Other Names for this Disease
- Darier White Disease
- Darier's disease
- Keratosis follicularis
Your QuestionI have had the diagnosis of Darier disease for many years. My skin has recently been getting worse. I'm looking for more information about treatment, research and how to find a dermatologist in my area who has experience with this condition.
We have identified the following information that we hope you find helpful. If you still have questions, please contact us.
Questions on this page
- What is Darier disease?
- How is Darier disease inherited?
- Are there certain triggers that can irritate the skin and cause flare-ups of Darier disease?
- How might Darier disease be treated?
- Where can I find out about research for Darier disease?
- How can I find a dermatologist in my area who has experience treating Darier disease?
A person can have a mutation in the ATP2A2 gene for different reasons. In some cases, an individual with Darier disease inherits the mutation from one affected parent. Other cases may result from a new mutations (called de novo) in the gene. These cases occur in people with no history of the disorder in their family. The linear form of Darier disease is generally not inherited but arises from random mutations in the body's cells that occur after conception. These alterations are called somatic mutations.
The affected skin may smell unpleasant, particularly in moist areas. The smell is part of the skin condition and does not mean that the skin is dirty. It is probably caused by bacteria growing in the rash. When bacterial overgrowth is suspected or crusting is prominent, application of antiseptics such as triclosan or soaks in astringents such as Burrow or Domeboro solution can be helpful. 
Topical medication may include topical retinoids (i.e., adapalene, tazarotene gel, or tretinoin). Recent studies have shown that topical retinoids can reduce hyperkeratosis in 3 months. However, irritation is a side effect. Other medication may include Acitretin, Isotretinoin, Ciclosporine, or oral retinoids (eg, acitretin, isotretinoin). Oral retinoids have been the most effective medical treatment for Darier disease, achieving some reduction of symptoms in 90% of affected individuals. However prolonged use is limited by their significant adverse effects. Other treatments may include oral antibiotics to clear bacterial infection, oral acyclovir to treat or suppress herpes simplex virus infection, dermabrasion (sanding off the surface of the skin) to smooth the hyperkeratotic lesions, electrosurgery and Mohs micrographic surgery to treat localized areas. Carbon dioxide laser ablation, Er:YAG laser, and photodynamic therapy have also been tried with some success.
The National Registry for Ichthyosis and Related Disorders at the University of Washington was created with the support of the National Institutes for Health to encourage research into the diagnosis and treatment of the ichthyoses and related disorders including Darier disease. People that are affected by these conditions can enroll in the Registry as well as share information about ongoing research projects with those who ask to be notified.
The Foundation for Ichthyosis and Related Disorders has funded ichthyosis-related research through the Dermatology Foundation Grant Program.
American Academy of Dermatology
930 E Woodfield Rd
Schaumburg, Illinois 60173-4729
Web site: http://www.aad.org/
The following organizations may be able to help you find a dermatologist who has had experience treating individuals with Darier disease.
Foundation for Ichthyosis and Related Skin Types
Web site: http://www.firstskinfoundation.org
Medical Advisory Board: http://www.firstskinfoundation.org/content.cfm/Ichthyosis/Medical-Scientific-Advisory-Board/page_id/739
Web page with Darier disease information: http://www.firstskinfoundation.org/content.cfm/Ichthyosis/Darier-Disease/page_id/544
American Skin Association, Inc.
Web site: http://www.americanskin.org/
- Darier disease. Genetic Home Reference. March 2008; http://ghr.nlm.nih.gov/condition=darierdisease. Accessed 9/14/2011.
- Kwok PY, Fitzmaurice S. Darier disease. eMedicine. 2009; http://emedicine.medscape.com/article/1107340-overview. Accessed 7/18/2011.
- Goldsmith, Lowell A., Baden, Howard P.. Darier-White Disease (Keratosis Follicularis) and Acrokeratosis Verruciformis. In: edited by Freedberg, Eisen, Wolff, Austen, Goldsmith and Katz. Fitzpatrick's Dermatology in General Medicine, 6th edition. McGraw Hill Companies; 2003;
- What is Darier disease?. The Darier Disease Resource Site. http://dariers.50.forumer.com. Accessed 7/13/2011.