Pityriasis Rubra Pilaris

Written by Rose Kivi | Published on July 10, 2012
Medically Reviewed by George Krucik, MD

What Is Pityriasis Rubra Pilaris?

Pityriasis Rubra Pilaris (PRD) is a rare skin disorder that causes constant inflammation and shedding of the skin. PRD can affect parts of your body or your entire body. The disorder may begin in childhood or adulthood and affects males and females equally.

PRD is not dangerous, although the disorder may be cosmetically unappealing. It can also cause mild physical discomfort.

Types of PRD

There are six types of PRD.

Classical Adult Onset

The most common type of PRD, this type begins in adulthood. The symptoms usually go away after a few years. In rare cases, the symptoms come back later.

Atypical Adult Onset

This type also begins in adulthood, but the symptoms may last for more than 20 years.

Classical Juvenile Onset

This form begins in childhood. The symptoms usually go away within a year, but may return later.

Circumscribed Juvenile Onset

This type of PRD begins in children before they reach puberty. It only affects the palms of the hands, soles of the feet, knees, and elbows. The symptoms may go away during teenage years.

Atypical Juvenile Onset

This form of PRD is sometimes inherited. It is present at birth or may develop during early childhood. The symptoms often last indefinitely.

HIV-Associated PRD

This form of PRD is associated with HIV infection and is very difficult to treat.

What Causes PRD?

The exact cause of PRD is unknown. Some cases of PRD are inherited, but most are not. Inherited PRD tends to be more severe. PRD most commonly occurs spontaneously for no discernable reason.

Classical adult onset PRD may be associated with an underlying skin cancer. However, the rate of occurrence of skin cancer with classical adult onset PRD is unknown. If you have classical onset PRD, have your doctor check you for skin cancer to be safe.

According to the National Organization for Rare Disorders (NORD), early research suggests that PRD may occur due to an abnormality in the way the body processes vitamin A. However, more research needs to be conducted to determine if this is true (NORD, 2012). There is also thought that PRD is connected to an atypical immune system response (GARD, 2010).

How Is PRD Inherited?

You may inherit PRD if one of your parents passes down the defective gene that causes the disorder. Your parent may be a carrier of the defective gene, but not actually have the disorder. If one of your parents is a carrier of the defective gene, there is a 50 percent chance that the gene will be passed to you. However, you may not develop PRD if you inherit the defective gene.

What Are the Symptoms of PRD?

PRD causes pink, red, or orange-red scaly patches on your skin. The patches are usually mildly itchy. You may only have the scaly patches on parts of your body. The scaly patches most often occur on the elbows, knees, hands, feet, and ankles. The skin on the palms of your hands and the soles of your feet may also become red and thick. The scaly patches may eventually spread over your entire body, including your nails and eyes.

How Is PRD Diagnosed?

PRD is often mistaken for other more common skin conditions, such as psoriasis. Psoriasis is another skin condition that is characterized by scaly patches typically red in color and itchy skin. However, unlike PRD, psoriasis can be more easily and successfully treated. Sometimes, it is not until the scaly patches fail to respond to treatment that PRD is considered.

If your doctor suspects PRD, he or she may perform a skin biopsy to make a diagnosis. A skin biopsy is a medical procedure in which a small sample of skin is removed so that the doctor can examine it under a microscope.

How Is PRD Treated?

There is no cure for PRD. Treatment consists of relieving your symptoms. Your doctor may prescribe one or more of the following treatments to relieve symptoms:

  • topical creams that contain urea or lactic acid
  • oral vitamin A—effectively reduces or relieves symptoms in one-third of patients, according to ORDR (ORDR, 2012)
  • oral retinoids like isotretinoin or acitretin—retinoids are medications that slow the growth and shedding of skin cells
  • ultraviolet light therapy—usually prescribed in combination with Psoralen (a medication that makes you less sensitive to the sun) and a retinoid

Will PRD Go Away?

Depending on the type of PRD you have, your symptoms may or may not go away. If you have classical adult onset PRD (the most common form of the disorder), your symptoms will most likely last only a few years and then never return.

Treatments may make symptoms less noticeable.

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