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Over Netherton, Ichthyosis en de stichting

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Bullous congenital ichthyosiform erythroderma

Bullous means: Blistering.  Congenital means that the condition is present at birth and caused by a genetic or inherited abnormality. Ichthyosiform refers to a skin that has a pattern like ‘fish-scales’. The skin is dry and scaly. Erythrodermic means red skin.

Bullous congenital ichthyosiform erythroderma is inherited as ‘Autosomal Dominant’. If one of the parents is affected there is a 50% risk for each child. The condition is related to an abnormality in keratins 1 and 10. Keratins play an important role in the structure and epidermisation of the skin.

From birth or soon after the skin of the child is red, scaly and blistering. As the child grows older the blistering will most likely become less. Thick dark scales will appear on the torso and in the skin creases of the armpits, elbows and knees.

Other characteristics of the condition are sparse hair grow on the scalp, thickening of the palms and soles and abnormalities in the nails.