Epidermolysis bullosa

Epidermolysis bullosa (EB) rukuni ne na rikice-rikice wanda kumburin fata keyi bayan ƙananan rauni. An wuce shi cikin dangi.

Akwai manyan nau'ikan EB guda huɗu. Sune:

• Dystrophic epidermolysis bullosa
• Epidermolysis bullosa simplex
• Hemidesmosomal epidermolysis bullosa
• Hanyar epidermolysis bullosa

Wani nau'ikan EB mai mahimmanci ana kiransa epidermolysis bullosa acquisita. Wannan salon yana tasowa bayan haihuwa. Rashin lafiya ne na rashin lafiyar jiki, wanda ke nufin jiki ya afka wa kansa da kansa.

EB na iya bambanta daga ƙarami zuwa m. Minoraramin sifa yana haifar da ɓarkewar fata. Siffar mai cutarwa tana shafar wasu gabobin. Yawancin nau'ikan wannan yanayin suna farawa ne daga haihuwa ko kuma jim kadan bayan haka. Zai yi wuya a gano ainihin nau'in EB da mutum yake da shi, kodayake takamaiman alamun alamomin yanzu suna samuwa ga mafi yawa.

Tarihin iyali yana da haɗari. Hadarin yafi girma idan iyaye suna da wannan yanayin.

Dangane da nau'in EB, alamun cututtuka na iya haɗawa da:

• Alopecia (asarar gashi)
• Buruji a kusa da idanu da hanci
• Bunƙasa a ciki ko kusa da bakin da maƙogwaro, yana haifar da matsalolin ciyarwa ko wahalar haɗiye
• Buruji akan fata sakamakon ƙananan rauni ko canjin yanayin zafin jiki, musamman ƙafa
• Fuskantarwa wacce ke haihuwa yayin haihuwa
• Matsalar hakora kamar lalacewar haƙori
• Kuka mai zafi, tari, ko wasu matsaloli na numfashi
• Whiteananan farin kumburi akan fata da ta ji rauni a baya
• Rashin ƙusa ko ƙusoshin ƙusa

Mai ba da lafiyar ku zai kalli fatarku don tantance cutar EB.

Gwaje-gwajen da ake amfani dasu don tabbatar da cutar sun hada da:

• Gwajin kwayoyin halitta
• Gwajin fata
• Gwaje-gwaje na musamman na samfuran fata a ƙarƙashin microscope

Za'a iya amfani da gwajin fata don gano nau'in EB.

Sauran gwaje-gwajen da za'a iya yi sun haɗa da:

• Gwajin jini don karancin jini
• Al'adar da za'a bincika cutar kwayan cuta idan raunuka suna warkewa da kyau
• Endarshen endoscopy ko jerin GI na sama idan alamun sun haɗa da matsalolin haɗiyewa

Za'a bincika saurin girma sau da yawa don jaririn da ke da ko kuma yana da EB.

Manufar magani ita ce hana bazuwar samuwarta da kuma guje wa matsaloli. Sauran maganin zai dogara ne da irin mummunan yanayin da yanayin yake.

Kulawar gida

Bi waɗannan jagororin a gida:

• Kula da fata sosai don kiyaye kamuwa da cututtuka.
• Bi shawarwarin mai ba ka idan wuraren da ke da ƙyalli sun zama ɓawatacce ko ɗanye. Kuna iya buƙatar maganin whirlpool na yau da kullun kuma don amfani da maganin shafawa na rigakafi zuwa yankuna masu kama da rauni. Mai ba ku sabis zai sanar da ku idan kuna buƙatar bandeji ko sutura, kuma idan haka ne, wane nau'in amfani da shi.
• Kila buƙatar amfani da magungunan steroid na baka na ɗan gajeren lokaci idan kuna da matsalolin haɗiye. Hakanan zaka iya buƙatar shan magani idan ka sami ciwon kandụ (yisti) a cikin bakin ko maƙogwaro.
• Kula sosai da lafiyar baki sannan a ringa duba lafiyar hakora. Zai fi kyau a ga likitan hakori wanda ke da ƙwarewar kula da mutane da EB.
• Ku ci abinci mai kyau. Lokacin da kake fama da rauni na fata, zaka iya buƙatar ƙarin adadin kuzari da furotin don taimakawa fatarka ta warke. Zabi abinci mai laushi kuma ku guji kwayoyi, kwakwalwan kwamfuta, da sauran kayan abinci masu raɗaɗi idan kuna da rauni a bakinku. Masanin ilimin abinci mai gina jiki zai iya taimaka muku game da abincinku.
• Yi aikin motsa jiki na jiki ya nuna maka don taimakawa ci gaba da haɗin gwiwa da tsokoki ta hannu.

Tiyata

Yin aikin tiyata don magance wannan yanayin na iya haɗawa da:

• Skin fata a wuraren da sosai suke da zurfi
• Raguwa (faɗaɗa) na esophagus idan akwai taƙaitawa
• Gyara nakasar hannu
• Cire duk wani ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar cuta

SAURAN MAGUNGUNA

Sauran jiyya don wannan yanayin na iya haɗawa da:

• Za'a iya amfani da magungunan da ke dankwafar da tsarin na rigakafi don yanayin autoimmune na wannan yanayin.
• Ana nazarin furotin da maganin jinsi da amfani da maganin interferon.

Hangen nesa ya dogara da tsananin rashin lafiyar.

Kamuwa da cututtukan wurare masu laushi abu ne gama gari.

Formsananan siffofin EB sun inganta tare da shekaru. Siffofin EB masu tsananin gaske suna da saurin mutuwa.

A cikin siffofin masu tsanani, tabo bayan fatar jiki na iya haifar da:

• Lalacewar kwangila (misali, a yatsu, gwiwar hannu, da gwiwoyi) da sauran nakasassu
• Matsalar hadiya idan bakin da majina sun shafi
• Yatsun hannu da yatsu
• Untataccen motsi daga tabo

Wadannan rikitarwa na iya faruwa:

• Anemia
• Rage tsawon rayuwa don mummunan yanayin yanayin
• Sountataccen sifa
• Matsalar idanu, gami da makanta
• Kamuwa da cuta, gami da sepsis (kamuwa da cuta a cikin jini ko kyallen takarda)
• Rashin aiki a hannu da ƙafa
• Ystwayar tsoka
• Cutar lokaci-lokaci
• Tsananin rashin abinci mai gina jiki wanda ya haifar da wahalar ciyarwa, wanda ke haifar da rashin cin nasara
• Cutar sankara ta fata

Idan jaririnku yana da wani laushi jim kadan bayan haihuwarsa, kira mai ba ku. Idan kuna da tarihin iyali na EB kuma kuna shirin samun yara, kuna iya samun shawarwarin kwayoyin halitta.

An ba da shawarar yin shawarwari game da kwayar halitta ga iyaye masu zuwa waɗanda ke da tarihin iyali na kowane nau'i na epidermolysis bullosa.

A lokacin daukar ciki, ana iya amfani da gwajin da ake kira samin samfurin chorionic villus don gwada jaririn. Ga ma'aurata da ke cikin haɗarin samun ɗa tare da EB, ana iya yin gwajin a farkon mako na 8 zuwa 10 na ciki. Yi magana da mai baka.

Don hana lalacewar fata da kumburi, sanya kwalliya a kewayen wuraren da ke fama da rauni kamar gwiwar hannu, gwiwoyi, idon sawu, da gindi. Guji wasannin tuntuba.

Idan kana da sayayyar EB kuma suna kan steroids fiye da wata 1, zaka iya buƙatar ƙwayoyin calcium da bitamin D. Wadannan kari na iya taimakawa wajen hana kasusuwa da kasusuwa (kasusuwa kasusuwa).

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

• Epidermolysis bullosa, mamaye dystrophic
• Epidermolysis bullosa, dystrophic

Denyer J, Pillay E, Clapham J. Mafi kyawun Sharuɗɗan forabi'a don Kulawa da Rauni a Epidermolysis Bullosa: Yarjejeniyar Internationalasa. London, UK: Raunuka na Duniya; 2017.

Lafiya, JD, Mellerio JE. A cikin: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2018: babi na 32.

Habif TP. Ciwon jijiyoyin jini da bullous. A cikin: Habif TP, ed. Clinical Dermatology. Na 6 ed. Philadelphia, PA: Elsevier; 2016: babi na 16.