I vunah hian hetiang hian bumps i nei em? Brooke-Spiegler Syndrome chungchang hi i zir dawn teh ang.

I vunah, a bîk takin i hmai, i kawr, a nih loh leh i lu vunah hliam tenau te, i tibuaitu i hmu ngai em? A châng chuan eng nge an nih leh engvângin nge an lo awm tih i hre lo mai thei. Vawiin hian vun natna pakhat, chutiang bumps thlen thei chu kan sawi dawn a, mahse a tlem deuh a, chu chu mi zawng zawngah a thleng lo tihna a ni. Hei hi Brooke-Spiegler Syndrome an ti a. Ngaihtuah buai suh, he thu hi awlsam takin sawi ila.

Brooke-Spiegler Syndrome hi eng nge ni?

A awlsam zawngin, Brooke-Spiegler Syndrome hi vun natna hmuh tur awm lo tak a ni . I vunah, a bik takin i hmai, i kawng leh lu vunah lump (tumor an tih bawk) a siam thin. A chang chuan heng lumps te hi i taksa peng dang ah pawh a lo awm thei bawk. A tam zawkah chuan i naupan lai, kum sawm leh pariat emaw kum sawmhnih emaw vel atang khan an rawn lang tan thin .

Heng lumps chungchang sawi dawn chuan a tam zawkah chuan cancer an ni lo (chu chu `benign` an ni tihna a ni), chu chu benign an ni tihna a ni. Mahse, kan hriat reng tur chu, a tlem berah, hun kal zelah, cancer an ni thei (chu chu `malignant` an ni tihna a ni). A châng chuan heng benign lumps te hi an lo lian chuan hliam an tuar thei a, `open wounds` an lo ni thei a, natna kai pawh an thlen thei bawk.

He natna hi i nu leh pate hnen atanga lo piang a ni tlangpui a, chu chu genetic condition a ni tihna a ni . Mahse, a tlem hle a, chhungkaw zingah tumahin he natna hi nei lo mah se, mi pakhatin Brooks-Spiegel syndrome a vei thei a ni.

A tha ber chu, hei hi enkawl dan tur a awm. A tam zawkah chuan surgery hi tih theih awmchhun a ni. Enkawlna dik tak an neih chuan he natna vei tam zawk hi chu nun pangngai, hlim tak leh active takin an nung thei a ni.

Hei hi hming dang a awm em?

Ni e, doctor-te chuan he Brooke-Spiegler syndrome hi hming dang engemaw zat an hmang a. Heng hmingte hi i hre mai thei bawk:

• Ancell-Spiegler-a cylindromas te chu a awm a
• CYLD vun natna (CCS) .
• Chhungkaw cylindromatosis (FC) a awm thei.
• Chhungkaw tam tak trichoepitheliomas (MFT) .

Heng hmingte hi a buaithlak deuhin lang mahse, an vai hian dinhmun inang hming an ni.

Hetiang dinhmun hi engtiang chiahin nge a awm?

Hei hi a takah chuan thil awm lo tak a ni . Zirchiangtute chuan mi maktaduai khat zinga pakhat velin Brooks-Spiegel syndrome an vei niin an sawi. Chuvangin, thil awmze nei tak a ni lo.

Eng nge a lan chhuah dan? Hengte pawh hi i nei em tih en rawh...

Hetiang natna lan chhuahna ber chu vun natna ( skin bumps ) a lo awm hi a ni . Chûngte chu i hmai, i kawr leh i lu vun aṭangin a inṭan tlangpui. Heng bumps te hi a tlangpuiin a pianzia chu a zungpui a, a lian zawng chu centimetre 0.5 atanga 3 (cm) inkar a ni thei.

Hun a kal zel a, heng lumps te hi a tam leh a lianin a pung thei a ni. A châng chuan an hmel a tichhe thei bawk. Vawi tam tak chu, heng lumps te hi a na hle . Tin, mipat hmeichhiatna hmun vunah an lo awm chuan mipat hmeichhiatna lama harsatna an thlen thei bawk. Thil dang chu a chang chuan heng zungte hi lu zawng zawngah a darh thei a , sam a ti tla thei bawk . Heng thilte hian lungngaihna a thlen thei bawk.

Heng bumps bakah hian a chang chuan i vunah thil te te, var, blister ang chi (milia) i hmu thei bawk. Heng ‘milia’ te hi a pawi lo hle a, mahse mi thenkhat tan chuan a buaithlak thei hle.

Heng tumor chi hrang hrangte hi engte nge ni? (BSS-a vun natna chi hrang hrang)

Brooke-Spiegler syndrome-ah hian lumps chi thum hmuh tur a awm a, chungte chu:

1. Cylindromas: Hei hi a tlangpuiin a hring, pink lumps a ni. Lu vunah an lo thang lian ber a, chutah chuan sam zung (hair follicles) a awm a ni. Hmai, beng leh lungah te pawh an lo thang lian thei bawk. Millimeter tlemte atanga centimetre engemaw zat thlengin an lian thei. Lu vunah an lo thanglian chuan an inzawm khawm thei a, jigsaw puzzle chi hrang hrang ang maiin an lang thei bawk.

2. Spiradenomas: Hei hi lump nghet, lumpy tak a ni. Lu vun, kawr leh taksa chunglam ah te an awm tam ber. An lian zawng hi centimetre khat aia tlem atanga centimetre engemaw zat thleng a ni thei. Anni hi a sen emaw, vun rawng emaw an ni fo. Mahse, grey, pink, purple, red, yellow emaw pawh an ni thei. Spiradenomas hi a chang chuan a na thei hle.

3. Trichoepitheliomas: Hengte hi lump khauh tak, eng mawi tak tak an ni bawk. Hmaiah, sam awmna hmunah te an awm tam ber. A tlangpuiin centimetre khat aia te zawk an ni. Blue, brown, vun rawng, pink, yellow te pawh an ni thei.

A pawimawh ber chu a chang chuan he Brook-Spiegler syndrome-ah hian heng tumor chi hnih emaw pathum emaw ziarang hi tumor pakhatah hian hmuh theih a ni.

Hetiang dinhmun hian taksa peng dang a nghawng em?

Ni e, a chang chuan heng lumps te hi taksa peng dangah a lo awm thei bawk. Chutiang hunah chuan chu taksa peng hrang hrangte hnathawh dan chu a nghawng thei a ni. Entir nan:

• If it forms in the airways , thawk harsatna a thlen thei.
• Bengah a lo awm a nih chuan , hriatna a nghawng thei.
• Mit-ah a awm chuan , mit hmuhna a nghawng thei.
• If they form on the genitals , mipat hmeichhiatna hman dan a nghawng thei.
• Kawng leh hmuiah a lo awm a nih chuan , chewing leh swallowing a nghawng thei.
• HnarA lo awm chuan rim hriatna a nghawng thei a ni.

Engvangin nge Brooke-Spiegler Syndrome hi a lo awm?

Hei hi a chhan ber chu kan taksaa `CYLD` gene-a genetic changes (`mutations`) vang a ni. Hetiang dinhmun i neih theih nan hian i nu leh pa zinga pakhat tal chu he genetic mutation kengtu a ni tur a ni. Chumi awmzia chu thlah khat atanga thlah dangah a kal zel tihna a ni .

Mahse, a tlem hle a, chhungkaw history nei lo mi chuan an taksaah he gene mutation (`de novo mutation`) hi a nei thei a ni. Chutiang hunah chuan gene mutation hi taksa hmun thenkhatah chauh a awm thei. Tichuan, chutiang hmunah chauh chuan tumor a lo awm thei a ni.

Hetiang hi tuin nge nei tam zawk?

Dik tak chuan, kum eng pawh ni se, eng chi pawh ni se, eng mipa pawh ni se, Brooks-Spiegel syndrome an nei thei a ni. Mahse, i nu leh pa zinga pakhatin chu natna nena inzawm gene mutation a neih chuan i vei theihna a sang zawk a ni.

Brooke-Spiegler syndrome hi autosomal dominant disorder a ni a, a natna hi a ni. A awlsam zawngin, i nu leh pa zinga pakhat chu carrier (i.e., symptoms nei) a nih chuan, he natna hi i vei theihna chance 50% i nei a ni .

Mi tam zawk chuan an naupan lai, kum sawm leh pariat emaw kum sawmhnih emaw vel an nih laiin symptoms an nei hmasa ber thin . Heng lumps te hi kum 30 leh 40 vel an nih chuan an pung tan thin. Hmeichhia hian mipa aiin lumps an nei tam zawk tih hmuhchhuah a ni bawk.

Engtin nge doctor-te hian hei hi an hriat chian?

Heng symptoms te hi i neih chuan doctor in i symptoms te, i medical history te, leh i chhungte medical history te a zawt hmasa ang che. Tichuan physical exam an ti leh ang.

Doctor chuan Brooke-Spiegler syndrome a rinhlelh chuan, skin biopsy , chu chu lump aṭanga cell sample tlemte lak a, microscope hmanga enfiah turin lab-a thawn a ni.

I doctor chuan genetic testing , Brooks-Spiegel syndrome thlentu genes inthlak danglamna a zawng thei bawk.

Hei hi damdawi famkim a awm em?

Vanduaithlak takin tunah hian Brooke-Spiegel syndrome hi damdawi a la awm lo . Mahse, lungngai suh. Heng lumps te hi paih bo tur leh natna kal zel dan tur control turin enkawlna tha tak tak a awm a.

Chuti a nih chuan BSS enkawlna tur engte nge ni?

I doctor chuan heng lumps te hi paih chhuah nan surgical excision a rawt mai thei. Hei hian scalpel an tih hmanraw hriam tak hmanga lumps te chu cut chhuah a ngai a ni. Chutianga an tih hian doctor-te chuan a chhehvêla vun hrisel chu a theih ang anga tihchhiat loh nan an fimkhur hle.

A châng chuan lu vun hmun zau tak chu lak chhuah a ngai a nih chuan i doctor pawhin a ti thei bawkSplit-thickness skin graft hman a tha thei bawk. Hei hian i taksa peng dang atanga vun layer pakhat lak a huam a, a tam zawk chu i hnute emaw, a kekawr chunglam emaw atanga lak a ni a, chu chuan hliam chu khar a, a dam theih nan a pui a ni.

Hei bakah hian Bruck-Spiegler syndrome enkawl dan dang engemaw zat a awm a:

• Carbon dioxide laser resurfacing: Hei hian laser beam hmangin vun layer te tak te te chu a chhehvel tissue tichhe lovin a paih chhuak thin.
• Cryotherapy: Hei hian lum lutuk hnawih a, lump chhunga tissue awm chu freeze leh tihchhiat a ni. Ice hmanga hal ang mai a ni.
• Dermabrasion: Hei hi vun chunglam layer lakchhuah nan hmanraw inher chak tak hman a ni.
• Electrodessication: Electric current hmangin lump chhunga tissue awm chu a vawt tir thin.
• Fulguration: Electric shock hmanga tumor tihchhiat a ni.
• Hyfrecation: Electric pulse chu needle te tak te hmangin a thawn a, chu chuan a kang a, a hnah chu a paih chhuak thin.
• Mohs surgery: Hetiang tih danah hian surgeon chuan a chhehvela tissue hrisel tlemte nen tumor chu a la chhuak a, enfiah turin a thawn thin.
• Photodynamic therapy: Hetah hian damdawi bik (photosensitizers) pek a ni a, chutah chuan êng chu tumor lam hawiin, damdawi te chu a tichak a, tumor te chu a tichhia a ni.

Heng enkawlna bakah hian doctor-te chuan tumor lian zel tur titawp thei damdawi pawh an rawt thei bawk.

Hetiang dinhmun nen hian eng nge i beisei theih ang?

Brooks-Spiegel syndrome hi dam chhung zawnga hriselna lama harsatna awm thei a ni . Mahse, a hmaa kan sawi tawh ang khan he natna vei te hian nun pangngai, famkim an hmang thei a ni.

Mahse, risk tlemte a awm. Bruck-Spiegler syndrome vei zinga 5% leh 10% inkar chuan malignant tumor an nei thin . Hetiang natna vei te hian cancer chi dang vei theihna an nei tlem hle bawk. Entir nan:

• Adenocarcinoma cancer natna a ni
• Basal cell carcinoma (vun cancer a ni bawk) .
• Salivary gland cancer, entirnan adenoid cystic carcinoma te pawh a awm thei
• Squamous cell carcinoma (vun cancer a ni bawk) .

Nakin hun chu eng nge ni ang? (Hmuh dan tur) .

Vawi tam tak chu, heng lumps te hi a lo awm leh thei . Chutiang a nih chuan, a hnah tharte chu paih chhuah nan operation tam zawk emaw, enkawlna dang emaw tih a ngai mai thei.

Hetiang thil thleng hi tihtlem dan tur a awm em?

Vanduaithlak takin Brook-Spiegel syndrome i vei theihna tur venna emaw tihtlem dan tur chiang tak a awm lo . Mahse, a hmaa kan sawi tawh ang khan enkawlna hian lump i neih zat leh a lian zawng a tihtlem thei a ni.

Brooke-Spiegler Syndrome vei angin engtin nge i in enkawl?

Hetiang dinhmuna i nunpui hian i in enkawl nan thil pawimawh tak tak i tih theih a awm a:

• Skin check turin dermatologist hmu fo ang che. Hei hi kum khatah vawi khat tal tih tur a ni. Mi thenkhat chuan breakout an neih fo chuan thla thum atanga thla li danah doctor an pan a ngai mai thei.
• Thla khatah vawi khat tal vun self-exam nei thin ang che. Bump thar emaw, a awm tawha inthlak danglamna emaw zawng rawh.
• Ni chhuahna hmuna chhuah dawnin sunscreen hmang ngei ang che. Hei hian ni zungin i vun a tihchhiat a ti tlem ang a, vun cancer vei theihna a ti tlem bawk ang.

Doctor hmuh hun tha ber berte chu engte nge ni?

I vun vun natnaah hian heng inthlak danglamna te hi i hmuh chuan doctor i pan nghal tur a ni :

• Lumps chu a na a nih chuan .
• Lumps chu thisen chhuak a nih chuan .
• Lumps rawng a inthlak chuan .
• Lumps te chu a lian nghal vat a nih chuan .
• Lump chu ulcer (`ulcerated`) ang maia a lan chuan .

Tin, fa i beisei a nih chuan, chu chu naupai i tum a nih chuan, doctor biak a, genetic counseling lak a pawimawh hle mai thei bawk .

Brooke-Spiegler Syndrome leh Neurofibromatosis hi eng nge an danglamna?

Heng natna pahnihte hi genetic condition a ni. An pahnih hian benign (non-cancerous) tumor an thlen ber a ni. Mahse, a danglamna tlem a awm.

• Brooke-Spiegler syndrome (BSS) -ah chuan vunah hian lumps a awm ber .
• Neurofibromatosis -ah chuan vun-ah te, vun hnuaiah te, leh i nerve-ah te lumps a lo awm thei a ni .

Neurofibromatosis hi chi thum a awm a. An chanchin tlem han zir ila:

• Neurofibromatosis type 1 (NF1): Hetiang dinhmunah hian vun chungah emaw, vun hnuaiah emaw lumps (neurofibromas) a lo awm thin.
• Neurofibromatosis type 2 (NF2): Hetiang neurofibromatosis chi khatah hian thluak nerve leh spinal cord (peripheral nervous system)-ah tumor (schwannomas) a lo awm a. A tam zawkah chuan auditory nerve te hi a nghawng thin.
• Schwannomatosis natna (Schwannomatosis) a ni.Hei hian schwannomas an tih tumor pawh a huam tel a, mahse peripheral nervous system-ah a awm thung. A tlangpuiin hriatna lama inrawlh nerve te chu an nghawng lo.

Chuvangin, heng natna pahnih hi genetic condition, lumps siamtu an nih laiin, an natna hmun leh lumps chi hrang hrangah hian danglamna tlem a awm.

Take-Home Thuchah a ni

Okay, chuvangin tunah chuan vawiin a kan sawi tawh Brooke-Spiegler Syndrome hi in hriatthiam ka beisei.

I vunah, a bik takin i hmai, i kawng emaw, i lu vunah emaw, vun thar i hmuh chuan emaw, a awm tawha thil danglam i hmuh chuan ngaihthah suh tih hre reng ang che. Doctor hmu a, enfiah tir a tha ber.

Brooke-Spiegel syndrome hi natna awm lo tak ni mahse, hriat a pawimawh hle. Heng lumps te hi a tha lo fo nachungin, cancer a ni thei lo va, chuvangin damdawi lam thurawn leh enkawlna dik tak lak a pawimawh hle . A hmaa hmuhchhuah a nih a, uluk taka enkawl a nih chuan nang pawh nun pangngai, hrisel takin i nung thei a ni. Thil ngaihtuah tur a awm lo, eng thil pawh hi doctor nena inbiain a chinfel theih a ni.

` Brooke-Spiegler syndrome, vun nodules, genetic natna, cylindroma, spiradenom, trichoepithelioma, CYLD gene