Epidermolysis bullosa, matšoao le kalafo ke eng

Litaba

• Matšoao a mantlha
• Lebaka la bullous epidermiolysis
• Mefuta ke efe
• Kalafo e etsoa joang
• Ha ho hlokahala opereishene
• Seo u lokelang ho se etsa ho thibela ponahalo ea bubble
• Mokhoa oa ho etsa moaparo
• Mathata ke afe

Bullous epidermolysis ke lefu la liphatsa tsa lefutso la letlalo le bakang ho thehoa ha machachetsi letlalong le kahara lera la ka morao, kamora khohlano kapa ts'itiso e nyane e ka bakoang ke ho teneha ha lebanta la liaparo letlalong kapa, ka ho tlosa tlamiso, ka mohlala. Boemo bona bo etsahala ka lebaka la liphetoho tsa liphatsa tsa lefutso tse fetisetsoang ho tloha ho batsoali ho ea ho bana ba bona, tse lebisang liphetohong tsa likarolo le lintho tse teng letlalong, joalo ka keratin.

Matšoao a lefu lena a hokahane le ponahalo ea machachetsi a bohloko letlalong le karolong efe kapa efe ea 'mele, mme e kanna ea hlaha molomong, liatleng le bohatong ba maoto. Matšoao ana a fapana ho latela mofuta le matla a epidermolysis bullosa, empa hangata a mpefala ha nako e ntse e tsamaea.

Kalafo ea bullous epidermolysis e na le tlhokomelo e ts'ehetsang, joalo ka ho boloka phepo e nepahetseng le ho apesa machachetsi a letlalo. Ntle le moo, ho ntse ho etsoa lithuto ho etsa phepelo ea moko oa masapo bakeng sa batho ba nang le boemo bona.

Matšoao a mantlha

Matšoao a mantlha a lefu la epidermolysis ke:

• Ho phatloha ha letlalo ka khohlano e fokolang;
• Malengelana a hlaha ka hara molomo esita le mahlong;
• Pholiso ea letlalo le nang le ponahalo e mpe le matheba a masoeu;
• Ho sekisetsa ha lipekere;
• Ho fokotsa moriri;
• Phokotso ea mofufutso kapa mofufutso o feteletseng.

Ho ipapisitse le boima ba "epidermolysis" e bohale, khaello ea menoana le menoana ea maoto le eona e ka hlaha, e lebisang bofokoli. Leha e le matšoao a ikhethang a epidermolysis, maloetse a mang a ka lebisa ho ponahalo ea machachetsi letlalong, joalo ka herpes simplex, epidermolytic ichthyosis, bullous impetigo le pigmentary incontinence. Tseba hore na impuligo e matla ke eng le kalafo ke eng.

Lebaka la bullous epidermiolysis

Bullous epidermolysis e bakoa ke liphetoho tsa lefutso tse fetisoang ho tloha ho motsoali ho ea ho ngoana, mme e kanna ea ba matla, moo motsoali a le mong a nang le lefutso la lefu, kapa a fetelletseng, moo ntate le mme ba jereng lefutso la lefutso empa ho se na ponahatso ea matšoao a lefu lena.

Bana ba nang le beng ka bona ba haufi ba nang le lefu lena kapa ba nang le lefutso la bullous epidermolysis ba na le monyetla oa ho tsoaloa ba e-na le mofuta ona, kahoo haeba batsoali ba tseba hore ba na le lefutso la lefu ka ho etsa liteko tsa lefutso, ho fanoa ka tlhabollo ea lefutso. Bona hore na tlhabollo ea lefutso ke eng le hore e etsoa joang.

Mefuta ke efe

Bullous epidermolysis e ka aroloa ka mefuta e meraro ho latela bokaholimo ba letlalo bo etsang machachetsi, joalo ka:

• Boloetse bo bonolo ba epidermolysis: makhopho a hlaha karolong e kaholimo ea letlalo, e bitsoang epidermis, mme ho tloaelehile hore li hlahe matsohong le maotong. Mofuteng ona ho ka khoneha ho bona manala a le makukuno a bile a le matenya 'me machachetsi a sa fole kapele;

• Dystrophic epidermolysis bullosa: machachetsi a mofuta ona a hlaha ka lebaka la liphoso tlhahiso ea mofuta oa V | I collagen mme e hlaha karolong e ka ntle ea letlalo, e tsejoang e le dermis;
• Kopano ea epidermolysis bullosa: e tšoauoa ka ho theoa ha machachetsi ka lebaka la ho arohana ha sebaka lipakeng tsa karolo e kaholimo-limo le e mahareng ea letlalo mme ketsahalong ena lefu lena le hlaha ka liphetoho liphatseng tsa lefutso tse hokahantsoeng le dermis le epidermis, joalo ka Laminin 332.

Kindler's syndrome le eona ke mofuta oa bullous epidermolysis, empa ha e fumanehe hangata ebile e kenyelletsa likarolo tsohle tsa letlalo, e lebisang ho senyeha ho feteletseng. Ho sa tsotelehe mofuta oa lefu lena, ho bohlokoa ho hlokomela hore bullous epidermolysis ha e tšoaetsanoe, ke hore, ha e fetele ho motho e mong ka ho kopana le liso tsa letlalo.

Kalafo e etsoa joang

Ha ho na kalafo e ikhethang bakeng sa epidermolysis bullosa, mme ho bohlokoa haholo ho ba le lipuisano khafetsa le dermatologist ho lekola boemo ba letlalo le ho qoba mathata, joalo ka mafu.

Kalafo ea lefu lena e na le mehato e ts'ehetsang, joalo ka ho tlamisa maqeba le ho laola bohloko 'me, maemong a mang, ho hlokahala hore motho a kene sepetlele ho etsa masira a sa hloekang, a se nang likokoana-hloko, e le hore meriana e keneloe ka kotlolloho nyeoe ea tšoaetso, le ho ntša machachetsi letlalong. Leha ho le joalo, lithuto tse ling li ntse li ntlafatsoa ho etsa li-transplant tsa lisele tsa kalafo kalafong ea dystrophic bullous epidermolysis.

Ho fapana le machachetsi a bakoang ke ho cha, machachetsi a bakoang ke epidermolysis bullosa a tlameha ho phunngoa ka nale e itseng, ho sebelisoa litomoso tse nyopa ho e thibela ho hasana le ho baka tšenyo e kholo letlalong. Kamora ho ts'ela, ho bohlokoa ho etsa sehlahisoa, joalo ka fafatsa antibacterial, ho thibela tšoaetso.

Ha ho hlokahala opereishene

Ho buuoa ka boloetse bo bakoang ke dermatitis hangata ho bonts'oa ketsahalong eo maqeba a siiloeng ke makhopho a sitisang motsamao oa 'mele kapa a baka bofokoli bo fokotsang boleng ba bophelo. Maemong a mang, ho buuoa ho ka sebelisoa ho etsa likarolo tsa letlalo, haholo maqeba a nkang nako e telele ho fola.

Seo u lokelang ho se etsa ho thibela ponahalo ea bubble

Kaha ha ho na pheko, kalafo e etsoa feela ho fokotsa matšoao le ho fokotsa menyetla ea machachetsi a macha. Mohato oa pele ke ho ba le tlhokomelo lapeng, joalo ka:

• Apara liaparo tsa k'hothone, u qobe masela a maiketsetso;
• Tlosa li-tag liphahlo tsohle;
• Apara liaparo tsa kahare li shebile fatše ho qoba ho kopana le letlalo;
• Apara lieta tse bobebe le tse pharaletseng ka ho lekaneng ho apara likausi tse se nang moroko;
• E-ba hlokolosi haholo ha u sebelisa lithaole ha u qeta ho hlapa, u penya letlalo ka bonolo ka thaole e bonolo;
• Sebelisa Vaseline ka bongata pele u tlosa liaparo tsa 'mele' me u se ke ua qobella hore e tlosoe;
• Haeba liphahlo li qetella li khomaretse letlalong, tloha sebakeng seo se kolobisitsoe ke metsi ho fihlela liaparo li lokoloha letlalong le le leng;
• Koahela maqeba ka moaparo o sa khomarelang le ka lesela le tlotsitsoeng le hlephileng;
• Robala ka likausi le liatlana ho qoba likotsi tse ka hlahang nakong ea boroko.

Ntle le moo, haeba ho na le letlalo le hlohlona, ​​ngaka e ka laela tšebeliso ea corticosteroids, joalo ka prednisone kapa hydrocortisone, ho kokobetsa ho ruruha ha letlalo le ho fokotsa matšoao, ho qoba ho ngoapa letlalo, ho hlahisa liso tse ncha. Ho hlokahala hape hore u hlokomele ha u hlapa, u qobe hore metsi a chesa haholo.

Ts'ebeliso ea botox maotong ho bonahala ho sebetsa hantle ho thibeleng machachetsi sebakeng sena, 'me gastrostomy e bontšoa ha ho sa khonehe ho ja hantle ntle le ponahalo ea machachetsi molomong kapa momeng.

Mokhoa oa ho etsa moaparo

Ho apara ke karolo ea tloaelo ea ba nang le lefu la 'mele le nang le ts'oaetso' me liaparo tsena li tlameha ho etsoa ka hloko e le hore li ka matlafatsa pholiso, li fokotsa khohlano le ho thibela ho tsoa mali letlalong, hobane ho bohlokoa ho sebelisa lihlahisoa tse sa khomaretseng letlalong. , ke hore, ha ba na sekhomaretsi se khomarelang ka matla haholo.

Ho roala maqeba a nang le liphiri tse ngata, ho bohlokoa ho sebelisa liaparo tse entsoeng ka lekoeba la polyurethane, kaha li monya maro ana le ho fana ka tšireletso khahlanong le likokoana-hloko.

Maemong ao maqeba a seng a omme, ho kgothaletswa ho sebelisa liaparo tsa hydrogel, kaha li thusa ho felisa lisele tsa letlalo tse shoeleng le ho kokobetsa bohloko, ho hlohlona le ho se utloise bohloko sebakeng seo. Liaparo li tlameha ho ts'oaroa ka maqhubu a maqhubu kapa a rekere, ha ho kgothaletswe ho sebelisa likhomaretsi letlalong.

Mathata ke afe

Bullous epidermolysis e ka baka mathata a mang a ts'oaetso, joalo ka ha sebopeho sa machachetsi se siea letlalo le le kotsing ea ho silafatsoa ke libaktheria le li-fungus, mohlala. Maemong a mang a tebileng le ho feta, libaktheria tsena tse kenang letlalong la motho ea nang le bullous epidermolysis li ka fihla maling 'me tsa namela' meleng kaofela, tsa baka sepsis.

Batho ba nang le epidermolysis bullosa le bona ba ka hlaseloa ke khaello ea phepo e nepahetseng, e hlahang makhopho a tsoang molomong kapa phokolo ea mali, e bakoang ke ho tsoa mali liso. Mathata a mang a meno, joalo ka ho loka, a ka hlaha, kaha lera la molomo le fokola haholo ho batho ba nang le lefu lena. Hape, mefuta e meng ea epidermolysis bullosa e eketsa menyetla ea hore motho a tšoaroe ke mofetše oa letlalo.