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Over Netherton, Ichthyosis en de stichting

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Congenital Ichthyosiform Erythroderma (CIE)

Congenital means the condition is present at birth and caused by a genetic abnormality. In Congenital ichthyosiform erythroderma a variety of genes can play a role. The abnormalities in the genes affect the skin. One out of four children from a couple both carrying a copy of the faulty gene inherits two defect genes and is affected. Both parents can have normal skin. The abnormality is autosomal recessive and the risk of any further child being affected is 25%. Ichthyosiform refers to a skin that appears to have the pattern of ‘fish-scales’. The skin is dry and scaly. The erythrodermic skin is red. The condition is shortened as CIE.

A child born with CIE often has a mild form of the collodion membrane. This is a tight shiny film around the body of the newborn. After the membrane dries and sheds the skin will be visible. The entire skin is red and has tiny white scales.

The severity varies from case to case. There can be a mild redness and mild scaling of the skin as well as a severe red and scaly skin. The condition can imply hearing problems caused by build up of dead skin in the ears. Other implications of the condition often are inability to cope with hot weather, skin infection, growth deficiencies, sparse hair grow and stunted growth.

Ichthyosis cannot be cured. But by applying regular creams, dryness of the skin can be prevented.