Definition

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

Causes

Darier disease is a genetic disorder. The faulty genes disrupt the way skin cells join together. The gene can be from either parent or the result of a gene change.

Risk Factors

Having a family member with Darier disease increases the risk of inheriting this condition.

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.

The bumps may appear on the chest, back, forehead, and scalp. They may also appear along skin folds. The fingernails may also be affected.

The bumps may improve and then return over time.

Diagnosis

You will be asked about your symptoms and medical history. A physical exam will be done.

The affected skin tissue will need to be examined. A biopsy, looking at a small sample under a microscope, may be needed.

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:

  • Using an antiseptic soap or cream
  • Avoiding too much heat, including hot baths and showers
  • Applying gentle moisturizer
  • Wearing sunscreen with an SPF of 30 or more to protect the skin
  • Wearing loose clothing to reduce friction and avoid sweating
  • Treating skin infections right away

Medications

Medications may be needed and may include:

  • Oral or topical retinoids to reduce symptoms
  • Corticosteroid creams to reduce itching
  • If a skin infection is also present, oral or topical anti-infection treatments may also be given.

Procedures

If skin care and medications are not effective, other treatments may be used. These may include:

  • Laser treatment to treat thick areas of skin
  • Photodynamic therapy—the use of a special medication on the skin to make it sensitive to light therapy
  • Surgical excision or dermabrasion of the top layer of skin

Prevention

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

Genetic counseling may be useful if you are planning to have a child and you have Darier disease or a family history of Darier disease. The counselor can let you know the risk your child may have of developing it.

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