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What is Dariers Disease?
Darier disease(DD) is also known as keratosis follicularis, Darier-White disease. It is caused by genetic disorder and is affected on the skin and over the seborrhoeic areas of the face, neck, and central chest and back. Most patients with Darier disease will have longitudinal broad stripes of white and reddish colour on the nails. A V-shaped nick at the free edge of the nail of Darier disease. DD occurs worldwide.Maes and females are equally affected. It is seen in between the age of 6 to 20 years. The disease is hereditary and dominant.
Causes of Dariers Disease
- Mutations in the gene ATP2A2
- Abnormality in the desmosome-keratin filament complex
- Dominant inheritance
- Increase in numbers of ordinary skin bacteria
- A defective gene
What are the symptoms of Dariers Disease?
- Skin rash
- Pain in affected skin areas.
- Skin biopsy
- Keratotic papules in the seborrheic regions
- Dark crusty patches on the skin, sometimes containing pus
Dariers DiseaseTreatment
- Darier disease may be treated successfully with topical retinoids.
- Retinoid medication such as acitretin or isotretinoin may be effective. Ciclosporin has been reported to be effective in a few patients.
- Moisturizers with urea or lactic acid can reduce scaling and hyperkeratosis.
- Topical 5-fluorouracil is used effectively in a few patients.
- Surgical -Electrosurgery and Mohs micrographic surgery have been used to treat DD areas, with good results.
- Benzoyl peroxide is applied over the affected area in the concentration of 2.5% although skin tolerance usually occurs after a week.
- Vaseline Intensive Care Dry Skin Lotion with Vitamins A and E applied to the rash twice a day can often clear the lesions in 2-3 weeks.
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