Pemphigus vulgaris: oonobangela, iimpawu, unyango, amayeza. izifo sokuhlasela

njani isifo, ezifana pemphigus? Treatment kunye iimpawu zesi sifo kuxoxwa ngezantsi. Uza kufunda kwakhona ngoonobangela yale nkqubo sifo nendlela isifo sakhe.

ulwazi olusisiseko

Pemphigus, photo inikwa kweli nqaku, - iqela kwezifo samathambo vesiculobullous onge kodwa ezinzima kakhulu, eya isiba kunye esibulalayo echaphazela ulusu kunye ngenwebu yangaphakathi.

Iingcali ukuchonga ntlobo zilandelayo sifo:

• pemphigus vulgaris;
• ifomu vegetans;
• igqabi;
• seborrhoeic (inokuba amagama afana Shenir-Usher syndrome, okanye erythematous).

iimpawu Jikelele sifo

vulgaris Pemphigus imele udidi izifo ulusu kunye izihlunu ongama, ophuma ngendlela esinganyangekiyo, likakade rhoqo.

Esi sifo hormone zixhomekeke. Lubonakala ngekhondo zenkqubela phambili, kwakunye ukuyilwa kumadyunguza intraepidermal.

Amaninzi, pemphigus vulgaris ichaphazela abantu abaneminyaka eyi-50 okanye ngaphezulu.

Kutheni le sifo kwenzeka? Izizathu kuphuhliso kwesi sifo, kukho kuphela yeengcinga.

Pemphigus: cishe izizathu

Phambi unyango, esi sifo ekuthethwa kubaluleke kakhulu ukufumana ukuba kutheni na kwavela sisifo samathambo evela isigulane ethile. Ngelishwa, ukufumana unobangela pemphigus kunzima. Iingcali ulwa nale ngxaki iminyaka engamashumi amaninzi. Ngeli xesha ke ziye babeka phambili hypothesis:

• yeemeko zangaphandle, okt ngokuthatha amayeza, kuquka penicillin kunye nezakhi zayo, kunye interferon ezahlukeneyo kunye nezinye izinto.
• izinto zemveli, kuquka omzimba kunye yemfuza.
• izinto Physical (asenokuzichaphazela bafumana ukutsha olubanzi radiation).
• Yonyango (ukusilela umz kwamadlala ebantwini).
• Virus (Herpesviruses).
• Ukuthatha ukutya okuthile.

Ngokusekelwe kwezi zinto, echaza isenzeko mhlawumbi zesi sifo, sigqiba: vulgaris pemphigus kunokuba ngumzimba, yonyango, ezosulelayo, neurogenic, ityhefu, njl Nakuba kunjalo, kufanele kuqatshelwe ukuba kusekho ayaziwa nokuba shintsho lokuqala okanye azinxibayo .. indalo zasesekondari, ekhawulelana nobangela.

Ngoko ke, lo nokungakwazi ukuchonga unobangela ngqo le ingqalelo isifo samathambo kukwaqhwalelisa kakhulu isigulo kwayo ngexesha elifanelekileyo, ngoko abantu abaninzi badla ukuphuhlisa iingxaki ezinzulu.

mechanism nucleation

Njani uphuhliso izifo ezifana, ezifana pemphigus? sigaba Ifoto yokuqala sifo, uyakwazi ukubona kweli nqaku. Iingcali zithi unobangela loo inkqubo autoaggressive pathologic Ukwakheka omzimba kwiiproteni ukubayinxalenye usapho desmoglein. Le yokugqibela bamele uhlobo "glue" bazihlanganisa iiseli epidermal ezikufutshane nge iimpawu ezizodwa kokuxhuma, ezo zibizwa ngokuba desmosomes.

lymphocyte kanye iyasebenza yaye autoantibodies kuhlasela desmogleins, iiseli epidermal yahlukanisiwe ukusuka kwenye nenye, kunye sangaphandle lube aneentunja yaye "baxhoma", ngaloo lula abade waza phantsi kwii-fungi ezahlukeneyo kunye neentsholongwane. Ukuziqhelisa lwezonyango, le nto kuthiwa acantholysis.

Ngenxa yokuba le nkqubo ingentla ogulayo namadyungudyungu kakhulu eluswini, ngokunjalo balo babulingana nesandla. Ngelo xesha linye ziyahlutha exudate kunye rhoqo yimbi. Ekuhambeni kwexesha, la maqamza abezichubile ungabikho amaphepha, exwebula buvele izicwili nokwakha ububomvu kunye nezilonda bosulelekile. Kwiimeko eziphambili, ukubunjwa loo bukugubungele kakhulu yonke kumphezulu umzimba.

ulwazi zembali

Ekuqaleni iingcali isifo dermatological abazi lutho. Igama elithi "pemphigus" kuzo zonke izitshanguba of uxakaxa kunye ulusu, acantholysis ephelekwa, ukubunjwa amaqamza kunye kwecala iimakhishithi kuphuhliso izilonda suppurating. Noko ke, ngo-1964 inqaku elalipapashwe kwijenali yezonyango, leyo batshintsha ukuqonda oogqirha kwi sifo ekuthethwa ngayo, kwakunye indlela yayo ku isifo kwaye afumane unyango. Ukususela ngelo xesha, ubukho antibodies egazini plasma izigulane ukuya desmogleina abe isibaluli sokuchonga pemphigus.

Hi ndlela leyi, ngo-1971 wapapasha elinye inqaku apho uhlobo samathambo le zaphandwa iinkcukachaa kunye indlela yophuhliso yesi sifo.

The main Iimpawu

Namadyungudyungu kakhulu eluswini, ezibangwa kuphuhliso bladderwort nekrwada, nokuba yengcolileyo, amele umqondiso lokuqala yokugula. Kufuneka kuqatshelwe ukuba olu hlobo lwesifo lwenzeka amaninzi. Ngokutsho kweengcali, ama-77% yazo zonke iintlobo ekhethiwe of pemphigus.

Ukwenzakala ngohlobo amaqamza asichaphazeli nje kuphela isikhumba yomguli, kodwa isifo emlonyeni nasemqaleni wayo. Kamva ke batwabulule phezu lomzimba, zangasese yangaphandle, ubuso njalo njalo.

Into ekufuneka uyazile nge ezi zifo ulusu? Ngokuqhelekileyo, pemphigus iphuhlisa ngesiquphe. Ngaloo esikhumbeni esempilweni ngokwangaphandle kwasekwa amaqamza ngezikhondo ezincinane ukuba ngokwesantya inzuzo imbonakalo appreciable iyathamba. imixholo yazo ulwelo serous ngaphaya (kancinane turbid).

Emva kokuvula i-papules kwakhiwa umphezulu layitshabalalisa, apho kamva ukuphilisa, kodwa ushiye imizila nombala brown.

Kuba isifo samathambo enjalo luphawulwa flow esinganyangekiyo yayinzima ifomu. Kufuneka kuqatshelwe ukuba abanye abantu ngaphandle unyango kwabonwa uphuculo zidityaniswe, apho indawo okubi.

izifo kakhulu Idla pemphigus vulgaris lesibini (candidiasis).

Ngenxa ilahleko protein, ulwelo kunye neziphumo ezasulelayo ALS kwesi sifo kunye neefom kakhulu ezimbi.

Ukukhomba izifo

Njengoko kutyhilwa vulgaris pemphigus? Lokufunyaniswa kwesifo oluqhutywa esibhedlele. Ubukho esi sifo ibanjiwe ngokusekelwe iimpawu zonyango kunye neziphumo wohlalutyo.

I yangaphambili ziquka:

• A uphawu asbestos-sikaHansen. Olu phawu ibanjiwe ngokucinezela umnwe okanye iglasi cover (ngamanye amazwi, kodwa yayize) kwiqamza. inkqubo enjalo lula kwecala sangaphandle kwindawo leyo omelene papules, ngokunjalo kwandiswa indawo yayo ngenxa uxinzelelo fluid phakathi kwiyunithi.
• Uphawu Nikolsky. Olu phawu ibanjiwe oonina kwiqamza entrainment kunye forceps kunye yokuwahlikihla inxalenye umnwe ngokwangaphandle isikhumba kakuhle kufuphi nesiza sisilonda. Ngenxa yoko kukho lamabutho sangaphandle.

Kufuneka kuqatshelwe ukuba pemphigus anento yokwenza ezi mpawu akukho ezithile, kunye uxilongo. Kufuneka kukhunjulwe ukuba ezinjalo ukuze kwenzeka kwezinye izifo.

laboratory

unesifo njani vulgaris pemphigus? Unyango lwesifo samathambo kufuneka lwenziwe kuphela emva kokuba axilongwe. Ukuze awayiboni isifo kusetyenziswa ezifana:

• Uhlalutyo Histological, ebandakanya iimviwo smears, okanye ekuthiwa iminwe ukuchonga amaqamza iiseli acantholytic (oko kukuthi, iiseli epidermal ukuba ngokwemigaqo utshintsho morphological).

Kufuneka kuqatshelwe ukuba ngenxa yokuba uhlalutyo histological lwe data bagqibe ukuba uphuhliso isifo samathambo akunakwenzeka. Oku kungenxa yokuba kukho nezinye izifo nomfanekiso efanayo.

• indlela Immunofluorescence ukuze ukubona iidipozithi intracellular of immunoglobulin G and A, kwakunye ukuchonga nezakhi ezinkulu nezincinane - desmoglein 3 desmoglein-1. Le ndlela yeyona uxilongo echanekileyo.

Ngenxa yoko, uxilongo "pemphigus anento yokwenza 'obekwe ngokususela kwidatha ezongezelelekileyo iimpawu zonyango kunye umfanekiso kwezonyango sifo kunye neziphumo immunofluorescence ubuchule kunye noviwo histologic.

unyango

Ekuqaleni kuphuhliso pemphigus vulgaris izigulane ezimiselweyo glucocorticoids. Iziyobisi kweli qela ezifunyenweyo ngeedosi kukothuka. Ukuqeshwa beziyobisi izixa ezikhulu ubungqina ebalulekileyo. Ngokuphathelele Izimo kunye neziphumo ebezingalindelekanga, ukuba aphakamileyo.

Kuthekani ukuba esi sifo umbuzo amisele glucocorticoids? Amalungiselelo yonyango puzyrchaki nekrwada inokuba ngolu hlobo lulandelayo: "Prednisolone" "Dexamethasone" yaye "Triamptsinolon".

Xa ukuphuculwa isigulane, ngamanye amazwi xa kungekho ngamaqamza ezintsha, ithamo ka iziyobisi licuthwa ngokucuthwa kwaye za uhambisa i axhasayo ukwenzela ukuthintela ukwenzeka. unyango olunjalo zezigulane elide ngokwaneleyo.

Ngaphandle glucocorticoids, izigulane angatyumba arhente cytotoxic, immunosuppressants, kuquka "Methotrexate", "Azathioprine" okanye "Prospidin". Ukuba ziyimfuneko ukususa iingxaki yendlela egwenxa emva kokungenisa amachiza ezisisiseko.

Ezinye iindlela zonyango

Ukuba kukho iimpawu ezibonisa ukuba izigulane isifo samathambo zinokunyangwa kunye nemithi, kunye neziyobisi ezixhasa inkqubo sentliziyo, elawula uxinzelelo lwegazi ze ukubuyisela ukusebenza kwesibindi kunye nezintso.

Kwakhona kufuneka potassium aqulethe agents, calcium kunye neevithamini. Kuba unyango ngezihloko amachiza-ukudumba pemphigus, infusions kunye decoctions womfuno ezinokusetyenziswa.

Kufuneka kuqatshelwe ukuba, ngokuxhomekeke kwimeko yomguli kusenokuba yabelwe iinkqubo ezifana gemabsorbtsiya, ukutofelwa igazi plasmapheresis.