Darier disease

A new disease profile is added to our database – Darier disease, (DD). Keratosis follicularis, also known as Darier disease (DD) or Darier-White disease, is an autosomal dominantly inherited genodermatosis characterized by greasy hyperkeratotic papules in seborrheic regions, nail abnormalities, and mucous membrane changes. The disease was first reported independently by Darier and White in 1889. White was first to recognize the genetic nature of DD by noticing that a mother and her daughter were affected. Mutations in the gene ATP2A2 cause DD. The disease occurs worldwide. The prevalence of DD has been estimated to range from 1 case in 30 000 population in Scotland to 1 case in 100 000 population in Denmark. Males and females are equally affected. DD most commonly manifests from age 6-20 years; however, a patient may first present as early as age 4 years or as late as age 70 years. To receive a full description of this rare disease absolutely free of charge, please click here and send us a request. The profile was written by our consultant in dermatology – Prof. Dr. Krasimira Chudomirova, MD, PhD.