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Over Netherton, Ichthyosis en de stichting

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Ichthyosis Vulgaris

Ichthyosis Vulgaris is a mild form of Ichthyosis and is the most common form: 1 out of 250 people. Ichthyosis means the skin appears to have the same structure as ‘fish-skin’. Vulgaris means ‘general’ or ‘common’.

Ichthyosis Vulgaris is an autosomal semi-dominant genetic disorder. When one of the parents has Ichthyosis Vulgaris there is a chance of 50% the disorder is passed to the child. The other parent without disorder will not influence this chance. A dominant inherited disorder will pass or will not pass. The abnormality lies in the filaggrin gene. The protein profilaggrin produces less and that causes abnormalities in building the skin barrier.

At birth the skin appears normal. After 2-6 months fine white or grey coloured scales develop. Most of the time the arms or under legs are affected. The severity varies widely from very mild cases of dry skin to severe areas of thickened skin and scaling over large areas of the body. Remarkably skin creases like the knee or elbow are not affected. The skin on palms and soles is thickened and fissures evolve. The lines on the palm appear darker. During summer the condition of the skin seems to improve as by sweating scales can be shed easier.

Often patients also have (Constitutional) eczema, asthma and hay fever. Further patients often have Keratosis Pilaris: a condition that hardens the hair follicle and give red marks.

There is no cure for Ichthyosis Vulgaris. But for half of the patients the symptoms will become less after puberty. It is necessary to keep the skin moisturised. Creams with urea can make the epidermis thinner. Steroid creams can be used for eczema.