I taksaa brown spot chu i hnawih hian a sen a, a itchy em? Hei hi Urticaria Pigmentosa a ni thei!

I fa emaw, nangmah taksaah emaw, a sen hring hring (red-brown spot) i hmu ngai em? I kut zungtang hmangin zawi zawiin i fawp mai mai pawh a ni mai thei, rang takin chu hmun chu a sen a, a hring tan a, a chungah chuan blister te tak te a lo lang ta a ni. He thil tawn hi i hriat chian a nih chuan tunlaia kan sawi tur dinhmun hi hriat chian a pawimawh hle.

A awlsam zawngin, Urticaria Pigmentosa chu eng nge ni?

Urticaria Pigmentosa hi vunah hives chi khat a ni a, vunah a awm thin. Naupang tê tê leh puitling tleirawlah hmuh tam ber a ni. A nihna takah chuan `mastocytosis' an tih dinhmun chi khat a ni.

Tunah chuan `mastocytosis` tih hi eng nge ni tih i ngaihtuah mai thei. A awlsam khawp mai. ``mast cells`` hi kan taksa immune system-a cell chi khat bik a ni. Ruang venhimtu ang mai an ni. Eng emaw chhan vanga heng mast cells te hi vun hmun khatah a tam lutuk a awm chuan Urticaria Pigmentosa kan ti thin. Mahse, heng mast cells te hi vunah chauh ni lovin, a chang chuan taksa peng dang ruh leh intestines-ah te an pung khawm thei a ni. Tichuan ``systemic mastocytosis`` tih dinhmunah a chang ta a ni. Mahse lungngai suh, a tam zawkah chuan naupang Urticaria Pigmentosa hi vunah chauh a ni.

Engvangin nge hetiang thil hi a lo thlen?

Hei hi a chhan ber chu a hmaa kan sawi tawh ``mast cells`` te hi a ni. Hengte hi thisen var chi khat, kan ruhro (bone marrow) atanga lo chhuak a ni. Heng cell te hi kan taksa zawng zawngah a awm a, a bik takin pawn lam khawvel, vun, lung leh intestines te nena kan inzawmna hmunah te a awm a. Hriselna ang chi thil taksa chhunga a luh chuan heng mast cells te hian taksa venhimna atana pui turin chemical ``histamine`` an tichhuak thin.

Urticaria Pigmentosa hi genetic mutation avanga lo awm a ni a, chu chuan heng mast cells te hi vunah a tam lutuk a tipung a ni. He genetic change hi nausen pum chhunga a la awm laiin a thleng tlangpui. Mahse, nu leh pate aṭanga fate rochun thil a ni lo . He mutation awm chhan dik tak hi hmuhchhuah a la ni lo.

Puitlingten he natna hi an neih chuan a na deuh thei bawk. Chu chu `indolent systemic mastocytosis` tih a ni. Chutiang a nih chuan vun bakah hian ruhro (bone marrow) ang hmunah te mast cells te hi an pung khawm thin. A tlem hle a, he natna hi cancer natna `mast cell leukemia` an tihah a lo chang thei a ni. Mahse, hetah pawh hian hengte hi thil awm lo tak tak a ni . Naupangte zinga Urticaria Pigmentosa awm zinga 99% chu chutiang chuan a na lo.

He natna lan chhuah dan engte nge ni?

A chhinchhiahna ber leh langsar ber chu kan sawi tawh `Darier chhinchhiahna` hi a ni. Chu chu, a sen hring (brown spots) chu a sen a, a hmin chuan a hmin tihna a ni. Tin, mi thenkhat chuan a hnuaia symptoms te hi an nei ve thei bawk.

Urticaria Pigmentosa vei zinga 75% aia tam chu naupang kum 10 hnuai lam an ni a, naupang tam zawkah chuan he natna hi vunah chauh a awm a, kum upat deuh deuhin a tha chho zel a ni.

Engtin nge doctor hian hei hi a hriat chian?

A tam zawkah chuan i doctor chuan i vunah hmun hrang hrang enin Darier chhinchhiahna a enfiah a, awlsam takin he natna hi a hre thei a ni. Test dang neih a ngai lo.

Mahse, he natna hi vunah chauh ni lovin taksa peng dang pawh a nghawng em tih rinhlelhna a awm chuan doctor chuan a hnuaia test te hi a rawt thei ang.

• Thisen test (`Blood count`): Thisen chhunga cell chi hrang hrang leh a zat te zir rawh.
• Serum tryptase test: Thisenah hian mast cells atanga chhuak enzyme a awm leh awm loh a enfiah thin.
• Bone density scan: I ruh a chak zia en rawh.
• Bone marrow test: Bone marrow hian cell hrisel a siam chhuak em tih a enfiah thin.

Enkawl dan tur leh natna thunun dan tur

Thu lawmawm tak chu naupang tam zawk tan chuan Urticaria Pigmentosa hi an lo upat deuh deuh chuan a mah chauhin a reh ang . Spot thar lo awm tur venna tur damdawi bik a awm lo. Spot awmsa te chu a bo theih nan kum tam tak a ngai thei.

He natna hi vunah chauh a nih chhung chuan enkawlna lian tham a ngai lo tlangpui. I doctor chuan i natna lan chhuah dan tur enkawl nan a hnuaia mi hi a rawn rawt thei ang:

• Oral antihistamines: Hriselna leh vun natna control.
• Corticosteroid cream: Itchy hmunah hnawih thin ang che.
• Cromolyn sodium: Damdawi a ni a, natna lan chhuah dan, luak chhuak leh riltam te enkawlna atana pek thin a ni.
• EpiPen: Allergic reaction na tak (anaphylactic reaction) nei te tan emergency-a hman tur hmanrua a ni. Hei hi mi zawng zawngin kan mamawh vek lo.

A bik takin pumpelh tur thil

He natnaah hian a pawimawh ber chu a natna lan chhuahna tizualtu triggers, chu chu a hmuna a hring chhuak leh a sen tirtu thilte atanga inthiarfihlim hi a ni.

Pawimawh: Nangmah emaw i fa emaw hian natna dang avanga damdawi ei a ngai a nih chuan Urticaria Pigmentosa chungchang hi i doctor hnenah hriattir ngei ang che .

Complication a awm thei em?

A tlem hle a, he natna hi `systemic mastocytosis`-ah a kal thei. Chutiang mite chuan allergy na tak `anaphylactic shock` an tih chu an nei nasa hle a, chu chuan nunna a tichhe thei a ni. Thâwk harsa leh hriatna hloh ang chi symptoms i neih chuan damdawi in emergency department (ETU) ah i kal nghal tur a ni .

Systemic mastocytosis avanga harsatna dangte chu:

• Osteoporosis: Ruh chak lo.
• Cytopenia: Taksa chhunga thisen cell siam chhuah tlem.
• Liver lama harsatna a awm.
• Cancer (a tlem hle).

Hetah pawh hian hengte hi harsatna awm lo tak tak an ni. Naupang Urticaria Pigmentosa vei zingah an tlem hle.