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Darier's Disease Information and Treatment

Darier's disease is a genetic disorder discovered by a French dermatologist Ferdinand-Jean Darier. It results in dark crusty patches on the skin which at times contain pus. The crusty patches are also known as keratotic papules and also called keratosis follicularisis. It is hereditary disease and is dominant. It tends to affect both genders but is not condidered to be contagious. With the discovery of the ATP2A2 gene, performing genetic tests to confirm the diagnosis of DD is now possible. 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 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 odor. This disease can also cause the fingernails to be fragile at the tips causing V-shaped notches at the end of the nails as pictured below.

Darier's Disease is a chronis skin disorder. Darier's Disease is a common condition which can occur in any age group. Darier disease is a stubborn rash which usually runs in families. Darier's disease is an autosomal dominantly inherited skin disorder, characterized by loss of adhesion between epidermal 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, mucosa, and nails.

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