Short answer · Medically reviewed summary · Last updated: 2023-07-13
Darier's disease, also known as Darier-White disease or keratosis follicularis, is a rare genetic skin disorder that affects the skin's ability to adhere properly. It is estimated to have a prevalence of approximately 1 in 30,000 to 1 in 100,000 individuals worldwide. Darier's disease is characterized by the presence of small, rough bumps on the skin, particularly in areas such as the scalp, forehead, chest, back, and groin.
What is the prevalence of Darier's disease?
Prevalence of Darier's disease: how many people are affected worldwide, differences by sex and region, with sources.
Darier's disease, also known as Darier-White disease or keratosis follicularis, is a rare genetic skin disorder that affects the skin's ability to adhere properly. It is estimated to have a prevalence of approximately 1 in 30,000 to 1 in 100,000 individuals worldwide.
Darier's disease is characterized by the presence of small, rough bumps on the skin, particularly in areas such as the scalp, forehead, chest, back, and groin. These bumps, known as keratotic papules, can be accompanied by other symptoms like thickened nails and white streaks in the mouth.
The condition typically begins in adolescence or early adulthood and may worsen over time. While the exact cause of Darier's disease is not fully understood, it is known to be caused by mutations in a specific gene called ATP2A2. These mutations disrupt the normal functioning of calcium channels in the skin cells, leading to the characteristic skin abnormalities.
Although Darier's disease is rare, it can significantly impact an individual's quality of life due to the physical appearance of the skin lesions and associated symptoms. Proper management involves a multidisciplinary approach, including dermatological care, genetic counseling, and symptomatic treatment.
