What is the definition of Pityriasis Rubra Pilaris?

Pityriasis rubra pilaris (PRP) is a rare skin disorder that causes inflammation and scaling (exfoliation) of the skin.

What are the alternative names for Pityriasis Rubra Pilaris?

PRP; Pityriasis pilaris; Lichen ruber acuminatus; Devergie disease

What are the causes for Pityriasis Rubra Pilaris?

There are several subtypes of PRP. The cause is unknown, although genetic factors and an abnormal immune response may be involved. One subtype is associated with HIV/AIDS.

What are the symptoms for Pityriasis Rubra Pilaris?

PRP is a chronic skin condition in which orange or salmon-colored scaly patches with thick skin develop on the hands and feet.

The scaly areas may cover much of the body. Small islands of normal skin (called islands of sparing) are seen within the areas of the scaly skin. The scaly areas may be itchy. There may be changes in the nails.

PRP can be severe. Although it's not life threatening, PRP can greatly reduce quality of life and limit activities of daily living.

What are the current treatments for Pityriasis Rubra Pilaris?

Topical creams containing urea, lactic acid, retinoids, and steroids may help. More commonly, treatment includes pills taken by mouth such as isotretinoin, acitretin, or methotrexate. Exposure to ultraviolet light (light therapy) may also help. Medicines that affect the body's immune system are currently being studied and may be effective for PRP.

What are the support groups for Pityriasis Rubra Pilaris?

This resource can provide more information on PRP:

  • National Organization for Rare Disorders -- rarediseases.org/rare-diseases/pityriasis-rubra-pilaris

When should I contact a medical professional for Pityriasis Rubra Pilaris?

Call your provider if you develop symptoms of PRP. Also call if you have the disorder and symptoms worsen.

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REFERENCES

James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM. Pityriasis rosea, pityriasis rubra pilaris, and other papulosquamous and hyperkeratotic diseases. In: James WD, Elston DM, Treat JR, Rosenbach MA, Neuhaus IM, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 13th ed. Philadelphia, PA: Elsevier; 2020:chap 11.

Patterson JW. Disorders of pigmentation. In: Patterson JW, ed. Weedon's Skin Pathology. 4th ed. Philadelphia, PA: Elsevier Churchill Livingstone; 2016:chap 10.

  • Condition: Pityriasis Rubra Pilaris Type 1
  • Journal: European journal of dermatology : EJD
  • Treatment Used: First-Line Retinoid or Second-Line Retinoid plus Methotrexate
  • Number of Patients: 0
  • Published —
This study investigated the best treatment for patients with pityriasis rubra pilaris type 1.
Clinical Trial
  • Status: Recruiting
  • Phase: Phase 2
  • Intervention Type: Biological
  • Participants: 15
  • Start Date: October 3, 2019
An Open Label Pilot Trial of Guselkumab in the Treatment of Adults With Pityriasis Rubra Pilaris (PRP)
Clinical Trial
  • Status: Enrolling by invitation
  • Phase: Phase 1
  • Intervention Type: Drug
  • Participants: 12
  • Start Date: April 1, 2018
Cosentyx (Secukinumab) for the Treatment of Adult Onset Pityriasis Rubra Pilaris: A Single Arm, Open-label Exploratory Trial