Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Darier disease


Other Names for this Disease
  • Darier White Disease
  • Darier's disease
  • Keratosis follicularis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.


Your Question

I 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.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Darier disease?

Darier disease is an inherited skin condition characterized by wart-like blemishes on the body usually located on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. Other features of Darier disease include nail abnormalities, such as red and white streaks in the nails with an irregular texture, and small pits in the palms of the hands and soles of the feet. The severity of the disease varies over time. A form of Darier disease known as the linear or segmental form is characterized by blemishes on localized areas of the skin.  Darier disease is not an infection and the blemishes are not contagious. Symptoms usually first appear in late childhood or early adulthood. This condition is caused by mutations in the ATP2A2 gene and inherited in an autosomal dominant fashion.[1]
Last updated: 3/6/2014

Are there certain triggers that can irritate the skin and cause flare-ups of Darier disease?

Heat, sweat, humidity, sunlight, UVB exposure, lithium, oral corticosteroids, and mechanical trauma (e.g. under the collar of sweaters) have been reported to exacerbate this condition. Some women report flares around menstruation.  Other common complications and causes of exacerbation are bacterial infections and infection with herpes simplex virus.[2]
Last updated: 3/6/2014

How might Darier disease be treated?

Basic measures to manage Darier disease may include using sunscreen, wearing cool cotton clothing, and avoiding hot environments. Itching is very common. Moisturizers with urea or lactic acid can reduce scaling and hyperkeratosis. A low- or mid-potency topical steroid is sometimes useful for inflammation.[3]

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.[3]

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.[3]

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.[2]
Last updated: 3/6/2014

Where can I find out about research for Darier disease?

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, there are clinical trials listed for Darier disease OR icthyosis. Click on the study title to learn more and review its 'eligibility' criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.
Last updated: 3/6/2014

How can I find a dermatologist in my area who has experience treating Darier disease?

You can find a dermatologist in your area on the homepage of  the American Academy of Dermatology's web site:

American Academy of Dermatology
930 E Woodfield Rd
Schaumburg, Illinois 60173-4729
Telephone: 847-330-0230
Fax: 847-330-0050
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
26126 N. Broad Street
Colmar, PA 18915
Telephone: 215-997-9400
Fax: 215-997-9403
E-mail: info@firstskinfoundation.org
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.
346 Park Avenue South, 4th Floor
New York, NY 10010
Toll-free: 800-499-SKIN
Telephone: 212-889-4858
Fax: 212-889-4959
E-mail: info@skinassn.org
Web site: http://www.americanskin.org/

Last updated: 3/6/2014

References
  • Darier disease. Genetics Home Reference. March 2008; http://ghr.nlm.nih.gov/condition=darierdisease. Accessed 3/6/2014.
  • 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;
  • Kwok PY, Fitzmaurice S. Darier disease. Medscape Reference. September 21, 2012; http://emedicine.medscape.com/article/1107340-overview. Accessed 3/6/2014.