Introduction:
Darier's disease (DAR) is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier. Darier's is characterized by dark crusty patches on the skin that are mildly greasy and that emit a strong odor. These patches, also known as keratotic papules, keratosis follicularis, or dyskeratosis follicularis, most often appear on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear.
Signs and Symptoms:
Mild forms of the disease are the most common, consisting solely of skin rashes that flare up under certain conditions such as high humidity, high stress, or tight-fitting clothes. Short stature, when combined with poorly-formed fingernails that contain vertical striations, is diagnostic even for mild forms of DAR. Symptoms will usually appear in late childhood or early adulthood between the ages of 15– 30 years and will vary over the course of one’s life.
Epidemiology:
Worldwide prevalence is estimated as between 1: 30,000 and 1: 100,000. Case studies have shown estimated prevalence by country to be 3.8: 100,000 in Slovenia, 1: 36,000 in north-east England, 1: 30,000 in Scotland, and 1: 100,000 in Denmark
Treatment:
Treatment of Darier disease depends on the severity of the presented clinical symptoms. In most minor cases, the disorder can be managed using sunscreen, moisturizing lotions, avoidance of non-breathable clothing, and excessive perspiration. In more severe cases of Darier's disease, affected individuals may display frequent relapse and remit patterns. In less severe cases, signs and symptoms may clear up completely through hygienic interventions. Most patients with Darier’s disease live normal, healthy lives. Rapid resolution of rash symptoms can be complicated due to the increased vulnerability of affected skin surfaces by secondary bacterial or viral infections. Epidermal Staphylococcus aureus, Human Papillomavirus (HPV) and Herpes Simplex Virus (HSV) infections have been reported. In these cases, topical and/or oral antibiotic/antiviral medications may need to be prescribed.
Typical recommendations are:
Application of antiseptics
Soak in astringents
Antibiotics
Benzoyl peroxide
Topical diclofenac sodium
If Darier’s is more localized, common treatments include:
Topical retinoids- used to help in the reduction of hyperkeratosis, retinoids work by causing the skin cells in the top layers to die and be shed off. The common retinoids used for this disorder are:
Adapalene
Tazarotene gel
Tretinoin
Dermabrasion
Removal of the top layer of skin to help smooth and stimulate new growth of the skin.
Electrosurgery
Used to help stop bleeding and remove abnormal skin growths.
Topical corticosteroids
If symptoms are severe, oral retinoids be prescribed and have been proven to be 90% effective. However, there can be many adverse side-effects associated with prolonged use. Common oral retinoids are:
Acitretin
Isotretinoin
Cyclosporine
Some patients are able to prevent flares with use of topical sunscreens and oral vitamin C.
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