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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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What causes pityriasis rubra pilaris?

In most cases, pityriasis rubra pilaris (PRP) occurs sporadically for unknown reasons.[1] 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.[2]
Last updated: 3/10/2014

  1. Vanessa Ngan. Pityriasis rubra pilaris. DermNet NZ. December 29, 2013; Accessed 3/10/2014.
  2. Familial Pityriasis Rubra Pilaris. Genetics Home Reference. March 2013; Accessed 3/10/2014.