by EBSCO Medical Review Board
(Keratosis Follicularis)


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


This is a caused by an issue with a gene. That issue causes problems with how skin cells join together. The gene may be passed from a parent or change on its own.

Risk Factors

The risk of Darier disease is higher in people with family members who have it.


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 go away 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 gene problem.

Skin Biopsy
Copyright © Nucleus Medical Media, Inc.


There is no cure. The goal of treatment is to manage symptoms. This may be done with:

  • 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—removes damaged skin with a rotating device
  • Laser treatment—removes layers of damaged skin with a laser
  • Surgery—removes damaged skin


Darier disease cannot be prevented.


Family Doctor—American Academy of Family Physicians 

National Organization for Rare Disorders 


Canadian Dermatology Association 

Health Canada 


Darier disease. DermNet New Zealand website. Available at: Accessed May 2, 2022.

Darier disease. EBSCO DynaMed website. Available at: Accessed May 2, 2022.

Darier disease. British Association of Dermatology website. Available at: Accessed May 2, 2022.

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; 38 (4): 343-356.

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