What is Darier’s disease?
It is a rare inherited skin condition, in which the skin in certain areas develops large numbers of small brownish warty bumps.
What causes Darier’s disease?
In the outer layer (epidermis) of normal skin, the skin cells are held together like bricks cemented in a wall. In Darier's disease the sticky junctions that hold the skin cells together are not made properly, and the skin may become scaly or lumpy or even form blisters. It is not due to an allergy and it is not contagious (catching).
Is it hereditary?
Yes. It runs in certain families, being inherited in a pattern known as dominant inheritance. This means that there is a 1 in 2 (50:50) chance that each child of an affected parent will inherit the condition. It affects both men and women. Its severity may vary considerably within a single family; and if a person is badly affected it does not necessarily mean that other family members who inherit the condition will also get severe disease.
What are the symptoms of Darier’s disease?
Itching is very common. The affected skin may smell unpleasant, particularly in moist areas. This is probably caused by increased numbers of ordinary skin bacteria growing in the affected skin. The appearance of the rash, and its smell, can be embarrassing.
A quarter of patients notice that the condition improves as they get older. Some people find that the sun causes their Darier’s disease to flare up. Some women notice that it worsens around the time of their periods.
What does it look like?
The first signs of the condition usually appear somewhere between the ages of 6 and 20. Little brownish, rough-topped bumps develop on the skin. The severity of the condition varies a lot and is unpredictable. The rash is often on the chest, neck or upper back at the start but warty bumps may occur on any part of the body including the skin creases and skin under the breasts. It is unusual for people to have much trouble on the face except for the skin on the forehead near the hairline.
The fingernails are usually affected. They tend to be rather fragile, split easily and look as if they have been bitten or appear dirty. There may be very obvious long red or white lines running the length of the nails. Nail changes and/or flat "warts" on the backs of the hands are often present in childhood, well before there are any other skin changes. Pits or small areas of hard skin occur on the palms of the hands and less often the soles of the feet. Occasionally there may be small spots inside the mouth and these may give the roof of the mouth a rough feeling.
How will it be diagnosed?
The diagnosis can often be made on the appearance of the rash and the fact that it runs in families. To confirm it, a small sample of skin (a biopsy) can be removed under a local anaesthetic and examined under the microscope in the laboratory.
Can Darier’s disease be cured?
No, there is no cure, but there are many ways of helping it.
For information about available treatments please visit this page on the website of the British Association of Dermatologists