Dariers Disease

Close-up of the skin of a patient with Darier's disease (keratosis follicularis). This hereditary (genetic) skin disorder is characterised by the eruption of small greasy pimples usually found on the torso and head.

Darier disease is also known as ‘keratosis follicularis. Darier’s disease affects both men & women. It is not catching or due to an allergy, it runs in certain families. Darier’s disease is hereditary and dominant. This disease may occur during or later than the teenage years. The disease affects the outermost layer of the skin, the epidermis, which thickens around the hair follicles. These lesions become darker over time and commonly fuse, forming papillomatous and warty malodorous growths. The skin cells (keratinocytes) stick together via structures called desmosomes and it seems the desmosomes do not assemble properly if there is insufficient calcium. Darier’s disease is hereditary and dominant. This disease may occur during or later than the teenage years.

Signs and symptoms of Darier’s disease vary between individuals. 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. 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. Widespread infection of the skin the skin with the herpes simplex (coldsore) virus is a well recognised complication. This can cause a severe flare and patients can feel quite unwell. Treatment is required only if there are troublesome symptoms.

The main treatment of the dariers disease is protection from the heat or sunlight. If symptoms are particularly severe then a trial of an oral retinoid medication such as acitretin or isotretinoin may be effective. Ciclosporin has been reported to be effective in a few patients. Secondary bacterial infection should be treated with antibiotics, and herpes simplex with antiviral agents. Properly during summer it is necessary to use sunscreen, cool clothing & avoidance of hot environments which helps to protect from heat. There are some surgical methods available such as electrosurgery, mohs micrographic surgery and another is dermabrasion. There is no medical cure for dariers disease but drug like accutane may be useful.

Dariers Disease -Prevention and Treatment Tips

1. Selection of the right clothing to avoid heat and sweating are usually sufficient.

2. Properly during summer it is necessary to use sunscreen.

3. Ciclosporin is also effective treatment for dariers disease.

4. Oral retinoid medication such as acitretin or isotretinoin may be effective.

5. Topical 5-fluorouracil has been used effectively in a few patients.

6. Oral contraceptives have been reported to help with perimenstrual DD flares

7. Etretinate has been reported useful if acitretin fails.