Pemphigoïde bulleuse

Chii ?

Bullous pemphigoid chirwere cheganda (dermatosis).

Iyo yekupedzisira inoratidzirwa nekuvandudzwa kwemabhuru makuru pane erythematous plaques (zvitsvuku zvitsvuku paganda). Kuonekwa kwemabhuru aya kunotungamirira kumaronda uye kazhinji ndicho chikonzero chekukwenya. (1)

Icho chirwere che autoimmune, mhedzisiro yekukanganiswa kweiyo immune system mumunhu akabatwa. Uyu murairo wemasoja ekudzivirira muviri unosanganisira kugadzirwa kweakananga masoja ekudzivirira chirwere anorwisa muviri wayo.

Iyi pathology haiwanzo asi inogona kuve yakakomba. Zvinoda kurapwa kwenguva refu. (1)

Kunyangwe chiri chirwere chisingawanzo, zvakare ndicho chakajairika che autoimmune bullous dermatoses. (2)

Kuwanda kwayo ndeye 1/40 (nhamba yezviitiko pamugari wega wega) uye inonyanya kubata vanhu vakuru (paavhareji makore anenge 000 ekuzvarwa, ane njodzi yakawedzera kuvakadzi).

Chimiro chemucheche chiripowo uye chinokanganisa mwana mugore rokutanga rehupenyu hwake. (3)

zviratidzo

Bullous pemphigoid chirwere che autoimmune dermatosis. Musoro unotambura nechirwere ichi nokudaro unoburitsa masoja ekudzivirira chirwere anorwisa muviri wake (autoantibodies). Aya anorwisa marudzi maviri emapuroteni: AgPB230 neAgPB180 iri pakati pematanho maviri ekutanga eganda (pakati pedermis uye epidermis). Nekukonzera mubatanidzwa pakati pezvikamu zviviri izvi zveganda, idzi auto-antibodies dzinotungamira pakuumbwa kwemabhubhu maitiro echirwere. (1)

Atypical zviratidzo zvebullous pemphigoid kutaridzika kwemabhuru makuru (pakati pe3 ne4 mm) uye yeruvara rwakareruka. Aya mabhuru anonyanya kuitika apo ganda rakatsvuka (erythematous), asi rinogonawo kuonekwa paganda rine utano.

Epidermal maronda anowanzo kugariswa muhunde nemakumbo. Chiso chinowanzochengetedzwa. (1)

A pruritus yeganda (itching), dzimwe nguva mangwanani apo mabhubhu anoonekwa, inokoshawo yechirwere ichi.


Mhando dzakawanda dzechirwere dzakaratidzwa: (1)

- iyo yakajairika fomu, zviratidzo zvayo kutaridzika kwemabhuru makuru machena uye itching. Iyi fomu ndiyo inonyanya kuzivikanwa.

- iyo vesicular fomu, iyo inotsanangurwa nechitarisiko chedikidiki blister mumaoko nekukwenya kwakanyanya. Iyi fomu haina kunyanya kuwanda.

- iyo urticaria fomu: sezvinoratidzwa nezita rayo, inokonzeresa zvigamba zvemukoko zvichikonzera kuvaviwa kwakanyanya.

- iyo prurigo-yakaita fomu, iyo itching iyo inowedzera kupararira asi yakanyanya. Ichi chimiro chechirwere chinogonawo kukonzera kushaya hope munyaya yakabatwa. Mukuwedzera, haasi mabhubhu anozivikanwa nenzira yerudzi rweprurigo asi crusts.


Vamwe varwere vanogona kunge vasina chero zviratidzo. Vamwe vanotanga kutsvuka zvishoma, kuvaviwa, kana kusvotwa. Pakupedzisira, zviitiko zvinowanzoitika zvinokura kutsvuka uye kukwenya kwakanyanya.

Mapundu anogona kuputika oita maronda kana maronda akazaruka. (4)

Kwakatangira chirwere

Bullous pemphigoid inonzi autoimmune dermatosis.

Mabviro echirwere aya anokonzeresa kugadzirwa kwemasoja ekudzivirira chirwere (mapuroteni emushonga unodzivirira zvirwere) nemuviri uchirwisa masero awo. Uku kugadzirwa kweautoantibodies kunotungamira mukuparadzwa kwematishu uye / kana nhengo pamwe nekuzvimba maitiro.

Tsananguro chaiyo yechiitiko ichi haisati yazivikanwa. Zvakadaro, zvimwe zvinhu zvingave nechakananga kana chisina kunanga chinongedzo nekuvandudzwa kweautoantibodies. Izvi ndezvezvakatipoteredza, mahomoni, mishonga kana kunyange genetic factor. (1)

Aya masoja ekudzivirira chirwere anogadzirwa neari kubatwa akanangana nemapuroteni maviri: BPAG1 (kana AgPB230) uye BPAG2 (kana AgPB180). Mapuroteni aya ane basa rekuita mukusangana pakati pedermis (yezasi layer) uye epidermis (yepamusoro layer). Aya macromolecules ari kurwiswa ne autoantibodies, ganda rinosvuuka uye rinoita kuti mabubbles aoneke. (2)


Uye zvakare, hapana kutapurirana kunofanirwa kuve kwakabatana neiyi pathology. (1)

Uyezve, zviratidzo zvinowanzongoerekana zvaitika uye zvisingatarisirwi.

Bullous pemphigoid haisi, zvisinei: (3)

- utachiona;

- allergy;

- mamiriro ane chokuita nemararamiro kana kudya.

Zvinhu zvinotyisa

Bullous pemphigoid chirwere che autoimmune, mupfungwa iyoyo hachisi chirwere chakagara nhaka.

Kunyanguvezvo, kuvapo kwemamwe majini kwaizova ngozi yokuva nehosha muvanhu vanotakura aya majini. Pamwe pane imwe genetic predisposition.

Iyi njodzi yepredisposition, zvisinei, yakaderera zvikuru. (1)

Sezvo avhareji yezera rekukura kwechirwere yakapoteredza 70, zera remunhu rinogona kuva imwe ngozi yekuwedzera pemphigoid bullous.

Mukuwedzera, hatifanire kufuratira chokwadi chekuti chirwere ichi chinotsanangurwa zvakare kuburikidza nefomu remwana. (3)

Mukuwedzera, kuwedzera kuduku kwechirwere kunoonekwa muvakadzi. Bonde remukadzi saka rinoita kuti rive chinhu chine njodzi. (3)

Kudzivirira uye kurapwa

Kusiyanisa kusiyanisa kwechirwere kunonyanya kuonekwa: kuonekwa kwemabhuru akajeka muganda.

Kuongororwa uku kunogona kusimbiswa neganda biopsy (kutora sampuli kubva paganda rakakuvadzwa kuti riongororwe).

Kushandiswa kwe immunofluorescence kunogona kushandiswa mukuratidza masoja ekudzivirira chirwere zvichitevera kuongororwa ropa. (3)

Mishonga inotarwa mumamiriro ekuvapo kwebullous pemphigoid inovavarira kudzikamisa kukura kwemafuru uye kuporesa mabhuru atovepo paganda. (3)

Mushonga unowanzobatanidzwa nechirwere ndeye systemic corticosteroid therapy.

Zvisinei, nokuda kwemaitiro emunharaunda e-bullous pemphigoid, topical corticosteroid therapy (inoshanda chete apo mushonga unoshandiswa), yakasanganiswa nekirasi I dermatocorticoids (mushonga unoshandiswa mukurapa ganda remunharaunda). (2)

Mushonga wemishonga inorwisa mabhakitiriya wemhuri yetetracycline (dzimwe nguva inobatanidzwa nekutora vhitamini B) inogonawo kushanda nachiremba.

Kurapa kunowanzorongedzerwa kwenguva refu uye kunoshanda. Mukuwedzera, kudzoka kwechirwere dzimwe nguva kunoonekwa mushure mekumira kurapwa. (4)

Mushure mekuongororwa kwekuvapo kwebullous pemphigoid, kubvunza chiremba wedermatologist kunokurudzirwa zvakasimba. (3)

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