Q80: Congenital ichthyosis

Q80.2: Lamellar ichthyosis

The top of layer of skin is made up of skin cells that are keratinized. These skin cells are initially in one of the lower skin layers. When the skin cells migrate upwards toward the skin’s surface, they keratinize, forming a solid layer.

The genetic information in your cells has changed. This disrupts the keratinization of your skin.

There are various forms of this condition. Some forms cause the skin to be red and flaky after birth. You can get colder than usual or lose fluid through the skin. The skin can become inflamed more easily than usual and large wounds can form. With other forms there is a firm layer all over the skin or on large parts of the skin. You may also have other symptoms.

The symptoms can change or disappear completely when one is older. You can get brownish scaly skin or firm patches on the skin.

This disease is hereditary. Other family members may also be affected.