Darier's Disease - Causes, Symptoms & Treatment

Darier's Disease is a chronis skin disorder. Darier's Disease is a comman condition which can occur in any age group. Darier's Disease is an autosomal dominantly inherited skin disorder, characterized by loss of adhesion between epidermak cells and abnormal keratinization. Darier's Disease is an autosmoal dominant disease that typically arises during the first or second decades of life. It involves the abnormal keratinization of the epidermis, moucosa and nails. Darier's disease is a type of disorder relating to genetical problems , It is a genetic disorder of keratinisation based mainly on hair follicles that is inherited as an autosomal dominant trait. It usually presents in the first or second decade of life. It is aggravated by sunlight. Darier's disease is known because of dark crusty patches on the skin, sometimes containing pus. The crusty patches are also known as keratotic papules and also called keratosis follicularisis.

Darier's disease is discovered by French dermatologist Darier Jean Ferdinand which is a genetic disorder. In this disease there are crusty patches occurs on the skin, sometimes these patches may contain pus. The crusty patches are also known as keratotic papules and also called keratosis follicularis. Mutations in the gene ATP2A2 cause Darier's disease. Family members with confirmed identical ATP2A2 mutations can exhibit differences in the clinical severity of disease, suggesting that other genes or environmental factors affect the expression of Darier's disease. With the discovery of the ATP2A2 gene, performing genetic tests to confirm the diagnosis of Darier's disease is now possible. The disease is hereditary and dominant . It affects both men and women and is not contagious. The disease often starts during or later than the teenage years, typically by the third decade. The symptoms of the disease are thought to be caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion. It most commonly affects the chest, neck, back, ears, forehead, and groin, but may involve other body areas.

Causes of Darier's Disease

Find common causes and risk factors of Darier's Disease :

• The main cause of this disease is abnormality in the gene, environmental factors, heat, humidity, and exposure to sunlight
• In summer a red eruption of the skin occurs due to heat & humidity
• This disease inherited from family to family, there are 50% chance that each child of an affected parents may inherit the problem.

Signs and Symptoms of Darier's Disease

Sign and symptoms may include the following :

• Some common features of this disease are itching, little brownish, rough-topped bumps may develop on the skin. And also small pits occur on the palms of the hands & the soles of the feet.
• In dariers disease an unpleasant smell may occur from patient.
• The ducts of the salivary glands in the cheeks may be obstructed, resulting in acute swelling of the parotid gland.

Treatment for Darier's Disease

Treatment may include:

• Topical and systemic antibiotics to treat secondary bacterial infections.
• There are some surgical methods available such as electrosurgery, mohs micrographic surgery and another is dermabrasion.
• Use of sunscreens.
• Use Topical retinoids - tazarotene, adapalene.
• There is no medical cure for dariers disease but drug like accutane may be useful.

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