Darier disease is a rare disorder in which thick, rough bumps appear on the skin. It is also known as keratosis follicularis.
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.
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.
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.
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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
Family Doctor—American Academy of Family Physicians http://www.familydoctor.org
National Organization for Rare Disorders https://rarediseases.org
Canadian Dermatology Association http://www.dermatology.ca
Health Canada http://www.canada.ca
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.
- Reviewer: EBSCO Medical Review Board James P. Cornell, MD
- Review Date: 12/2020
- Update Date: 02/15/2021