Pityriasis rubra pilaris

What causes pityriasis rubra pilaris?

In the vast majority of people with PRP, the cause is unknown. Most cases are thought to result from a combination of unknown genetic factors and environmental triggers.

Rarely, PRP is due to changes (mutations) in a gene called CARD14. Mutations in this gene are found particularly in people with PRP type V. This gene gives the body instructions for making a protein involved in regulating the body's immune responses and inflammatory reactions. While the protein is present in many of the body’s tissues, it is found in great amounts in the skin. Mutations in this gene are thought to trigger an abnormal inflammatory response, leading to the signs and symptoms of PRP.

Last updated on 05-01-20

What is the long term outlook for people with pityriasis rubra pilaris?

The severity and course of the condition varies depending on the type of PRP a person has. PRP may go away on its own, have periods of remission (when symptoms improve or go away), improve over time, or it may be chronic (long- lasting). In some cases, the condition goes away and then returns (relapses) after therapy is stopped. The following is a general overview of what to expect with each type:

  • Type I (classic adult type), the most common type, goes away on its own within 3 years in about 80% of people. After it goes away, relapses are uncommon.
  • Type II (atypical adult type) can last for a very long time, sometimes more than 20 years.
  • Type III (classic juvenile type) usually goes away within one year. Rarely, this type persists for a longer period of time.
  • Type IV (circumscribed juvenile) may be associated with alternating periods of getting better and worse. About one-third of people with this type have improvement with age.
  • Type V (atypical juvenile type) is usually chronic.
  • Type VI (HIV-associated) tends to be resistant to most treatments.

PRP can significantly affect quality of life, especially if there is ongoing pain, itching, or sleep problems. People with PRP have a normal life expectancy.

Last updated on 05-01-20

How might pityriasis rubra pilaris be treated?

PRP can be difficult to treat and many treatment options have been tried. There is little information on which treatments are best because none have been studied in large clinical trials. Treatment options may vary based on the symptoms in each person but often involve a combination of medicines taken internally and applied topically to the skin.

Topical therapy options may include corticosteroids, keratolytics, calcipotriol, tretinoin, and tazarotene. For people with mild PRP, using one or more of these may be enough to control symptoms.

Most people with PRP also need additional medicines to control their symptoms, especially if the condition affects a large part of the body. Oral retinoids are usually tried first. Examples include acitretin and isotretinoin. Methotrexate, an immune system suppressant, may be tried when oral retinoids are not safe for a particular person, or when they have stopped working. Other immune system suppressants that have been helpful in some case reports include cyclosporine and azathioprine. Biologic therapies may be another option, based on limited data. Examples include various biologic TNF-alpha inhibitors and ustekinumab.

For more detailed information about treatment options for PRP, you can view information from Medscape Reference or the National Organization for Rare Disorders (NORD).

Many of these treatments have shown results in only a few patients, and some can have serious side effects. The risks and benefits of each treatment option should be discussed with your doctor.

Last updated on 05-01-20

Clinical Research Resources

PRP Alliance

The PRP Alliance includes information on current research for pityriasis rubra pilaris within the PRP Survival Guide.

Last updated on 04-27-20

Social Networking Websites

RareConnect

RareConnect, an online community partnered with EURORDIS, an international patient organization, has a Pityriasis rubra pilaris community

Last updated on 04-27-20

Name: Foundation for Ichthyosis and Related Skin Types FIRST 2616 North Broad Street
Colmar, PA, 18915 , United States
Phone: +1-215-997-9400 Toll Free: 1-800-545-3286 Email: info@firstskinfoundation.org Url: http://www.firstskinfoundation.org/
Name: PRP Alliance 1500 Commerce Drive
Plano, TX, 75093-2640,
Phone: 214-205-0574 Url: http://prpalliance.org

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