I-Lamellar ichthyosis

ILamellar ichthyosis (LI) yisimo esingajwayelekile sesikhumba. Kubonakala lapho kuzalwa futhi kuyaqhubeka impilo yonke.

I-LI isifo se-autosomal recessive. Lokhu kusho ukuthi umama nobaba kumele bobabili badlulisele ikhophi eyodwa engafanele yesakhi sofuzo enganeni yabo ukuze ingane ithole lesi sifo.

Izinsana eziningi ezine-LI zizalwa zinolwelwesi olucacile, olucwebezelayo nolunamafutha esikhumba okuthiwa ulwelwesi lwe-collodion. Ngalesi sizathu, lezi zingane zaziwa njengezingane ze-collodion. I-membrane sheds ngaphakathi kwamasonto amabili okuqala okuphila. Isikhumba esingaphansi kolwelwesi sibomvu futhi sine-scaly esifana nobuso benhlanzi.

Nge-LI, ungqimba olungaphandle lwesikhumba olubizwa ngokuthi i-epidermis alukwazi ukuvikela umzimba njenge-epidermis enempilo. Ngenxa yalokho, ingane ene-LI ingaba nezinkinga zempilo ezilandelayo:

• Kunzima ukondla
• Ukulahlekelwa uketshezi (ukoma)
• Ukulahlekelwa ibhalansi yamaminerali emzimbeni (ukungalingani kwe-electrolyte)
• Izinkinga zokuphefumula
• Izinga lokushisa lomzimba elingazinzile
• Izifo zesikhumba noma zomzimba wonke

Izingane ezindala nabantu abadala abane-LI bangaba nalezi zimpawu:

• Izikali ezinkulu ezimboza ingxenye enkulu yomzimba
• Ukwehla kwekhono lokujuluka, kubangele ukuzwela ekushiseni
• Ukulahleka kwezinwele
• Umunwe ongajwayelekile nezinzwani
• Isikhumba sezintende namathesi sikhuluphele

Izingane zeCollodion imvamisa zidinga ukuhlala egunjini lokunakekela izingane ezisanda kuzalwa (NICU). Zibekwa endaweni yokufudumeza enomswakama omkhulu. Bazodinga ukuphakelwa okwengeziwe. Ama-moisturizer kudingeka ukuthi asetshenziswe esikhunjeni. Ngemuva kokuchitheka kolwelwesi lwe-collodion, izingane zivame ukuya ekhaya.

Ukunakekelwa kwesikhumba impilo yonke kubandakanya ukugcina isikhumba sinomswakama ukunciphisa ubukhulu bezikali. Izinyathelo zifaka:

• Amafutha afakwa esikhunjeni
• Imithi ebizwa ngama-retinoids athathwa ngomlomo ezimweni ezinzima
• Indawo enomswakama ophezulu
• Ukugeza ukukhulula izikali

Izingane zisengozini yokutheleleka lapho zichitha ulwelwesi lwe-collodion.

Izinkinga zamehlo zingenzeka kamuva empilweni ngoba amehlo awakwazi ukuvala ngokuphelele.

LI; Ingane yeCollodion - lamellar ichthyosis; Ichthyosis azalwe naso; I-Autosomal recessive congenital ichthyosis - uhlobo lwe-lamellar ichthyosis

• Ichthyosis, etholwe - imilenze

UMartin KL. Ukuphazamiseka kwe-keratinization. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS. I-Tasker RC, i-Wilson KM, ama-eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 677.

IPatterson JW. Ukuphazamiseka kokuvuthwa kwe-epidermal kanye ne-keratinization. Ku: Patterson JW, ed. I-Weedon's Skin Pathology. Umhlaka 5. IPhiladelphia, PA: Elsevier; 2021: isahluko 10.

URichard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, kanye nokuphazamiseka okuhlobene. Ku: Bolognia JL, Schaffer JV, Cerroni L, ama-eds. Isikhumba. Umhla wesi-4. IPhiladelphia, PA: Elsevier; 2018: isahluko 57.