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.

Treatment


Newline Maker

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.[1]

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.[1]

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.[1]

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

References
  1. Kwok PY, Fitzmaurice S. Darier disease. Medscape Reference. September 21, 2012; http://emedicine.medscape.com/article/1107340-overview. Accessed 3/6/2014.
  2. 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;


Clinical Trials & Research for this Disease