Darier’s disease

Dariers disease is an autosomal disorder and is non-contagious. Crusty patches with pus are characteristic of this disease, it affects any part of the body. The condition becomes worse during high humidity, stress and tight clothing. Vertical striation in fingernails & fragile nails is diagnostic features of the disease. Sometimes a patient may develop depression and epilepsy. Homoeopathic medicines really help in managing the symptoms of the disease.

dariers disease

 

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Dariers disease is an autosomal dominant disorder. Darier’s disease is first described by dermatologist Ferdinand-Jean Dairer. Dark crusty patches, sometimes containing pus, is the characteristic appearance of skin in darier’s disease. It most commonly affects the chest, neck, back, ears, forehead, and groin, but may involve other body areas. Skin condition aggravates during high humidity, stress and wearing tight-fitting clothes. Vertical striation in fingernails and fragile nails are diagnostic features of darier’s disease. Rashes in darier’s disease have a distinct odor. Intraoral papule can be found, palms and soles may become thick. Darier’s disease is not a contagious disease.

Commonly affected parts of the body are scalp, forehead, behind the ears, arms, elbows, back, chest and knee. Mucous membranes also get involved.

Occasionally patient with Darier’s disease may develop neurological disorders as depression, epilepsy or mild intellectual disability. A minor form of the disease may remain undiagnosed lifelong.

Commonly Seen In
Darier’s disease affects both males and females.
Darier’s disease commonly starts from 6-20 years of age. However, a patient of age 4 and up to 70 years are also seen affected by the disease.

Mortality/morbidity of Darier’s Disease
In dariers disease, there is increased susceptibility to cutaneous bacterial and viral infections. Misdiagnosis of darier’s disease leads to under-treatment of these infections and may prove fatal.

However, the patient with darier’s disease has a life expectancy as that of the general population.

Causes of Darier’s Disease ?
The cause of development of Dariers disease is a mutation in the gene that codes for enzyme SERCA2 (sarcoendoplasmic’s reticulum CA2+-ATPase). The gene ATP2A2 is localized in chromosome 12 and controls the synthesis of the SARCA2 enzyme. This enzyme pumps calcium across the cell membrane. If one of the two genes ATP2A2 is affected, the normal functionality in skin cells is not maintained, there is the impaired passage of calcium in skin cells resulting in the development of horny cells prematurely before they reach the surface of the skin.

What are the sign and symptoms ?
Symptoms start with the appearance of rough papules in the outermost layer of skin, mainly around the hair follicle. The papules then develop into a brownish rash then grow together and form a thick plaque with a moist surface. Plaques are susceptible to infections from bacteria and herpesvirus. The severity of the disease varies over time. Hot and humid climate aggravate the rashes. There is increased growth of bacteria on skin lesions that emit an unpleasant odor. Rashes are moist and bleed less often. Acute swelling of parotid gland occurs as a result of obstruction of salivary glands.

Investigations for Darier’s Disease
Skin biopsy shows degenerated cells in the epidermis and increased keratinization.

How To Treat Darier’s Disease ?
• Treatment aims at relieving the symptoms.
• Softening creams are used to counteract the thickening of the horny layer.
• Oral retinoids are used to normalize the keratinization process. But the restricted use of retinoid is recommended as it has side effects. It is strictly avoided in pregnant women as it can cause birth defects.
• Surgery may be required in case of obstruction of salivary glands.
• In the case of skin infection, antibacterial treatment is required. In herpes virus infection antiviral agents are used.

How to prevent Darier’s Disease?
• Avoid exposure to direct sunlight.
• Loose fitting clothes are advised to wear.
• Frequent showering proves beneficial but excessive scrubbing may cause weeping sores.

Differential diagnosis of Darier’s Disease
• Seborrheic dermatitis.
• Transient Acantholytic Dermatosis.
• Familial benign pemphigus.
• Acrokeratosis verruciformis of Hopf.

Dariers Disease

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