Epidermolysis nzombe

Epidermolysis bullosa (EB) iboka rezvinetso umo ganda rinoputika mushure mekukuvara kuduku. Inopfuudzwa pasi mumhuri.

Kune mana makuru marudzi eEB. Vari:

• Dystrophic epidermolysis bullosa
• Epidermolysis bullosa simplex
• Hemidesmosomal epidermolysis bullosa
• Junctional epidermolysis nzombe

Imwe mhando isingawanzoitika yeEB inonzi epidermolysis bullosa acquisita. Iyi fomu inokura mushure mekuzvarwa. Iyo autoimmune kusagadzikana, zvinoreva kuti muviri unozvirwisa wega.

EB inogona kusiyanisa kubva kudiki kusvika kune inouraya. Iyo fomu diki inokonzeresa kuputika kweganda. Iyo inouraya fomu inokanganisa dzimwe nhengo. Mhando zhinji dzeiyi mamiriro dzinotangira pakuzvarwa kana nguva pfupi yapfuura. Zvinogona kuva zvakaoma kuziva iyo chaiyo mhando yeEB munhu anayo, kunyangwe chaiwo majini mamaki ave kuwanikwa mazhinji.

Nhoroondo yemhuri njodzi. Njodzi yacho yakakwira kana mubereki aine mamiriro aya.

Zvichienderana nechimiro cheEB, zviratidzo zvinogona kusanganisira:

• Alopecia (kurasikirwa bvudzi)
• Mapundu akakomberedza maziso nemhino
• Blister mukati kana kutenderedza muromo nehuro, zvichikonzera kudyisa matambudziko kana kumedza kuoma
• Blister paganda semhedzisiro yekukuvara kudiki kana shanduko tembiricha, kunyanya yemakumbo
• Kufuridzira izvo zviripo pakuzvarwa
• Matambudziko emazino akadai sekuora kwemeno
• Kuchema kuchema, kukosora, kana zvimwe zvinetso zvekufema
• Matinji machena machena paganda rakakuvara kare
• Chipikiri kurasikirwa kana zvipikiri zvakaremara

Anopa hutano hwako anotarisa paganda rako kuti aongorore EB.

Miedzo iyo inoshandiswa kusimbisa kuongororwa inosanganisira:

• Kuongorora genetic
• Ganda biopsy
• Special bvunzo dzeganda masampuli pasi pe microscope

Ganda bvunzo dzinogona kushandiswa kuzivisa fomu reEB.

Mimwe bvunzo inogona kuitwa inosanganisira:

• Ropa bvunzo yeanemia
• Tsika yekutarisa hutachiona hutachiona kana maronda ari kupora zvisirizvo
• Kumusoro endoscopy kana yepamusoro GI akateedzana kana zviratidzo zvinosanganisira kumedza matambudziko

Kukura kwehuwandu kunozoongororwa kazhinji kune mwana ane kana anogona kunge ane EB.

Chinangwa chekurapa ndechekudzivirira mablisters kubva mukuumba uye kudzivirira kunetseka. Kumwe kurapwa kunoenderana nekuti mamiriro acho akaipa sei.

KUCHENGETA KWEMUMBA

Tevedzera nhungamiro idzi kumba:

• Chengetedza zvakanaka ganda rako kudzivirira hutachiona.
• Tevedza kuraira kwemupi wako kana nzvimbo dzakatumbuka dzikatsemuka kana kuva mbishi. Iwe ungangoda nguva dzose whirlpool kurapwa uye kushandisa maantibiotic maronda kune maronda-senge nzvimbo. Wako mupi achakuzivisa iwe kana iwe uchida bandage kana kupfeka, uye kana zvirizvo, rudzii rwekushandisa.
• Ungangoda kushandisa emumuromo steroid mishonga kwenguva pfupi kana uine matambudziko ekumedza. Iwe unogona zvakare kutora mushonga kana iwe ukawana candida (mbiriso) hutachiona mumuromo kana pahuro.
• Tora hutano hwakanaka hwemuromo wako uye tora kugara uchiongororwa mazino. Zviri nani kuona chiremba wemazino ane ruzivo rwekurapa vanhu vane EB.
• Idya kudya kunovaka muviri. Paunenge uine zvakawanda zvekukuvara kweganda, ungangoda mamwe macalorie uye mapuroteni kubatsira ganda rako kupora. Sarudza zvekudya zvakapfava uye dzivirira nzungu, machipisi, uye zvimwe zvinokachidza kana uine maronda mumuromo mako. Nyanzvi yezvokudya inogona kukubatsira iwe nedhayeti yako.
• Ita maekisesaizi ekurapa kwepanyama anokuratidza kuti ubatsire kuchengeta majoini uye mhasuru nhare

Kuvhiya

Kuvhiya kwekurapa mamiriro aya kunogona kusanganisira:

• Ganda kubatanidza munzvimbo dzine maronda akadzika
• Kupararira (kuwedzerwa) kwesopu kana paine kudzikira
• Kugadziriswa kwemaoko akaremara
• Kubviswa kwechero squamous cell carcinoma (rudzi rwegomarara reganda) rinoitika

MIMWE MIRAPO

Mimwe mishonga yekurapa iyi inogona kusanganisira:

• Mishonga inodzvinyirira immune immune inogona kushandiswa kune iyo autoimmune fomu yeichi chiitiko.
• Mapuroteni uye kurapa kwemajini uye kushandiswa kweiyo drug interferon kuri kuongororwa.

Maonero anoenderana nekukura kwechirwere.

Kutapukirwa kwenzvimbo dzine blist kwakajairika.

Mafomu akapfava eEB anovandudza nezera. Maitiro akakomba kwazvo eEB ane huwandu hwakanyanya hwekufa.

Mune mafomu akaomarara, kupora mushure memablisters fomu kunogona kukonzera:

• Kontrakiti yekuremara (semuenzaniso, paminwe, magokora, nemabvi) uye zvimwe zvakaremara
• Kumedza matambudziko kana muromo negurokuro zvikanganiswa
• Yakabatanidzwa minwe nezvigunwe
• Kufamba kushoma kubva pakuvenga

Aya matambudziko anogona kuitika:

• Anemia
• Yakaderedzwa hupenyu hurefu hwemhando dzakakomba dzemamiriro acho
• Esophageal kudzikira
• Matambudziko eziso, kusanganisira bofu
• Utachiona, kusanganisira sepsis (hutachiona muropa kana zvinyama)
• Kurasikirwa kwebasa mumaoko nemakumbo
• Tsandanyama dystrophy
• Periodontal chirwere
• Kusadya kwakakwana kwakakonzerwa nekudya kunetseka, zvichitungamira mukukundikana kubudirira
• Kenza yeganda squamous sero

Kana mwana wako achinge achinge achinge achinge achinge achinge achangoberekwa, danai mupi wako. Kana iwe uine nhoroondo yemhuri yeEB uye uronga kuita vana, ungangoda kuve neijenasi kuraira.

Genetic counselling inokurudzirwa kune vangangove vabereki vane nhoroondo yemhuri yechero mhando yeepidermolysis bullosa.

Panguva yekuzvitakura, bvunzo inonzi chorionic villus sampling inogona kushandiswa kuyedza mwana. Kune vakaroorana vari panjodzi huru yekuve nemwana aneEB, bvunzo inogona kuitwa kutanga svondo 8 kusvika 10 yekuzvitakura. Taura nemupi wako.

Kuti udzivise kukuvara kweganda uye kuputika, pfeka padding pakakomberedza nzvimbo dzinokuvara senge magokora, mabvi, makumbo, uye magaro. Dzivisa kusangana mitambo.

Kana iwe uine EB acquisita uye uri pane steroid kwenguva yakareba kupfuura mwedzi mumwe, ungangoda calcium uye vhitamini D zvinowedzera. Izvi zvinowedzera zvinogona kubatsira kudzivirira osteoporosis (kuonda mapfupa).

EB; Junctional epidermolysis nzombe; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Weber-Cockayne chirwere; Epidermolysis bullosa simplex

• Epidermolysis bullosa, yakanyanya dystrophic
• Epidermolysis nzombe, dystrophic

Denyer J, Pillay E, Clapham J. Yakanakisa Maitiro Ekutungamira eGanda uye Kurapa Kuchengeta muEpidermolysis Bullosa: An International Consensus. London, UK: Maronda Emarudzi Akawanda; 2017.

Zvakanaka, JD, Mellerio JE. Mune: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2018: chits. 32.

Habif TP. Zvirwere uye zvinotyisa. Mune: Habif TP, rakanyorwa. Clinical Dermatology. 6th ed. Philadelphia, PA: Elsevier; 2016: chits.