The Darier disease leads to the appearance of rough and hard growths and blemishes on the body, named warts. These have a hard consistency, are usually yellow, have a strong smell and can be quite oily. They can emerge on various areas of the body but most often of the scalp, forehead, behind the ear, chest, back, knees, elbows and the upper part of the arms. They can sometimes appear on sensitive mucous tissues, for example on the mouth palate, throat, gums, tongue or on the inside cheeks. Besides the warts, Darier disease can have other symptoms like irregular red or white lines on the nails or tiny depressed areas on the feet soles or hand palms.
The skin warts that are the trademark of this disease develop at the end of childhood or in young adults. Aging tends to reduce the severity of Darier disease in time. Typically, people who suffer from it will go through remission periods with few warts and periods of exacerbation when many of them appear. Environmental causes also have a direct influence on the outbreaks. Heat and moisture during the summer will make more warts emerge on people who suffer from the disease. Other factors than can lead to more warts emerging are rubbing or scratching the skin, friction, small injuries but also some drugs.
Darier disease is sometimes connected with serious neurological issues ranging from depression to epilepsy and a moderate intellectual handicap. They can also have learning and behaviour problems of various severity. It is unknown if these problems are connected with the genetic faults that causes Darier disease, since they do occur in many people who don't have them. It might be nothing but a coincidence. However, some doctors suspect that people with skin warts caused by the disease are shunned by society and end up having behavioural and social challenges.
Darier disease has a particular type named the linear or segmental form. In this case, the blemishes only develop on some specific areas of the skin and are not found on most of the body, like in the normal variety of the disease. Another particularity of the linear form is that patients can develop the nail problems encountered on the normal disease, but on just half of the body not on both sides.
The Darier disease is usually chronic and the first signs start in adolescence. It is a genetic disease which is caused by just one gene inherited from one parent, which is designed as an autosomal dominant pattern in scientific terms. Children have a chance of about 50% to inherit the gene from one of their parents. However, many people who get the faulty gene will never develop the actual disease.
The faulty gene that can lead to Darier disease symptoms is named ATP2A2. Scientists have not been able to determine yet the exact mechanism of how the disease starts from this gene. They suspect that the link between skin cells could be affected. Keratinocytes are bound on the skin by dedicated structures named desmosomes. The gene might affect the available amount of calcium and the desmosomes fail to stick together without this mineral.
Patients can have various symptoms of the Darier disease. The condition can be very mild in some cases, with small signs that are hard to observe and can only be found after a thorough investigation. At the same time, other people might develop a massive amount of blemishes, with a devastating psychological effect. The severity of the symptoms changes in time.
Typical for the disease are the long-lasting warts, which can be oily and covered with scales. These usually occur on specific zones on the face that are rich in sebum, like the ears, forehead, and edges of the scalp, nostrils, beard or the eyebrows. They can also appear on the chest, back, scalp, neck and any area where the skin is folded such as between the buttocks, on the groin, armpits or under the breasts.
The warts are usually the same color as the skin but can also be yellow or brown. They have a firm feel and a rough surface that resembles sandpaper. The papules are small but they can unite into a larger one that develops a nasty smell when located in areas where the skin is folded. Sometimes they are covered with a rash or a crust, similar to other skin diseases.
Sometimes, Darier disease can have unusual symptoms. These include papules that look like blisters, large-seize skin lesions, small and flat ones resembling freckles, or warts arranged in lines along the so-called dermatomal distribution.
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