Epidermolysis Bullosa

Chidule cha Epidermolysis Bullosa

Epidermolysis bullosa ndi gulu la mikhalidwe yosowa yomwe khungu limakhala lolimba komanso lophulika mosavuta. Misozi, zilonda, ndi matuza pakhungu zimachitika pamene chinachake chipukuta kapena kugunda pakhungu. Amatha kuwonekera paliponse pathupi. Zikavuta kwambiri, matuza amathanso kupanga mkati mwa thupi, monga mkamwa, mmero, m'mimba, m'matumbo, m'matumbo, m'chikhodzodzo, ndi kumaliseche.

Anthu ambiri omwe ali ndi epidermolysis bullosa amatenga jini yosinthika (yosinthidwa) kuchokera kwa makolo awo. Kusintha kwa jini kumasintha momwe thupi limapangira mapuloteni omwe amathandiza kuti khungu likhale logwirizana komanso kukhala lolimba. Ngati muli ndi epidermolysis bullosa, imodzi mwa mapuloteniwa sanapangidwe bwino. Zigawo za khungu sizimalumikizana, zomwe zimapangitsa khungu kung'ambika ndi matuza mosavuta.

Chizindikiro chachikulu cha epidermolysis bullosa ndi khungu losalimba lomwe limatsogolera ku matuza ndi kung'ambika. Zizindikiro za matendawa nthawi zambiri zimayamba pakubadwa kapena ali wakhanda ndipo zimayambira pang'onopang'ono mpaka zovuta kwambiri.

Palibe mankhwala a matendawa; komabe, asayansi akupitiriza kufufuza njira zothandizira epidermolysis bullosa. Dokotala wanu amachiza zizindikiro, zomwe zingaphatikizepo kuchepetsa ululu, kuchiza mabala obwera chifukwa cha matuza ndi misozi, ndi kukuthandizani kulimbana ndi matenda.

Ndani amalandira epidermolysis bullosa?

Aliyense akhoza kutenga epidermolysis bullosa. Zimachitika m’mitundu ndi mafuko onse ndipo zimakhudza amuna ndi akazi mofanana.

Mitundu ya epidermolysis bullosa

Pali mitundu inayi yayikulu ya epidermolysis bullosa. Khungu limakhala ndi pamwamba kapena kunja komwe kumatchedwa epidermis ndi dermis layer yomwe ili pansi pa epidermis. Pansi pa khungu ndi pamene zigawo za khungu zimakumana. Madokotala kudziwa mtundu wa epidermolysis bullosa potengera malo kusintha khungu ndi kuzindikiridwa jini masinthidwe. Mitundu ya epidermolysis bullosa ndi:

• Epidermolysis bullosa simplex: matuza amapezeka m'munsi mwa epidermis.
• Borderline epidermolysis bullosa: Matuza amapezeka pamwamba pa nembanemba yapansi chifukwa cha zovuta zomata pakati pa epidermis ndi nembanemba yapansi.
• Dystrophic epidermolysis bullosa: Matuza amapezeka kumtunda kwa dermis chifukwa cha zovuta zomata pakati pa nembanemba yapansi ndi dermis yapamwamba.
• Kindler's syndrome: matuza amapezeka m'magulu angapo a khungu, kuphatikiza nembanemba yapansi.

Ofufuza apeza mitundu yopitilira 30 ya matendawa, yomwe imagawidwa m'magulu anayi akuluakulu a epidermolysis bullosa. Podziwa zambiri za subtypes, madokotala akhoza kuganizira za kuchiza matendawa.  

Mtundu wachisanu wa matenda, anapeza epidermolysis bullosa, ndi osowa autoimmune matenda amene chitetezo cha m'thupi kuukira mtundu winawake wa kolajeni pakhungu la munthu. Nthawi zina izi zimachitika ndi matenda ena, monga kutupa kwamatumbo. Kaŵirikaŵiri mankhwala amachititsa matenda. Mosiyana ndi mitundu ina ya epidermolysis bullosa, zizindikiro zimatha kuwoneka pazaka zilizonse, koma anthu ambiri amakhala ndi zizindikiro zazaka zapakati.

Zizindikiro za epidermolysis bullosa

Zizindikiro za epidermolysis bullosa zimasiyana malinga ndi mtundu wa epidermolysis bullosa. Aliyense amene ali ndi vutoli ali ndi khungu losalimba lomwe limatuluka mosavuta komanso misozi. Zizindikiro zina, mwa mtundu ndi subtype, zikuphatikizapo zotsatirazi.

• Epidermolysis Bullosa Simplex ndi matenda ofala kwambiri. Anthu omwe ali ndi mtundu wofatsa amakhala ndi matuza m'manja mwawo komanso pansi pa mapazi awo. Zina, zowopsa kwambiri, matuza amawonekera thupi lonse. Kutengera subtype ya matendawa, zizindikiro zina zingaphatikizepo:
  • Kukhuthala kwa khungu m'manja ndi m'mapazi.
  • Zikhadabo zolimba, zokhuthala, kapena zosowa zala kapena zala.
  • Matuza m'kamwa.
  • Kusintha kwa mtundu (mtundu) wa khungu.
• Bullous nodular epidermolysis nthawi zambiri zolemera. Anthu omwe ali ndi mawonekedwe owopsa kwambiri amatha kukhala ndi matuza otseguka kumaso, torso, ndi miyendo, zomwe zimatha kutenga kachilomboka kapena kutaya madzi m'thupi chifukwa chotaya madzi. Matuza amathanso kukula mkamwa, kummero, kumtunda kwa kupuma, m'mimba, matumbo, mkodzo, ndi kumaliseche. Zizindikiro zina ndi mavuto okhudzana ndi matendawa zingaphatikizepo:
  • Zikhadabo zolimba ndi zokhuthala kapena zosowa ndi zikhadabo.
  • Maonekedwe a khungu loonda.
  • Matuza pamutu kapena kuthothoka tsitsi ndi zipsera.
  • Kuperewera kwa zakudya m'thupi chifukwa chodya zakudya zopatsa mphamvu zopatsa mphamvu komanso mavitamini chifukwa cha matuza m'kamwa ndi m'mimba. 
  • Anemia
  • Kukula kwapang'onopang'ono.
  • Zosapanga bwino dzino enamel.
• Bullous dystrophic epidermolysis ali ndi zizindikiro zosiyana pang'ono, kutengera ngati matendawa ndi aakulu kapena ochuluka; komabe, anthu ambiri ali ndi recessive subtype.
  • Recessive subtype: Zizindikiro zimayambira pang'ono mpaka zowopsa ndipo zingaphatikizepo:
    • Matuza nthawi zambiri amawonekera pazigawo zazikulu za thupi; nthawi zina, matuza amatha kuwoneka pamapazi, m'zigongono, ndi mawondo okha.
    • Kutaya misomali kapena misomali yolimba kapena yokhuthala.
    • Kuphulika kwa khungu, komwe kungapangitse khungu kukhala lolimba kapena lochepa.
    • Milia ndi tokhala ting'onoting'ono toyera pakhungu.
    • Kuyabwa
    • Anemia
    • Kukula kwapang'onopang'ono.

Mitundu yambiri ya recessive subtype ingayambitse kukhudzidwa kwa maso, kutayika kwa mano, kutuluka m'kamwa ndi m'mimba, ndi kuphatikizika kwa zala kapena zala. Chiwopsezo chokhala ndi khansa yapakhungu chimakhalanso chachikulu. Khansara iyi imakonda kukula ndikufalikira mwachangu mwa anthu omwe ali ndi epidermolysis bullosa kuposa omwe alibe matendawa.

• • Subtype yayikulu: Zizindikiro zingaphatikizepo:
    • Matuza okha pa mikono, miyendo, zigongono ndi mawondo.
    • Kusintha mawonekedwe a misomali kapena kugwa kuchokera ku misomali.
    • Milia.
    • Matuza m'kamwa.

• Kindler syndrome alibe subtypes, ndipo matuza akhoza kupanga mu zigawo zonse za khungu. Matuza nthawi zambiri amawonekera m'manja ndi m'miyendo ndipo, zikavuta kwambiri, amafalikira ku ziwalo zina za thupi, kuphatikizapo kummero ndi chikhodzodzo. Zizindikiro zina ndi khungu lopyapyala, lokwinya; mabala; miliyamu; ndi kukhudzidwa kwa khungu ndi kuwala kwa dzuwa.

Zifukwa za epidermolysis bullosa

Kusintha (kusintha) kwa majini otengera makolo kumayambitsa mitundu yambiri ya epidermolysis bullosa. Majini amakhala ndi chidziŵitso chimene chimatsimikizira makhalidwe amene makolo anu amakupatsirani. Tili ndi makope awiri a majini athu ambiri, imodzi kuchokera kwa kholo lililonse. Anthu omwe ali ndi vutoli ali ndi jini imodzi kapena zingapo zomwe zimakhala ndi malangizo olakwika opangira mapuloteni ena pakhungu.

Pali mitundu iwiri ya cholowa:

• Dominant, zomwe zikutanthauza kuti mumatengera kopi imodzi yabwinobwino komanso jini imodzi yomwe imayambitsa epidermolysis bullosa. Kope losazolowereka la jini limakhala lamphamvu kapena "likulamulira" mawonekedwe abwinobwino a jini, zomwe zimayambitsa matenda. Munthu amene ali ndi kusintha kwakukulu amakhala ndi mwayi wa 50% (1 mwa 2) wopatsira matendawa kwa mwana aliyense.
• Zowonjezereka, zomwe zikutanthauza kuti makolo anu alibe vutoli, koma makolo onse awiri ali ndi jini yosadziwika bwino yomwe imayambitsa epidermolysis bullosa. Makolo onse akakhala ndi majini ochulukirachulukira, pali mwayi wa 25% (1 mwa 4) wokhala ndi mwana wokhala ndi vutoli pamimba iliyonse. Pali mwayi wa 50% (2 mwa 4) woti mimba ikhale ndi mwana yemwe adzalandira jini imodzi yosadziwika bwino, zomwe zimapangitsa kuti akhale chonyamulira. Ngati kholo limodzi lili ndi kusintha kwa majini, ana awo onse adzakhala ndi jini yosadziwika bwino, koma sadzakhala ndi epidermolysis bullosa.

Ofufuza amadziwa kuti epidermolysis bullosa yomwe imapezeka ndi matenda a autoimmune, koma sadziwa chomwe chimapangitsa kuti thupi liwukire collagen pakhungu la munthu. Nthawi zina, anthu omwe ali ndi matenda amtundu wa autoimmune amakhalanso ndi epidermolysis bullosa. Nthawi zina, mankhwala amachititsa matendawa.