Pemphigus

Overview of pemphigus

Pemphigus yog ib yam kab mob uas ua rau cov hlwv ua rau ntawm daim tawv nqaij thiab sab hauv ntawm lub qhov ncauj, qhov ntswg, caj pas, qhov muag, thiab qhov chaw mos. Tus kab mob no tsis tshua muaj nyob hauv Tebchaws Meskas.

Pemphigus yog ib hom kab mob autoimmune uas lub cev tiv thaiv kab mob ua yuam kev tawm tsam cov hlwb nyob rau saum txheej ntawm daim tawv nqaij (epidermis) thiab cov mucous membranes. Cov neeg uas muaj tus mob no tsim cov tshuaj tiv thaiv kab mob tiv thaiv desmogleins, cov proteins uas khi cov tawv nqaij rau ib leeg. Thaum cov ntawv cog lus no tawg, daim tawv nqaij ua nkig thiab cov kua dej tuaj yeem sib sau ntawm nws cov khaubncaws sab nraud povtseg, tsim cov hlwv.

Muaj ntau ntau hom pemphigus, tab sis ob lub ntsiab yog:

• Pemphigus vulgaris, uas feem ntau cuam tshuam rau ntawm daim tawv nqaij thiab mucous daim nyias nyias, xws li sab hauv ntawm lub qhov ncauj.
• Pemphigus foliaceus, tsuas yog cuam tshuam rau ntawm daim tawv nqaij.

Tsis muaj kev kho rau pemphigus, tab sis nyob rau hauv ntau zaus nws tuaj yeem tswj tau nrog tshuaj.

Leej twg tau pemphigus?

Koj yuav tau txais pemphigus yog tias koj muaj qee yam kev pheej hmoo. Qhov no suav nrog:

• haiv neeg keeb kwm. Thaum pemphigus tshwm sim ntawm cov haiv neeg thiab haiv neeg, qee cov neeg muaj kev pheej hmoo ntau dua rau qee hom kab mob. Cov neeg Yudais (tshwj xeeb tshaj yog Ashkenazi), Khab, Sab Hnub Tuaj Tebchaws Europe, lossis Middle Eastern caj ces muaj kev cuam tshuam ntau dua rau pemphigus vulgaris.
• Geographic txoj hauj lwm. Pemphigus vulgaris yog hom ntau tshaj plaws thoob ntiaj teb, tab sis pemphigus foliaceus muaj ntau dua nyob rau qee qhov chaw, xws li qee qhov chaw nyob deb nroog hauv Brazil thiab Tunisia.
• Tub los ntxhais thiab hnub nyoog. Cov poj niam tau txais pemphigus vulgaris ntau dua li cov txiv neej, thiab lub hnub nyoog pib feem ntau yog nyob nruab nrab ntawm 50 thiab 60 xyoo. Pemphigus foliaceus feem ntau cuam tshuam rau txiv neej thiab poj niam sib npaug, tab sis hauv qee cov pej xeem, poj niam cuam tshuam ntau dua li txiv neej. Txawm hais tias lub hnub nyoog pib ntawm pemphigus foliaceus feem ntau yog nyob nruab nrab ntawm 40 thiab 60 xyoo, hauv qee qhov chaw, cov tsos mob yuav tshwm sim thaum menyuam yaus.
• Cov noob. Cov kws tshawb fawb ntseeg tias qhov tshwm sim ntau dua ntawm tus kab mob hauv qee cov pej xeem yog vim yog ib feem ntawm caj ces. Piv txwv li, cov ntaub ntawv qhia tau hais tias qee yam txawv hauv tsev neeg ntawm lub cev tiv thaiv kab mob hu ua HLA yog txuam nrog kev pheej hmoo siab ntawm pemphigus vulgaris thiab pemphigus foliaceus.
• Tshuaj. Tsis tshua muaj, pemphigus tshwm sim los ntawm kev noj qee yam tshuaj, xws li qee yam tshuaj tua kab mob thiab tshuaj ntshav siab. Cov tshuaj uas muaj cov tshuaj hu ua thiol kuj tau txuas nrog pemphigus.
• Crayfish. Muaj tsawg zaus, kev loj hlob ntawm cov qog, tshwj xeeb tshaj yog kev loj hlob ntawm cov qog nqaij hlav, tonsil lossis thymus caj pas, tuaj yeem ua rau tus kab mob.

Hom pemphigus

Muaj ob hom ntawv tseem ceeb ntawm pemphigus thiab lawv raug cais raws li txheej ntawm daim tawv nqaij qhov chaw hlwv thiab qhov chaw hlwv nyob rau hauv lub cev. Hom tshuaj tiv thaiv tawm tsam ntawm daim tawv nqaij hlwb kuj pab txiav txim seb hom pemphigus.

Ob yam tseem ceeb ntawm pemphigus yog:

• Pemphigus vulgaris yog hom ntau tshaj plaws hauv Tebchaws Meskas. Blisters tsim nyob rau hauv lub qhov ncauj thiab nyob rau lwm yam mucosal nto, nrog rau ntawm daim tawv nqaij. Lawv tsim nyob rau hauv qhov sib sib zog nqus khaubncaws sab nraud povtseg ntawm lub epidermis thiab feem ntau mob. Muaj ib tug subtype ntawm tus kab mob hu ua pemphigus autonomicus, nyob rau hauv uas hlwv tshwm sim nyob rau hauv lub puab tsaig thiab nyob rau hauv lub armpits.
• Nplooj pemphigus tsawg dua thiab cuam tshuam rau ntawm daim tawv nqaij xwb. Blisters tsim nyob rau hauv lub Upper txheej ntawm lub epidermis thiab tej zaum yuav khaus los yog mob.

Lwm yam tsawg kawg ntawm pemphigus muaj xws li:

• Paraneoplastic pemphigus. Hom no yog tus cwj pwm los ntawm qhov ncauj thiab daim di ncauj, tab sis feem ntau kuj blistering los yog o ntawm daim tawv nqaij thiab lwm yam mucous daim nyias nyias. Nrog rau hom no, mob ntsws hnyav tuaj yeem tshwm sim. Cov neeg uas muaj tus kab mob no feem ntau muaj cov qog, thiab tus kab mob yuav zoo dua yog tias cov qog raug tshem tawm.
• IgA pemphigus. Daim ntawv no yog tshwm sim los ntawm ib hom tshuaj tiv thaiv hu ua IgA. Cov pob khaus lossis pob khaus feem ntau tshwm sim hauv pawg lossis cov nplhaib ntawm daim tawv nqaij.
• medicinal pemphigus. Qee cov tshuaj, xws li qee cov tshuaj tua kab mob thiab cov tshuaj ntshav siab, thiab cov tshuaj uas muaj cov tshuaj hu ua thiol, tuaj yeem ua rau pob khaus lossis pob khaus zoo li pemphigus. Cov hlwv thiab mob feem ntau yuav ploj mus thaum koj tsis noj tshuaj.

Pemphigoid yog ib yam kab mob uas txawv ntawm pemphigus tab sis qhia qee yam nta. Pemphigoid ua rau kev sib cais ntawm qhov sib txuas ntawm cov epidermis thiab cov dermis hauv qab, ua rau cov hlwv tob tob uas tsis yooj yim tawg.

Cov tsos mob ntawm pemphigus

Cov tsos mob tseem ceeb ntawm pemphigus yog blistering ntawm daim tawv nqaij thiab, qee zaum, mucous daim nyias nyias xws li qhov ncauj, qhov ntswg, caj pas, qhov muag, thiab qhov chaw mos. Cov hlwv yog nkig thiab zoo li tawg, ua rau mob hnyav. Cov hlwv ntawm daim tawv nqaij yuav coalesce, tsim cov tawv nqaij tawv uas ua rau kis kab mob thiab tsim cov kua dej ntau. Cov tsos mob txawv me ntsis nyob ntawm seb hom pemphigus.

• Pemphigus vulgaris Cov hlwv feem ntau pib hauv qhov ncauj, tab sis lawv yuav tshwm sim tom qab ntawm daim tawv nqaij. Daim tawv nqaij tuaj yeem ua nkig kom nws flakes tawm thaum rub nrog tus ntiv tes. Cov mucous membranes xws li lub qhov ntswg, caj pas, qhov muag, thiab qhov chaw mos kuj raug cuam tshuam.

  Cov hlwv tsim nyob rau hauv txheej tob ntawm cov epidermis thiab feem ntau mob.

• Nplooj pemphigus tsuas cuam tshuam rau ntawm daim tawv nqaij. Cov pob khaus feem ntau tshwm sim ntawm lub ntsej muag, tawv taub hau, hauv siab, lossis sab nraub qaum, tab sis lawv tuaj yeem kis mus rau lwm qhov chaw ntawm lub cev thaum lub sijhawm. Qhov chaw ntawm daim tawv nqaij yuav ua mob thiab flaky nyob rau hauv cov khaubncaws sab nraud povtseg los yog nplai. Blisters tsim nyob rau hauv lub Upper txheej ntawm lub epidermis thiab tej zaum yuav khaus los yog mob.

Ua rau pemphigus

Pemphigus yog ib hom kab mob autoimmune uas tshwm sim thaum lub cev tiv thaiv kab mob tawm tsam cov tawv nqaij noj qab haus huv. Cov tshuaj tiv thaiv kab mob hu ua antibodies tsom cov proteins hu ua desmogleins, uas pab khi cov tawv nqaij nyob sib ze rau ib leeg. Thaum cov ntawv cog lus no tawg, daim tawv nqaij ua nkig thiab cov kua dej tuaj yeem sib sau ua ke ntawm cov txheej txheem ntawm cov hlwb, tsim cov hlwv.

Feem ntau, lub cev tiv thaiv kab mob tiv thaiv lub cev los ntawm kev kis kab mob thiab kab mob. Cov kws tshawb fawb tsis paub tias dab tsi ua rau lub cev tiv thaiv kab mob ua rau lub cev muaj protein ntau, tab sis lawv ntseeg tias cov caj ces thiab ib puag ncig muaj feem cuam tshuam. Qee yam hauv ib puag ncig tuaj yeem ua rau pemphigus hauv cov neeg uas muaj kev pheej hmoo vim lawv cov caj ces predisposition. Tsis tshua muaj, pemphigus tuaj yeem tshwm sim los ntawm qog nqaij hlav lossis qee yam tshuaj.