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.
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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.
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
26126 N. Broad Street
Colmar, PA 18915
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
Web site: http://www.americanskin.org/
- Darier disease. Genetic 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.