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Pityriasis Rubra Pilaris

Last updated June 13, 2022

Reviewed by: Lester Fahrner, MD

Approved by: Krish Tangella MD, MBA, FCAP

Pityriasis Rubra Pilaris is a benign, chronic skin disorder in which the skin becomes red, swollen, rough, and dry.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Devergie’s Disease
  • Familial Pityriasis Rubra Pilaris
  • Lichen Ruber Pilaris

What is Pityriasis Rubra Pilaris? (Definition/Background Information)

  • Pityriasis Rubra Pilaris is a benign, chronic skin disorder in which the skin becomes red, swollen, rough, and dry. Reddish-orange patches appear on the skin, either throughout the body, or in selected parts such as the elbows, knees, palms, and soles
  • The condition affects males and females of all ages, though rarely, cases may be congenital in nature. It may be diagnosed through a physical examination of the skin and a skin biopsy consistent with the diagnosis
  • The treatment of Pityriasis Rubra Pilaris involves topical application of corticosteroid creams and oral medications such as methotrexate and vitamin A derivatives. The prognosis of the skin disorder varies according to the type of Pityriasis Rubra Pilaris

They are of different types of Pityriasis Rubra Pilaris, namely:

  • Classic adult type of Pityriasis Rubra Pilaris
  • Atypical adult type of Pityriasis Rubra Pilaris
  • Classic juvenile type of Pityriasis Rubra Pilaris
  • Circumscribed juvenile type of Pityriasis Rubra Pilaris
  • Atypical juvenile type of Pityriasis Rubra Pilaris
  • HIV-associated type of Pityriasis Rubra Pilaris

Who gets Pityriasis Rubra Pilaris? (Age and Sex Distribution)

  • Individuals of all ages may be affected by Pityriasis Rubra Pilaris. Sometimes, the condition may have a genetic association and it may be congenital
  • These is no gender predilection observed
  • No racial or ethnical preference is noted

What are the Risk Factors for Pityriasis Rubra Pilaris? (Predisposing Factors)

The risk factors associated with Pityriasis Rubra Pilaris include:

  • Adults over 40 years of age
  • In some cases, the disorder may be inherited at the time of birth

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pityriasis Rubra Pilaris? (Etiology)

  • The exact cause of Pityriasis Rubra Pilaris is presently not known
  • Pityriasis Rubra Pilaris is usually sporadic, but in a few cases, it may be inherited; hence, genetic factors may play a role in such cases
  • In some cases, fungal infections may be the causative factor

What are the Signs and Symptoms of Pityriasis Rubra Pilaris?

The common signs and symptoms manifested by Pityriasis Rubra Pilaris are:

  • Pink swellings (bumps) on the skin; pink scaly patches of skin
  • Development of thick skin on the palms and soles
  • Seborrhea of the scalp
  • Itching and rashes
  • Malaise: Generalized feeling of weakness, fatigue
  • Anorexia
  • Fever and chills

The lesions usually start on the face and scalp. It then proceeds to involve the palms and soles and then there is a generalized involvement of the entire skin.

How is Pityriasis Rubra Pilaris Diagnosed?

A diagnosis of Pityriasis Rubra Pilaris may be done by:

  • Physical examination of the skin along with evaluation of one’s medical history
  • Skin biopsy: An examination of a tissue sample under the microscope by a pathologist. This is required in cases where the diagnosis is in doubt and cannot be made through a physical exam alone

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pityriasis Rubra Pilaris?

The complications arising out of Pityriasis Rubra Pilaris are:

  • Hyperkeratosis: Unusual thickness of the skin
  • Thickening of the palms and soles, which is medically termed as palmoplantar keratoderma, with resultant fissures and cracking
  • Congestion of capillaries causing redness of the skin
  • Structure of nail may be affected (nail dystrophy) causing nail shedding or onycholysis (detachment of the nail from the nail bed)
  • Hair loss (alopecia)
  • Dry, itchy, tender skin with extensive distribution
  • Secondary bacterial skin infection and sepsis

How is Pityriasis Rubra Pilaris Treated?

The treatment for Pityriasis Rubra Pilaris (PRP) involves:

  • Steroidal creams and ointments for the rashes
  • Emollients to help dry and cracked skin
  • Oral medications, such as methotrexate and vitamin A derivatives (acitretin and isotretinoin), are generally used
  • lcipotriol, a vitamin D analogue has also been used, but further research is required to determine its effectiveness
  • The topical retinoid tazarotene is used effectively in controlling juvenile-circumscribed Pityriasis Rubra Pilaris
  • Infliximab is used off label (not specifically FDA-approved for this disease) in the treatment of PRP

Phototherapy is also used which includes:

  • Narrow band phototherapy
  • PUVA (Psoralen, given by mouth, with ultraviolet A light)
  • Extracorporeal photopheresis

How can Pityriasis Rubra Pilaris be Prevented?

Currently, there are no specific methods or guidelines to prevent Pityriasis Rubra Pilaris, which may sometimes be a genetic condition.

  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited genetic disorders

What is the Prognosis of Pityriasis Rubra Pilaris? (Outcomes/Resolutions)

  • The prognosis of Pityriasis Rubra Pilaris varies with the type of the skin disorder. The inherited form of the disorder may be present for a lifetime
  • The acquired form may be cured over a period from months to a few years

Additional and Relevant Useful Information for Pityriasis Rubra Pilaris:

  • Pityriasis alba is a common, benign, chronic skin disorder generally occurring in children and teenagers
  • Pityriasis rosea is a benign, skin disorder characterized by the presence of skin rashes, affecting individuals between 10-35 years of age
  • The word “pityriasis” refers to skin conditions with notable small scales as a feature

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: May 27, 2015
Last updated: June 13, 2022