Ichthyosis congenita biliary atresia


Type of disease: Rare conditions

Ichthyosis congenita biliary atresia is one form of ichthyosis, a rare genetic skin disorder, in which dead skin cells accumulate in thick, dry scales on your skin’s surface from birth accompanied by biliary atresia. Bile is a fluid produced by the liver to help digest food. Sometimes the tubes that are supposed to carry the bile from the liver to the gut do not develop appropriately before birth – this is called biliary atresia. This leads to bile backing up in the liver and other problems, such as jaundice or yellowing of the skin.

Exposure to sunlight may improve or worsen the condition. In some cases, excess dead skin sheds much better from wet tanned skin after bathing or a swim, although the dry skin might be preferable to the damaging effects of sun exposure. There is no cure for ichthyosis. The main goal of treatment is to moisturize and exfoliate. This helps prevent dryness, scaling, cracking and build-up of skin.

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