Darier's disease

Darier's disease is a hereditary skin disease with cornification disorder (hyperkeratosis).

The cause of the disease is a mutation in the ATP2A2 gene, which causes a disruption of protein production. The consequence is an insufficient distribution of calcium in the cytoplasm of the keratinocytes of the skin.

Most patients manifest the disease between the ages of 6 and 20 years. While a complete remission is rare, the syndrome usually runs in episodes with phases of largely lacking in appearance.

The symptoms are usually redness, swelling, reddish and crusty papules, white papules in the area of ​​the mucous membranes, grooves in the nails, as well as itching and unpleasant body odor. The lesions usually occur in seborrheic areas.