Like many rare diseases, this one goes by several names. Darier disease is also known as Darier White disease, DAR, keratosis follicularis and Darier’s Disease, according to the National Institutes of Health.
Patients who suffer from this condition present with wart-like blemishes that are typically located on the scalp, forehead, upper arms or chest. They also form on the back, knees, elbows and behind the ears. Darier sufferers often also have red and white streaks in their nails and nails with an irregular texture. They also sometimes note small pits in the palms of their hands and in the soles of their feet.
A variant of this disease is the linear or segmental form. Patients with this type of Darier have blemishes on localized areas of the skim. With either type, symptoms usually first appear between the ages of 6 and 20. The peak incidence occurs between ages 11 and 15. Darier blemishes are not contagious.
According to DermNet NZ, an accurate diagnosis requires a skin body. The pathology is similar to and sometimes mistaken with that of Grover’s disease (also known as transient acantholytic dermatosis). Disease severity varies greatly from one patient to another. Some might have lesions so small that they’re visible only on close inspection.
Others initially develop persistent small bumps that are greasy because they tend to appear on areas of the body such as the edge of the scalp, forehead, ears, nostrils, eyebrows and beard area. Other oily areas include the neck, central chest, and back. Areas where the skin folds are also often affected. Sometimes blisters develop. Darier patients also sometimes show a white cobblestone pattern on the mucous membranes or an overgrowth on their gums.
Some patients have such a mild form of the disease that it goes unnoticed throughout most if not all of their lives. Treatment varies for others because the condition tends to go into remission, then relapse. Since sunlight can cause the disease to flare, a day at the beach can signal the beginning of a bout. Sometimes doctors prescribe steroids to control symptoms.
When a bacterial infection kicks off a flare, it’s necessary to appropriately treat the infection as well as the Darier symptoms. One well-recognized complication is a widespread skin infection with the herpes simplex virus, which can cause a severe flare.
In general, physicians treat Darier disease only when the symptoms become troublesome. For mild cases, sometimes simple things such as moisturizers, protection from the sun, choosing the right clothing and avoiding anything that will cause the patient to sweat are adequate.
When Darier disease is localized, doctors sometimes use dermabrasion to sand off the skin’s surface or prescribe topical retinoids. These chemical compounds are related to Vitamin A and regulate epithelial cell growth.
For secondary bacterial infections such as staph, antibiotics are in order. Doctors prescribe antiviral agents to treat herpes simplex. Cyclosporine has also proven effective for some patients.
Wikipedia reports that this condition was named after Ferdinand-Jean Darier, the French dermatologist who discovered it. The disease is caused by a genetic flaw. It’s classified as an autosomally dominant inherited mutual in the gene known as ATP2A2. This gene is located on chromosome 12q23-24.1. Researchers are still looking for the way this abnormal gene causes the disease to appear. It’s linked to transporting calcium within cells. Medical professionals believe the defective gene somehow affects the way skin cells stick together when there is insufficient calcium.
Because the genetic cause of the disease is autosominally dominant, it takes only a single gene from one parent for the problem to occur. The chance of inheriting this abnormal gene if just one parent is affected is one in two. However, not everyone who inherits the abnormal gene will actually develop Darier symptoms.