Darier’s Disease

What is Darier disease?

It is a rare inherited skin condition, estimated to affect 1 to 4 people per 100,000 of the population and is characterised by a change in the way skin cells (keratinocytes) stick together within the upper layer of the skin (epidermis). This leads to changes in the skin and nails, and inside the mouth can sometimes be affected. Other names for Darier disease include Darier-White disease and Keratosis Follicularis.

What causes Darier disease?

The movement of calcium within cells is disrupted, leading to a change in the way skin cells are held together. Normally, these cells are held together like bricks cemented in a wall. In Darier disease, the ‘cement’ that holds the skin cells together is weakened, so the cells separate easily and do not form a good barrier against the outside world. This causes the skin to become easily irritated, inflamed and it may begin to weep. It is not due to an allergy and it is not contagious (catching). Darier disease is often aggravated by heat, sunlight, skin friction, excessive sweating, and it can make the skin more prone to infection. Some females may notice their skin flares before their period. Certain prescribed medications (usually taken by mouth) may also make the rash worse.

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