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. Women and men are both affected equally and the disease onsets between 6 and 20 years of age.

Symptoms of Keratosis Follicularis

The papules rise out of hair follicles. The lestions have a greasy warty texture and are skin colored or yellow-brown color. They most commonly appear on the face, ears, chest and back. These lesions may grow larger or spread. Some patients experience periods of remission and relapse of symptoms. Specific symptoms, periods of change and responsiveness to treatment vary from person to person.

People affected experience nail abnormalities which are important for diagnostic reasons. Nails form red bands, ridges, and often show V-shaped splits at the end. Hyperkeratosis can also develop beneath the nails.

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

Treatment for Keratosis Follicularis

Sunscreen, loose cool clothing, and avoiding hot environments and usually prevent uncomfortable outbreaks. Moisturizing creams that have urea or lactic acid can reduce scaling and hyperkeratosis. For inflammation, a topical mid-potency steroid can help. There are also some topical prescription creams, injections, and oral medications that can also significantly relieve uncomfortable symptoms associated with follicular keratosis.

For more serious cases, surgical options are also offered. Electrosurgery has been used to treat localized keratosis follicularis. New treatment methods using carbon dioxide lasers also help to reduce symptoms. A combination treatment using carbon dioxide lasers, curettage and shaving can lead to longer remission periods. 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.