Pityriasis Rubra Pilaris - Medtick
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Pityriasis Rubra Pilaris

What is it?

A rare skin disease that causes constant inflammation and shedding of the skin.

  • 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.

PRP types

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.

Cause

  • Unknown
  • Family history
  • May be problem in the way the body processes vitamin A
  • Mutations in the CARD14 gene found in many body tissues

Symptoms

  • Redness and scaling of the skin and scalp, which often develops into itchy, orange-red plaques?
  • Plaques may first occur on only some parts of the body, but may eventually spread over the whole body (the elbows, knees, ankles, hands and feet) are most commonly affected?
  • Thinning of the hair?
  • Plaques and irritation in the mouth?
  • Dryness of the eyes and/or ectropion (outward turning of the eyelid)?
  • Thickening of the skin on the palms and soles?
  • Reduced quality of life associated with persistent pain, itching, or sleep disturbances?

Nail changes:

rarediseases.info.nih.gov

Complications /Information to beware of/General tips:

This condition can occur with:

This condition may show similar symptoms to:

Please talk to your healthcare professional (i.e. Medical Doctor/Pharmacist) for further advice

Detailed Information

Please copy and paste any key words from the title: Pityriasis Rubra Pilaris in the following respective 'Medtick References and/or Sources' to find out more about the disease (this also may include diagnosis tests and generic medical treatments).

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  • National Organisation of Rare Diseases

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