Dariers disease is a stubborn rash which usually runs in families. The rash often starts when one is a teenager or older. It most commonly affects the chest, neck, back, ears, forehead, and groin, but may involve other body areas. At times the rash will cause a bad odour. This disease can also cause the fingernails to be fragile at the tips.

The condition was described in 1889 by Jean Darier, a French Dermatologist.

In normal skin, the skin cells are held together by special "glues" and "cables", like bricks cemented in a wall. In this condition the skin cells are not held together properly and the skin may become scaly or lumpy or even form blisters. We do not understand why the skin behaves like this

It is a rare inherited skin condition which occurs in certain families and affects both men and women. The condition is pass~Z on fro a one generation to the next in the same way that the colour of the eyes may be inherited by cera~ members of a family. The condition is dominantly inherited and therefore there is a 1 in 2 chance [or 50:50 chance] that each child of an affected parent will inherit the skin problem. People affected by Darier’s disease develop itchy, warty spots, but the condition is not an infection and these spots are not contagious. You cannot give Darier’s disease to someone in the way that you may pass on your cold or sore throat.

The first signs of the condition usually appear somewhere between the ages of 6 and 20, but it may begin when people are older or rarely younger. Little brownish, rough topped bumps develop on the skin. The severity of the condition varies enormously and is unpredictable.

Patients have scattered spots which cause very little trouble but in others the disease is much more obvious. The chest, iieck or upper back ~-e cf~en involved at the beginning but warty bumps may occur on any part of the body. It is unusal for people to have much trouble on the face except for the skin on the forehead near the hairline. The scalp and skin around the ears may be scaly and itchy and most patients notice some small spots in the armpits, in the grains or, in women, under the breasts.

The finger nails 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 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 "corns’ occur on the palms of their hands and less often the soles of the feet.

Occasionally there may be small spots inside the mouth and these give the roof of the mouth a rough feeling.

Infections

There does seem to be an increased chance of developing skin infections if you have the condition. The "cold sore" virus, Herpes simplex, sometimes causes widespread skin infections in people with Darier’s disease. If the condition suddenly gets worse and is much more painful than usual this may be a sign of Herpes simplex infection. The GP or Dermatologist should be contacted urgently in these circumstances.

Outcome

A quarter of patients notice that the condition improves as they get older.
Most people lead full, normal lives and have no other medical problems. Less than a quarter need any time off work or school because the skin is a problem.

This page is an article published in March 1999, detailing a genetic study into Dariers Disease. We have put this link in just to show you that there is currently research being carried out on the disease. Hopefully, by discovering the cause of the disease, a cure can be found.
***Warning- This text is very technical ***