulwelwesi lomlomo lubuthathaka kakhulu kubantu abanalesi sifo. Kwakhona, ezinye iintlobo ze-epidermolysis bullosa zonyusa umngcipheko wokuba umntu abe nomhlaza wolusu.