Lamellar ichthyosis (LI) yog ib yam tsis tshua muaj kab mob ntawm daim tawv nqaij uas tshwm sim thaum yug . Nws yog ib qho ntawm peb kab mob ntawm daim tawv nqaij hu ua autosomal recessive congenital ichthyoses (ARCI). Lwm ob lub npe hu ua harlequin ichthyosis harlequin ichthyosis Ichthyosis congenita (collodion baby; congenital ichthyosiform erythroderma; xeroderma; desquamation of the newborn) yog ib yam kab mob ntawm daim tawv nqaij. Nws yog tus cwj pwm los ntawm kev nthuav dav, txawv txav liab, qhuav, thiab tawv nqaij nrog cov ntxhib loj thiab nplua dawb. Kev khaus (pruritus) feem ntau kuj tshwm sim. https://rarediseases.org › rare-kab mob › ichthyosis
Ichthyosis - NORD (National Organization for Rare Disorders)
thiab congenital ichthyosiform erythroderma.
Yam hloov pauv dab tsi ua rau lamellar ichthyosis?
Kev hloov pauv hauv TGM1 noob yog lub luag haujlwm rau kwv yees li 90 feem pua ntawm cov neeg mob lamellar ichthyosis. TGM1 noob muab cov lus qhia ua ib qho enzyme hu ua transglutaminase 1.
Lamellar ichthyosis tau txais txiaj ntsig li cas?
Txawm hais tias tus mob yuav tshwm sim los ntawm kev hloov pauv (kev hloov pauv) hauv ib qho ntawm ntau cov noob sib txawv, kwv yees li 90% ntawm cov neeg mob tshwm sim los ntawm kev hloov pauv hauv TGM1 noob. Lamellar ichthyosis feem ntau tshwm sim nyob rau hauv ib autosomal recessive yam. Kev kho mob yog ua raws li cov tsos mob thiab cov tsos mob tshwm sim hauv txhua tus neeg.
YYog ichthyosis yog caj ceskev nyuaj siab?
Ichthyosis vulgaris yog feem ntau tshwm sim los ntawm kev hloov caj ces uas tau los ntawm ib tug lossis ob leeg niam txiv. Cov menyuam yaus uas tau txais cov noob tsis zoo los ntawm ib tus niam txiv tsuas muaj tus kab mob me me. Cov neeg uas tau txais ob lub noob caj noob ces muaj qhov hnyav dua ntawm ichthyosis vulgaris.
Puas lamellar ichthyosis tuag?
Txawm hais tias feem ntau cov menyuam mos uas muaj autosomal recessive congenital ichthyosis (ARCI) yog cov menyuam mos liab, qhov kev nthuav qhia thiab qhov hnyav ntawm ARCI yuav txawv heev, xws li harlequin ichthyosis, feem ntau mob hnyav thiab feem ntau tuag daim ntawv, rau lamellar ichthyosis (LI) thiab (nonbullous) congenital ichthyosiform …