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(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

This problem is a caused by a faulty gene. It cause problems with the way skin cells join together. The gene may be passed from a parent or change on its own.

Risk Factors

The risk of this problem is higher in people who have other family members with it.

Symptoms

Symptoms usually start in the teen years. Darier disease bumps:

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

The bumps may improve and then come back over time.

Diagnosis

The doctor will ask about your symptoms and health history. A physical exam will be done. It will focus on the skin. This is often enough to make the diagnosis.

The skin may also be tested. This can be done with a biopsy. A small sample of skin will be removed. It will be sent to a lab for testing.

A blood test may also be done to look for signs of the faulty gene.

Skin Biopsy
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Treatment

There is no cure. The goal of treatment is to manage symptoms. Choices are:

  • Skin care, such as using an antiseptic soap, wearing SPF 30 sunblock, and using moisturizers
  • Lifestyle changes, such as avoiding heat and wearing loose clothing
  • Medicines to control symptoms, such as:
    • Corticosteroid cream or pills
    • Retinoids cream or pills
    • Fluorouracil cream

People who are not helped by these methods may need:

  • Dermabrasion to remove damaged skin using a rotating device
  • Laser treatment to remove layers of damaged skin using a laser
  • Surgery to remove damaged skin

Prevention

There are no known ways to prevent this health problem.

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.canada.ca 

References

Darier disease. DermNet New Zealand website. Available at: http://www.dermnetnz.org/topics/darier-disease. Accessed February 15, 2021.

Darier disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/darier-disease. Accessed February 15, 2021.

Darier’s disease. British Association of Dermatology website. Available at: http://www.bad.org.uk/shared/get-file.ashx?id=193&itemtype=document. Accessed February 15, 2021.

Nellen RG, Steijlen PM, et al. Mendelian Disorders of Cornification Caused by Defects in Intracellular Calcium Pumps: Mutation Update and Database for Variants in ATP2A2 and ATP2C1 Associated with Darier Disease and Hailey-Hailey Disease. Hum Mutat. 2017 Apr;38(4):343-356.

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