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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 papules are yellow-brown or skin colored with a warty greasy texture. They usually appear on the face, ears, chest, and back. These keratotic lesions are progressive and can 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.

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. Sometimes hyperkeratoses are seen beneath the nails.

Mucosal lesions appear on approximately 15 percent of patients suffering from keratosis follicularis. They develop in the mouth as white papules with a center depression. 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. Steroid creams can reduce inflammation. There are also treatments such as topical creams, injections, and oral medications that deal with uncomfortable symptoms of follicular keratosis.

For more serious cases, surgical options are also offered. Electrosurgery is more commonly 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. Acid therapy, or photodynamic therapy, is proven effective for about 68 percent of sufferers and can control symptoms for up to 3 years.