What Is Pityriasis Rubra Pilaris?
Pityriasis Rubra Pilaris (PRP) is a rare skin disorder that causes constant inflammation and shedding of the skin. PRP can affect parts of your body or your entire body. The disorder may begin in childhood or adulthood and affects males and females equally.
PRP is not dangerous, although the disorder may be cosmetically unappealing. It can also cause mild physical discomfort.
Types of PRP
There are six types of PRP.
Classical Adult Onset
The most common type of PRP, 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 PRP 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 PRP is sometimes inherited. It is present at birth or may develop during early childhood. The symptoms often last indefinitely.
This form of PRP is associated with HIV infection and is very difficult to treat.
What Causes PRP?
The exact cause of PRP is unknown. Some cases of PRP are inherited, but most are not. Inherited PRP tends to be more severe. PRP most commonly occurs spontaneously for no discernable reason.
Classical adult onset PRP may be associated with an underlying skin cancer. However, the rate of occurrence of skin cancer with classical adult onset PRP is unknown. If you have classical onset PRP, have your doctor check you for skin cancer to be safe.
According to the National Organization for Rare Disorders (NORD), early research suggests that PRP 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 PRP is connected to an atypical immune system response (GARD, 2010).
How Is PRP Inherited?
You may inherit PRP 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 PRP if you inherit the defective gene.
What Are the Symptoms of PRP?
PRP 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 PRP Diagnosed?
PRP 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 PRP, psoriasis can be more easily and successfully treated. Sometimes, it is not until the scaly patches fail to respond to treatment that PRP is considered.
If your doctor suspects PRP, 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 PRP Treated?
There is no cure for PRP. 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 PRP Go Away?
Depending on the type of PRP you have, your symptoms may or may not go away. If you have classical adult onset PRP (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.