What Is Darier Disease?

Darier disease, also known as Darier’s disease, Darier-White disease or keratosis follicularis, is a skin condition in which yellow, greasy, wart-like blemishes occur on the body. These blemishes characterize the disease, although other symptoms might appear as well. For the most part, this disease is an inherited disorder, meaning an affected person receives it from his or her parent. As the affected person inherits the condition in an autosomal dominant pattern, he or she only needs to receive a copy of the mutated gene from one parent to have the disease. Treatment for Darier disease varies, depending on the severity of a person’s condition and includes medications, surgery and prevention measures.

Accompanied by an odor, blemishes appear on the scalp, forehead and the back of the ears. In addition, they also commonly develop on the upper arms and elbows, as well as the chest, back and knees. They can even appear in the mouth and throat, such as on the gums, inside cheek and tongue. Aside from these blemishes, other symptoms of Darier disease include nail abnormalities and small pits, both of which might occur on the hands or feet. Nail abnormalities include red and white bands, ridges and V-shaped nicks.

Symptoms of Darier disease typically first appear during the teenage years. In general, the severity of the disease might change over time, with flareups occurring every now and then, depending on varying factors. The heat and humidity of summer, for one, can be responsible for an increase in blemishes. Other factors that affect the amount of blemishes at any given time include friction, injury and ultraviolet (UV) light.

Although most people with Darier disease inherit a gene mutation, some people do not have a family history of the disease and appear to develop the condition through a new mutation. Others might exhibit a localized form of the disease known as linear or segmental Darier disease. In this form, an affected person experiences the same symptoms associated with the usual form of the disease, except the blemishes are confined to a smaller area. Also, the symptoms might only appear on one side of his or her body.

A variety of treatment methods can provide relief from Darier disease symptoms, including oral and topical medications, surgery and taking preventative measures to inhibit flareups. Examples of oral medications that might help with the condition are retinoids, antibiotics and contraceptives. Topical medications include retinoids and corticosteroids. In addition, surgical procedures such as dermabrasion, electrosurgery and laser ablation can also help in treating the disease. Preventative measures include avoiding heat and humidity when possible, using sunscreen, and wearing clothing that allows the skin to breathe.