Keratosis follicularis, also known as Darier’s disease, Dyskeratosis follicularis or Darier–White disease, is a genetic skin condition that usually affects the skin of the seborrheic regions and associated with fingernail abnormalities and mucous membrane changes. There is no definitive cure but the disease can be controlled with proper treatment.
Keratosis follicularis occurs in both male and female equally. The usual age of onset may be from 6-20 years but few cases have been described as early as 4 years or late age as 70 years. The disease in often worse during summer and is aggravated by ultraviolet light exposure ( especially UVB ) , heat, humidity and friction.
The rash associated with Keratosis follicularis are scaly, warty, greasy papules which occurs frequently on seborrhoeic areas of the body. The lesion most commonly affects the scalp, ears forehead, neck, chest and back. It also affects skin folds areas like under breasts, armpit and groins. The lesions can also be found on palms, soles and mucosal surface. The lesion are usually hard, thickened, greasy, skin colored or yellow brown and has a distinct odor. The initial lesion which occurs in teenage years may be itchy. These lesions are progressive and gradually gets bigger and spreads.
Finger nails change shows red and white longitudinal bands, longitudinal nail ridges and longitudinal splitting. Finger nails becomes fragile. Other additional symptoms and signs may be present in some cases. Atypical presentation and coexistence with other disease may be common. It can coexist with chronic familial pemphigus ( Hailey-Hailey Disease )
Treatment of Keratosis follicularis:
Basic treatment and preventive measures:
- Avoidance of hot environment, irritation to the skin and use cool cotton clothing and regular use of sunscreen may help prevent fares.
- Use of gentle cleanses and moisturizers with lactic acid or urea may help reducing scaling and hyperkeratosis.
- In case of inflammation low to mild potency topical steroids creams may be useful.
- Topical antibiotics may be used if any bacterial infection is suspected.
Topical retinoids like adapalene, tazarotene or tretinoin have been shown effective, but irritation may be the major issue. In such case, emollients and mild topical corticosteroids may be combined to reduce the irritation by retinoids. Intralesional corticosteroids and botulinum toxin type A have been used in few cases with some benefit.
- Oral retinoids are the most effective treatment for keratosis follicularis with 90% successive results in reducing the symptoms. They are effective in reducing the hyperkeratosis, reduce the odor and smoothen the rash.
- In some severe cases Etretinate and Acitretin have also shown some satisfactory results.
- Oral contraceptive pills are also reported to be effective in few of the cases, especially in case of pre-menstrual keratosis follicularis flares.
- Oral antibiotics may be necessary in case of secondary bacterial infection and as a prophylaxis to prevent further infection.
- In some widespread cases Cyclosporine has also been used with some beneficial results.
Variety of surgical techniques are being used with acceptable results. Laser ablation with carbon dioxide lasers, pulsed-dye lasers and Er;YAG lasers have shown to be successful in treating the lesions. For some localized lesions Electro-surgery and Mohs micrographic surgery have shown beneficial results. Dermabrasion can be used to smooth the hyperkeratotic lesions.