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by Cornel J
(Keratosis Follicularis)

Definition

Darier disease is a rare disorder in which thick, rough bumps appear on the skin. It is also known as keratosis follicularis.

Causes

Darier disease is a caused by a problem with a specific gene. The faulty genes disrupt the way skin cells join together. The gene may be passed from a parent or change on its own.

Genetic testing can determine if you have the gene. A counselor will help you estimate the risk of passing it to your child.

Risk Factors

Having a family member with Darier disease increases the risk of having it.

Symptoms

Symptoms usually begin during the teen years. Darier disease bumps:

  • Are small and hard bumps
  • May be skin-colored, brown, or yellow
  • Can have a greasy discharge with a foul scent
  • Can be itchy
  • May be worse when exposed to sunlight or with rubbing or certain medications.
  • May appear on the chest, back, forehead, scalp, or along skin folds and nails

The bumps may improve and then return over time.

Diagnosis

You will be asked about your symptoms and past health. A physical exam will be done.

The affected skin tissue will be examined. A biopsy may be done. A small sample of the area will be removed. It will be sent to a lab for exam.

Skin Biopsy
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Treatment

There is no known cure for Darier disease. Treatment is aimed at decreasing symptoms.

Skin Care

Proper skin care may allow the skin to heal. Treatment may include:

  • Antiseptic soap or cream
  • Avoid exposure to heat such as hot baths and showers
  • Moisturizers
  • Sunscreen with an SPF of 30 or more
  • Loose clothing to reduce friction and decrease sweating

Medications

Medicine may be needed and may include:

  • Retinoids pills or creams to reduce symptoms
  • Corticosteroid creams to reduce itching
  • Anti-infection pills or creams

Procedures

Other treatments may be needed if skin care and medicine do not work. Options include:

  • Laser treatment—to treat thick areas of skin
  • Photodynamic therapy—light therapy
  • Surgical removal or dermabrasion of the top layer of skin

Prevention

Darier disease is caused by a genetic change. There is no known way to prevent it.

RESOURCES

Family Doctor—American Academy of Family Physicians  http://www.familydoctor.org 

National Organization for Rare Disorders  https://rarediseases.org 

CANADIAN RESOURCES

Canadian Dermatology Association  http://www.dermatology.ca 

Health Canada  http://www.hc-sc.gc.ca 

References

Darier’s disease. British Association of Dermatology website. Available at: http://www.bad.org.uk/shared/get-file.ashx?id=193&itemtype=document. Updated October 2017. Accessed January 11, 2019.

Darier disease. DermNet New Zealand website. Available at: http://www.dermnetnz.org/topics/darier-disease. Published October 2016. Accessed January 11, 2019.

Darier disease. Genetics Home Reference—US National Library of Medicine website. Available at: https://ghr.nlm.nih.gov/condition/darier-disease. Updated January 8, 2019. Accessed January 11, 2019.

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