Q80.3: Congenital bullous ichthyosiform erythroderma

You have a congenital skin disorder where the skin cells do not keratinize properly.

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 produce blisters or wounds 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. The condition can change and, for example, improve when one is older. You can then have red and scaly skin, for example.

With other forms, you only get blisters on the skin, for example. The blisters can occur without any external cause or after minor injuries, among other things. Various other symptoms may occur as well.

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