Koj puas muaj cov pob khaus zoo li no ntawm koj daim tawv nqaij? Cia peb kawm txog Brooke-Spiegler Syndrome.

Koj puas tau pom muaj cov pob me me ntawm koj daim tawv nqaij, tshwj xeeb tshaj yog ntawm koj lub ntsej muag, caj dab, lossis taub hau, uas ua rau koj ntxhov siab? Tej zaum koj yuav tsis paub tias lawv yog dab tsi lossis vim li cas lawv thiaj tshwm sim. Hnub no peb yuav tham txog ib qho mob ntawm daim tawv nqaij uas tuaj yeem ua rau muaj cov pob no, tab sis nws tsis tshua muaj, txhais tau tias nws tsis tshwm sim rau txhua tus. Qhov no hu ua Brooke-Spiegler Syndrome. Tsis txhob txhawj, cia peb tham txog qhov no yooj yim.

Brooke-Spiegler Syndrome yog dab tsi?

Hais yooj yim xwb, Brooke-Spiegler Syndrome yog ib yam mob tawv nqaij uas tsis tshua muaj . Nws ua rau muaj pob (tseem hu ua qog nqaij hlav) rau ntawm koj daim tawv nqaij, tshwj xeeb tshaj yog ntawm koj lub ntsej muag, caj dab, thiab taub hau. Qee zaum cov pob no kuj tuaj yeem tsim rau lwm qhov ntawm koj lub cev. Feem ntau, lawv pib tshwm sim thaum koj tseem hluas, thaum muaj hnub nyoog kaum yim lossis nees nkaum xyoo .

Thaum nws los txog rau cov pob no, feem ntau lawv tsis yog mob qog noj ntshav (uas yog, lawv yog 'benign'), uas yog, lawv yog benign. Txawm li cas los xij, peb kuj yuav tsum nco ntsoov tias, tsis tshua muaj, dhau sijhawm, lawv tuaj yeem dhau los ua mob qog noj ntshav (uas yog, lawv yog 'malignant'). Qee zaum, thaum cov pob benign no loj hlob, lawv tuaj yeem raug mob, dhau los ua 'qhov txhab qhib', thiab tuaj yeem ua rau muaj kev kis kab mob.

Feem ntau tus mob no yog los ntawm koj niam koj txiv, uas txhais tau tias nws yog ib qho mob caj ces . Txawm li cas los xij, tsis tshua muaj ib tus neeg uas muaj tus mob Brooks-Spiegel syndrome txawm tias tsis muaj leej twg hauv tsev neeg muaj tus mob no los xij.

Qhov zoo tshaj plaws yog, muaj kev kho rau qhov no. Feem ntau, kev phais yog tib txoj kev xaiv. Yog tias kho kom zoo, feem ntau cov neeg uas muaj tus mob no tuaj yeem ua neej nyob zoo li qub, zoo siab, thiab nquag.

Puas muaj lwm lub npe rau qhov no?

Yog, cov kws kho mob siv ntau lub npe rau tus mob Brooke-Spiegler no. Tej zaum koj kuj yuav hnov ​​​​​​cov npe no:

• Ancell-Spiegler cylindromas
• CYLD cutaneous syndrome (CCS)
• Kab mob cylindromatosis hauv tsev neeg (FC)
• Ntau tsev neeg trichoepitheliomas (MFT)

Txawm hais tias cov npe no yuav zoo li nyuaj me ntsis, lawv txhua tus yog cov npe rau tib yam mob.

Qhov teeb meem no feem ntau tshwm sim li cas?

Qhov no yog ib qho mob tsis tshua muaj . Cov kws tshawb fawb hais tias muaj li ib ntawm ib lab tus tib neeg uas muaj tus mob Brooks-Spiegel syndrome. Yog li, nws tsis yog ib yam dab tsi uas tshwm sim ntau heev.

Cov tsos mob yog dab tsi? Saib seb koj puas muaj cov no thiab...

Cov tsos mob tseem ceeb ntawm tus mob no yog kev tsim cov pob tawv nqaij . Cov no feem ntau pib ntawm koj lub ntsej muag, caj dab, thiab taub hau. Cov pob no feem ntau yog puag ncig thiab tuaj yeem loj li ntawm 0.5 txog 3 centimeters (cm).

Raws li lub sijhawm dhau mus, cov qog no tuaj yeem nce ntxiv ntau thiab loj zuj zus. Qee zaum lawv tuaj yeem ua rau lub cev puas tsuaj. Feem ntau, cov qog no mob heev . Tsis tas li ntawd, yog tias lawv tsim rau ntawm daim tawv nqaij ntawm thaj chaw poj niam txiv neej, lawv tuaj yeem ua rau tsis muaj zog sib deev. Lwm yam yog tias qee zaum cov qog no tuaj yeem kis mus thoob plaws lub taub hau thiab ua rau plaub hau poob . Cov no kuj tuaj yeem ua rau muaj kev nyuaj siab.

Ntxiv rau cov pob no, tej zaum koj yuav pom cov khoom me me, dawb, zoo li cov hlwv (milia) ntawm koj daim tawv nqaij. Cov 'milia' no tsis ua rau muaj kev phom sij, tab sis lawv tuaj yeem ua rau qee tus neeg mob.

Cov qog no yog dab tsi? (Hom qog ntawm daim tawv nqaij hauv BSS)

Muaj peb hom qog tseem ceeb uas pom muaj nyob rau hauv Brooke-Spiegler syndrome:

1. Cylindromas: Cov no feem ntau yog cov pob dawb mos mos. Lawv feem ntau tshwm sim rau ntawm lub taub hau, qhov chaw uas muaj cov plaub hau follicles. Lawv kuj tuaj yeem tshwm sim rau ntawm lub ntsej muag, pob ntseg, thiab tsis tshua muaj nyob rau hauv lub ntsws. Lawv tuaj yeem muaj qhov loj me ntawm ob peb millimeters mus rau ob peb centimeters. Thaum lawv tshwm sim rau ntawm lub taub hau, lawv tuaj yeem sib sau ua ke thiab zoo li daim ntawm daim duab sib dhos.

2. Spiradenomas: Cov no yog cov pob tawv nqaij uas tawv thiab tawv nqaij. Feem ntau pom muaj nyob rau ntawm lub taub hau, caj dab, thiab lub cev sab saud. Lawv tuaj yeem loj li ntawm tsawg dua ib centimeter mus txog ob peb centimeters. Feem ntau lawv yog xim xiav lossis xim tawv nqaij. Tab sis lawv kuj tuaj yeem yog xim grey, liab dawb, ntshav, liab, lossis daj. Spiradenomas qee zaum tuaj yeem mob.

3. Trichoepitheliomas: Cov no kuj yog cov pob tawv, ci ntsa iab. Lawv feem ntau tshwm sim ntawm lub ntsej muag, hauv cov cheeb tsam uas muaj plaub hau. Lawv feem ntau me dua ib centimeter. Lawv tuaj yeem yog xiav, xim av, xim tawv nqaij, liab dawb, lossis daj.

Qhov tseem ceeb yog tias qee zaum hauv Brook-Spiegler syndrome no, cov yam ntxwv ntawm ob lossis peb hom qog no tuaj yeem pom hauv tib lub qog.

Tus mob no puas cuam tshuam rau lwm qhov ntawm lub cev?

Yog, qee zaum cov qog no tuaj yeem tshwm sim rau lwm qhov ntawm lub cev. Hauv cov xwm txheej zoo li no, kev ua haujlwm ntawm cov kabmob ntawd tuaj yeem raug cuam tshuam. Piv txwv li:

• Yog nws tsim nyob rau hauv txoj hlab pa , nws tuaj yeem ua rau ua pa nyuaj.
• Yog tias nws tshwm sim hauv pob ntseg , kev hnov ​​​​lus yuav raug cuam tshuam.
• Yog tias nws tshwm sim rau hauv qhov muag , qhov muag yuav raug cuam tshuam.
• Yog tias lawv tsim rau ntawm qhov chaw mos , kev ua haujlwm ntawm kev sib deev yuav raug cuam tshuam.
• Yog tias nws tshwm sim hauv qhov ncauj thiab cov qog salivary , kev zom thiab nqos yuav raug cuam tshuam.
• Qhov ntswgYog tias nws tshwm sim, nws tuaj yeem cuam tshuam rau qhov hnov ​​​​​​tsw.

Vim li cas Brooke-Spiegler Syndrome thiaj tshwm sim?

Qhov laj thawj tseem ceeb rau qhov no yog kev hloov pauv caj ces (`kev hloov pauv`) hauv cov noob 'CYLD' hauv peb lub cev. Yuav kom koj muaj tus mob no, tsawg kawg yog ib tug ntawm koj niam koj txiv yuav tsum yog tus nqa cov kev hloov pauv caj ces no. Qhov no txhais tau tias nws tau dhau los ntawm ib tiam dhau ib tiam .

Txawm li cas los xij, tsis tshua muaj ib tus neeg uas tsis muaj keeb kwm tsev neeg muaj tus kab mob no tuaj yeem tsim cov kev hloov pauv gene no ('de novo mutation') hauv lawv lub cev. Hauv cov xwm txheej zoo li no, cov kev hloov pauv gene tsuas yog muaj nyob rau hauv qee qhov ntawm lub cev xwb. Tom qab ntawd, cov qog nqaij hlav tsuas yog tsim nyob rau hauv cov qhov ntawd xwb.

Leej twg yuav muaj feem ntau dua los tsim qhov no?

Qhov tseeb, txhua tus neeg txawm hnub nyoog li cas, haiv neeg twg, lossis poj niam txiv neej twg los xij tuaj yeem muaj tus kab mob Brooks-Spiegel syndrome. Txawm li cas los xij, yog tias ib tug ntawm koj niam koj txiv muaj qhov kev hloov pauv ntawm cov noob caj noob ces uas cuam tshuam nrog tus mob no, koj muaj feem yuav muaj tus mob no ntau dua.

Brooke-Spiegler syndrome yog ib qho autosomal dominant disorder. Hais yooj yim xwb, yog tias ib tug ntawm koj niam koj txiv yog tus nqa kab mob (piv txwv li, muaj cov tsos mob), koj muaj 50% feem pua ​​​​​​ntawm kev mob .

Feem ntau cov neeg yuav muaj cov tsos mob thawj zaug thaum lawv tseem hluas, thaum muaj hnub nyoog kaum yim lossis nees nkaum xyoo . Cov pob no pib nce ntxiv thaum lawv muaj hnub nyoog 30 thiab 40 xyoo. Kuj tau pom tias cov poj niam muaj pob ntau dua li cov txiv neej.

Cov kws kho mob kuaj mob li cas?

Yog koj muaj cov tsos mob no, tus kws kho mob yuav xub nug koj txog koj cov tsos mob, koj keeb kwm kev mob nkeeg, thiab koj tsev neeg keeb kwm kev mob nkeeg. Tom qab ntawd lawv yuav kuaj lub cev.

Yog tias tus kws kho mob xav tias muaj Brooke-Spiegler syndrome, nws yuav hais kom ua qhov kev kuaj tawv nqaij , uas yog kev coj ib qho qauv me me ntawm cov hlwb los ntawm lub pob thiab xa mus rau chav kuaj mob kom kuaj hauv qab lub tshuab microscope.

Koj tus kws kho mob kuj tseem yuav qhia kom kuaj cov noob caj noob ces , uas tuaj yeem nrhiav kev hloov pauv hauv cov noob caj noob ces uas ua rau muaj Brooks-Spiegel syndrome.

Puas muaj kev kho kom zoo tag nrho rau qhov no?

Tu siab kawg li, tam sim no tsis muaj tshuaj kho tau tus kab mob Brooke-Spiegel syndrome no. Tiam sis tsis txhob txhawj. Muaj cov kev kho mob zoo uas yuav pab tshem tawm cov qog no thiab tswj kev loj hlob ntawm tus kab mob.

Yog li ntawd, cov kev kho mob rau BSS yog dab tsi?

Koj tus kws kho mob yuav pom zoo kom phais tshem cov pob no. Qhov no suav nrog kev txiav cov pob tawm nrog lub cuab yeej ntse hu ua rab riam phais. Thaum ua li ntawd, cov kws kho mob yuav ceev faj kom tsis txhob ua rau cov tawv nqaij noj qab haus huv ib puag ncig puas tsuaj ntau li ntau tau.

Qee zaum, yog tias thaj chaw loj ntawm daim tawv taub hau yuav tsum tau tshem tawm, koj tus kws kho mob yuavTej zaum yuav pom zoo kom muab daim tawv nqaij sib cais ua ob daim. Qhov no suav nrog kev muab ib txheej tawv nqaij los ntawm lwm qhov ntawm koj lub cev, feem ntau yog los ntawm lub pob tw lossis sab saud ntawm lub duav, los kaw qhov txhab thiab pab kom nws kho.

Ntxiv rau qhov no, muaj ntau txoj hauv kev los kho Bruck-Spiegler syndrome:

• Kev kho dua tshiab ntawm cov tawv nqaij los ntawm cov pa roj carbon dioxide: Qhov no siv cov teeb laser los tshem tawm cov txheej nyias nyias ntawm daim tawv nqaij yam tsis ua rau cov ntaub so ntswg nyob ib puag ncig puas tsuaj.
• Kev kho mob Cryotherapy: Qhov no suav nrog kev siv cua txias heev los khov thiab rhuav tshem cov ntaub so ntswg hauv lub pob. Nws zoo li hlawv nws nrog dej khov.
• Dermabrasion: Qhov no suav nrog kev siv lub cuab yeej tig sai sai los tshem tawm cov txheej sab saud ntawm daim tawv nqaij.
• Electrodessication: Ib qho hluav taws xob siv los ziab cov ntaub so ntswg hauv lub pob.
• Fulguration: Kev siv hluav taws xob los rhuav tshem cov qog.
• Hyfrecation: Lub zog hluav taws xob raug xa mus los ntawm ib rab koob me me, uas hlawv thiab tshem tawm cov pob khaus.
• Kev phais mob Mohs: Hauv txoj kev phais no, tus kws phais yuav tshem tawm cov qog nrog rau me me ntawm cov nqaij noj qab haus huv nyob ib puag ncig nws thiab xa mus kuaj mob.
• Kev kho mob photodynamic: Hauv qhov no, cov tshuaj tshwj xeeb (photosensitizers) raug muab, thiab tom qab ntawd lub teeb raug taw qhia rau ntawm qhov mob qog, ua kom cov tshuaj ua haujlwm thiab rhuav tshem cov qog.

Ntxiv rau cov kev kho mob no, cov kws kho mob kuj tseem yuav muab cov tshuaj uas pab tiv thaiv kev loj hlob ntawm cov qog.

Koj tuaj yeem cia siab li cas thaum nyob nrog tus mob no?

Brooks-Spiegel syndrome yog ib qho mob uas yuav tsum tau kho mus tas li . Txawm li cas los xij, raws li tau hais ua ntej lawm, cov neeg uas muaj tus mob no tuaj yeem ua neej nyob zoo li qub thiab muaj kev noj qab haus huv zoo.

Txawm li cas los xij, muaj ib qho kev pheej hmoo me me. Nruab nrab ntawm 5% thiab 10% ntawm cov neeg uas muaj Bruck-Spiegel syndrome yuav mob qog nqaij hlav . Cov neeg uas muaj tus mob no kuj muaj kev pheej hmoo me ntsis ntawm kev mob qog nqaij hlav lwm yam. Piv txwv li:

• Cov qog nqaij hlav adenocarcinoma
• Basal cell carcinoma (tseem yog mob qog nqaij hlav ntawm daim tawv nqaij)
• Mob qog nqaij hlav ntawm cov qog salivary, piv txwv li adenoid cystic carcinoma
• Squamous cell carcinoma (tseem yog mob qog nqaij hlav ntawm daim tawv nqaij)

Yav tom ntej yuav muaj dab tsi? (Outlook)

Feem ntau, cov qog no tuaj yeem rov tshwm sim dua . Yog tias qhov no tshwm sim, yuav tsum tau phais ntxiv lossis lwm yam kev kho mob kom tshem tawm cov qog tshiab.

Puas muaj ib txoj kev los txo qhov teeb meem no?

Tu siab kawg li, tsis muaj ib txoj kev tshwj xeeb los tiv thaiv lossis txo koj txoj kev pheej hmoo ntawm kev mob Brook-Spiegel syndrome. Txawm li cas los xij, raws li tau hais ua ntej lawm, kev kho mob tuaj yeem txo cov qog uas koj muaj thiab lawv qhov loj.

Koj yuav saib xyuas koj tus kheej li cas ua ib tus neeg uas muaj Brooke-Spiegler Syndrome?

Thaum nyob nrog tus mob no, muaj qee yam tseem ceeb uas koj tuaj yeem ua tau los saib xyuas koj tus kheej:

• Mus ntsib kws kho mob tawv nqaij tsis tu ncua kom kuaj xyuas daim tawv nqaij. Qhov no yuav tsum tau ua tsawg kawg ib xyoos ib zaug. Ib txhia neeg yuav tsum tau mus ntsib kws kho mob txhua peb txog plaub lub hlis yog tias lawv muaj pob khaus ntau zaus.
• Ua ib qho kev kuaj xyuas daim tawv nqaij tsawg kawg ib hlis ib zaug. Nrhiav seb puas muaj pob tshiab lossis kev hloov pauv ntawm cov pob uas twb muaj lawm.
• Nco ntsoov siv tshuaj pleev thaiv hnub thaum tawm mus rau hauv lub hnub. Qhov no yuav pab txo qhov kev puas tsuaj ntawm lub hnub rau koj daim tawv nqaij thiab txo qhov kev pheej hmoo ntawm kev mob qog nqaij hlav ntawm daim tawv nqaij.

Lub sijhawm twg zoo tshaj plaws mus ntsib kws kho mob?

Yog koj pom ib qho ntawm cov kev hloov pauv no ntawm koj cov tawv nqaij, koj yuav tsum mus ntsib kws kho mob tam sim ntawd :

• Yog tias cov qog ua rau mob .
• Yog tias cov qog ntshav ntws los .
• Yog tias cov xim ntawm cov lumps hloov pauv .
• Yog tias cov qog loj zuj zus sai sai .
• Yog tias lub pob zoo li lub qhov txhab ('ulcerated') .

Tsis tas li ntawd, yog tias koj tab tom yuav muaj menyuam, uas yog, yog tias koj npaj yuav cev xeeb tub, nws yuav tseem ceeb heev rau koj tham nrog kws kho mob thiab tau txais kev qhia txog noob caj noob ces .

Qhov txawv ntawm Brooke-Spiegler Syndrome thiab Neurofibromatosis yog dab tsi?

Ob yam kab mob no yog cov mob caj ces. Ob yam no feem ntau ua rau muaj qog nqaij hlav (tsis yog qog nqaij hlav). Txawm li cas los xij, muaj qhov sib txawv me ntsis.

• Hauv Brooke-Spiegler syndrome (BSS), cov qog feem ntau tshwm sim ntawm daim tawv nqaij .
• Hauv neurofibromatosis, cov qog tuaj yeem tsim ntawm daim tawv nqaij, hauv qab daim tawv nqaij, thiab raws koj cov hlab ntsha .

Muaj peb hom neurofibromatosis tseem ceeb. Cia peb kawm me ntsis txog lawv:

• Neurofibromatosis hom 1 (NF1): Hauv qhov no, cov qog (neurofibromas) tshwm sim rau ntawm lossis hauv qab daim tawv nqaij.
• Neurofibromatosis hom 2 (NF2): Hauv hom neurofibromatosis no, cov qog nqaij hlav (schwannomas) tshwm sim hauv cov hlab ntsha ntawm lub hlwb thiab cov hlab ntsha qaum (peripheral nervous system). Feem ntau, cov hlab ntsha ntawm lub pob ntseg raug cuam tshuam.
• Kab mob Schwannomatosis:Qhov no kuj cuam tshuam txog cov qog hu ua schwannomas, tab sis lawv nyob hauv lub paj hlwb peripheral. Lawv feem ntau tsis cuam tshuam rau cov hlab ntsha uas koom nrog kev hnov ​​​​lus.

Yog li, thaum ob qho kab mob no yog cov mob caj ces uas ua rau muaj pob, muaj qhov sib txawv me ntsis hauv thaj chaw uas lawv cuam tshuam thiab cov hom pob.

Cov Lus Qhia Txog Kev Coj Mus Tsev

Zoo, yog li kuv vam tias koj tam sim no nkag siab zoo dua txog Brooke-Spiegler Syndrome uas peb tau tham txog hnub no.

Nco ntsoov, yog tias koj pom muaj pob tshiab ntawm koj daim tawv nqaij, tshwj xeeb tshaj yog ntawm koj lub ntsej muag, caj dab, lossis taub hau, lossis yog tias koj pom muaj kev hloov pauv ntawm qhov uas twb muaj lawm, tsis txhob tsis quav ntsej. Nws yog qhov zoo tshaj plaws mus ntsib kws kho mob thiab coj nws mus kuaj mob.

Txawm hais tias Brooke-Spiegel syndrome yog ib qho mob tsis tshua muaj, nws yog ib qho tseem ceeb uas yuav tsum paub txog nws. Txawm hais tias cov qog no feem ntau tsis muaj teeb meem, lawv tsis tshua muaj peev xwm ua rau mob qog noj ntshav, yog li nws yog ib qho tseem ceeb uas yuav tsum nrhiav kev kho mob thiab kev kho mob kom zoo . Yog tias kuaj pom thaum ntxov thiab tswj hwm kom zoo, koj kuj tuaj yeem ua neej nyob zoo li qub. Tsis muaj dab tsi yuav tsum txhawj txog, txhua yam tuaj yeem daws tau los ntawm kev tham nrog koj tus kws kho mob.

Brooke -Spiegler syndrome, cov qog ntawm daim tawv nqaij, cov kab mob caj ces, cylindroma, spiradenom, trichoepithelioma, CYLD gene