Darier Disease |
The occurrence of Darier disease is rare with the age of onset usually in the first or second decade of life. It is frequently worse in the summer with heat and humidity as major precipitating factors and can be exacerbated by sun exposure, trauma, or bacterial infections.
Patients diagnosed with Darier disease are recommended to wear sunscreen, cool cotton clothing, and avoidance of warm environments to prevent flares, especially during the summer season. Moisturizers with urea or lactic acid can help reduce scaling and thickening of the lesions. Low to medium strength topical steroids are sometimes useful for reducing inflammation and when bacterial growth is suspected, application of antiseptics can be helpful. Topical retinoids such as tazarotene and adapalene have been shown to be effective in reducing the localized symptoms of this disease but the most effective medical treatment for severe cases by far has been the use oral retinoids such as acitretin and isotretinoin. The prolonged use of oral retinoids, however, is often limited by its side effects which includes, but are not limited to, mucosal dryness, photosensitivity, abnormal liver functions, and bone deformities. Oral retinoids are highly teratogenic and should not be given to female patients without proper counseling and contraception. Surgical treatment of Darier disease includes dermabrasion, electrosurgery, and MOHS micrographic surgery, however, recent advances in the use of lasers have been reported with positive outcomes of remission up to two years.
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