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Genetic and Rare Diseases Information Center (GARD)

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Pityriasis rubra pilaris


Other Names for this Disease
  • Familial pityriasis rubra pilaris
  • Pityriasis rubra pilaris--familial type
  • PRP
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Overview



What is pityriasis rubra pilaris?

What are the signs and symptoms of pityriasis rubra pilaris?

What causes pityriasis rubra pilaris?

How might pityriasis rubra pilaris be treated?


What is pityriasis rubra pilaris?

Pityriasis rubra pilaris (PRP) refers to a group of skin conditions that cause constant inflammation and scaling of the skin.[1] Affected people have reddish-orange colored patches; they may occur everywhere on the body or only on certain areas.[2] There are several types of PRP, which are classified based on age of onset, body areas affected, and whether other associated conditions are present.[2][3] PRP is usually sporadic (occurring randomly) but some forms may be inherited.[2]
Last updated: 3/10/2014

What are the signs and symptoms of pityriasis rubra pilaris?

Features of this condition vary greatly between affected individuals. The onset is gradual in the familial type and can be more rapid in the acquired type. Redness and scaling of the face and scalp are often seen first, followed by redness and thickening of the palms and soles.[4] Overall, the elbows, knees, backs of the hands and feet, and ankles are most commonly affected.[5] A more widespread eruption consisting of scaling orange-red plaques can be observed on the trunk and extremities. The lesions may expand and coalesce and eventually cover the entire body. When the disease becomes widespread, the nails, mucous membranes and eyes may be affected.[4] The familial type often persists throughout life, but the acquired form may have periods of remission (periods of time where symptoms improve or completely resolve).[4]
Last updated: 9/20/2010

What causes pityriasis rubra pilaris?

In most cases, pityriasis rubra pilaris (PRP) occurs sporadically for unknown reasons.[2] In a few families with the inherited form, familial PRP, the condition is caused by mutations in the CARD14 gene. This gene gives instructions for making a protein that turns on other proteins that regulate genes that control the body's immune responses and inflammatory reactions. It also protects cells from certain signals that would otherwise cause them to self-destruct. The CARD14 protein is particularly abundant in the skin. Mutations in the gene can cause a person to have an abnormal inflammatory response. Researchers are trying to find out how these mutations cause the specific features of familial PRP.[6]
Last updated: 3/10/2014

How might pityriasis rubra pilaris be treated?

Treatment of pityriasis rubra pilaris (PRP) is mainly based on reports of patients' experiences. No controlled trials have been done, so the effectiveness and safety of treatments is unclear. Currently there are no treatments approved by the US Food and Drug Administration (FDA) or the European Medicines Agency (EMA) for use in PRP.[7]

Management of PRP often involves systemic and topical therapies combined. Topical therapies can help with the symptoms and may be enough for people with mild PRP. Topical treatments are usually combined with systemic therapy for PRP that affects a large part of the body. Most people need systemic therapy to control the condition.[7]

Oral retinoids (synthetic vitamin A derivatives) are usually preferred as a first-line systemic treatment for PRP. Methotrexate may be an alternative option for people who should not use systemic retinoids, or who don't respond to systemic retinoid therapy. For people who don't respond well to retinoid or methotrexate therapy, options may include biologic TNF-alpha inhibitors, azathioprine, cyclosporine, and/or phototherapy. Topical treatments used for PRP may include topical corticosteroids, keratolytics, tar, calcipotriol, topical tretinoin, and tazarotene.[7]

Some of the medications used to treat PRP can harm a developing fetus and are not recommended for use right before or during pregnancy.[7] People seeking information about specific treatment options for themselves or family members should speak with their health care provider.
Last updated: 3/10/2014

References
  1. Pityriasis rubra pilaris. MedlinePlus. April 2009; http://www.nlm.nih.gov/medlineplus/ency/article/001471.htm. Accessed 6/30/2011.
  2. Vanessa Ngan. Pityriasis rubra pilaris. DermNet NZ. December 29, 2013; http://www.dermnetnz.org/scaly/pityriasis-rubra-pilaris.html. Accessed 3/10/2014.
  3. Shenefelt PD. Pityriasis Rubra Pilaris. eMedicine. May 2010; http://emedicine.medscape.com/article/1107742-overview. Accessed 6/30/2011.
  4. Goldsmith LA, Baden HP. Pityriasis Rubra Pilaris. In: Freedberg, et al., ed. Fitzpatrick's Dermatology in General Medicine, 6th edition. New York, NY: McGraw-Hill; 2003;
  5. Sybert VP. Pityriasis Rubra Pilaris. Genetic Skin Disorders. New York, NY: Oxford University Press; 1997;
  6. Familial Pityriasis Rubra Pilaris. Genetics Home Reference. March 2013; http://ghr.nlm.nih.gov/condition/familial-pityriasis-rubra-pilaris. Accessed 3/10/2014.
  7. Andreas Katsambas and Clio Dessinioti. Pityriasis rubra pilaris. UpToDate. Waltham, MA: UpToDate; March, 2014; Accessed 3/10/2014.