It is a genetic disorder which was discovered by a French dermatologist Ferdinand Jean Darier. It results in dark crusty patches on the skin which at times contain pus. These crusty patches are known as keratotic papules. It is hereditary disease and is dominant. It tends to affect both genders but is not condidered to be contagious. The discovery of the ATP2A2 gene has made it possible to perform genetic tests in order to confirm the diagnosis of this disease.

This disease is also referred to as keratosis follicularis. It is considered to be a rare genetic disorder which is predominantly manifested by skin changes. Usually in adolescence the onset of skin changes occur and the disease is generally chronic. It gets inherited by an autosomal dominat pattern which implies that a single gene that passes on from one parent causes this condition. Not every human being with abnormal gene develops symptoms of this disease. It can be diagnosed by its appearance and family history but is sometimes mistaken for other skin problems. For diagnosing Darier’s disease a skin biopsy may be required. It normally affects the neck, back, ears, forehead and groin but may also affect other body areas. It may also result the fingernails to become fragile at the tips causing V-shaped notches at the end of the nails.

Cause

It is considered to caused by a defective disease. The gene is generally inhereted from one’s parent. As it is not contagious, it cannot spread from person to person. The rashes worsen during summers due to heat and humidity.

Symptoms

The signs and symptoms of Darier’s disease vary between individuals. Some show subtle signs that are asymptomatic and can be found only on careful inspection. Some tend to develop extensive lesions which can result in considerable distress to the affected individual. In case of an affected individual the severity of the disease can fluctuate over time. The lesions of the skin are characterised by greasy and scaly papules which tend to occur over the seborrhoeic areas of the face, neck, back and central chest.

Treatment

Treatment is required only in cases of troublesome symptoms. Patients suffering from mild disease can use simple moisturiser, select the right kind of clothing and protect one self from sun. Localised Darier’s disease can be treated with topical retinoids. Secondary bacterial infections can be treated with antobiotics. If syptoms are quite severe then a trial of an oral retinoid meication such as acitrentin or isotretinoin may be effective.