Keratosis follicularis, also known as Darier disease, is slowly developing inherited skin disorder characterized by hyperkeratotic papules. As well as lesions, this skin affection causes nail abnormalities and mucous membrane changes. The disease onsets between 6 and 30 years of age and can affect men and women equally.

Symptoms of Keratosis Follicularis

The papules rise out of hair follicles. The lesions can develop into skin colored or yellow brown papules with a greasy uneven, or warty texture. The face, ears, chest and back are where they most commonly appear. These keratotic lesions are progressive and can grow larger or spread. Patients often experience periods of lesser symptoms (remission) and other periods of heightened discomfort (relapse). Specific characteristics of symptoms as well as periods of change depend on the patient.

Nail changes are important for diagnostic reasons because this symptom is unique to keratosis follicularis. Nails develop red bands and ridges as well as suffer from splitting and sometimes a V-shaped nick at the free margin of the nail. Hyperkeratosis can also develop beneath the nails.

Mucosal lesions develop in about 15 percent of patients. They develop in the mouth as white papules with a center depression. Oral lesion can obstruct salivary glands.

Treatment for Keratosis Follicularis

UV protection, cool cotton clothing and staying out of the heat can prevent outbreaks. Lubricating creams with urea or lactic acid can help with scaling and hyperkeratosis. Steroid creams can reduce inflammation. There are also injections, topical gels and oral medications that can reduce discomforting symptoms of follicular keratosis.

For more sever cases, surgical options are also available. Electrosurgery is more commonly used to treat localized keratosis follicularis. There is also new methodology using carbon dioxide lasers that help lessen symptoms. A longer lasting method is actually a combination of a few such as carbon dioxide lasers, curettage and the shaving away of lesions. This can put lesions into remission for up to 2 years. Photodynamic therapy consists of acid therapy proven to be effective in about 69 percent of patients, and can put symptoms in remission for up to 3 years.