What is Pityriasis Rubra Pilaris: Types, Causes, Symptoms, Treatment, Prevention

What is Pityriasis Rubra Pilaris?

Pityriasis rubra pilaris is a type of skin condition that causes persistent inflammation and shedding or scaling of the skin. (1,2) The condition was first described by Tarral in 1828 and then named by Besnier in 1889. (3,4) The condition causes thickening of the nails, shedding of hair and skin, and inflammation of the skin. The name of the disorder stands for pityriasis (scaling), rubra (redness), and pilaris (involvement of the hair follicles). (5,6)

Usually, Pityriasis Rubra Pilaris starts off as a small spot that appears somewhere on the face and then spreads to the back, followed by the rest of the body. (7) Pityriasis Rubra Pilaris is known to affect different parts of the body in different ways and can even affect a person for an unpredictable amount of time. The inflammation also may cover either the entire body or just certain parts of the body, especially the elbows, knees, soles, and palms. (8) Pityriasis Rubra Pilaris may progress and leave certain distinct parts of uninvolved skin, known as skip areas or ‘islands of sparing.’

Pityriasis Rubra Pilaris typically affects people in the first, sixth, and seventh decades of life and tends to commonly impact adults. However, there is also a significant number of children who get affected by Pityriasis Rubra Pilaris. Both men and women are equally affected by this disease. (9)

Types of Pityriasis Rubra Pilaris

There are six different types of pityriasis rubra pilaris, each one with its own set of symptoms and characteristics. Let us look at the different types of the disorder:

Classical Adult-Onset Pityriasis Rubra Pilaris: This is the most common type of pityriasis rubra pilaris. It develops in adulthood, and the symptoms tend to disappear within a few years. In some rare cases, though, the symptoms can come back at a later stage. Studies have found that this type of pityriasis rubra pilaris tends to resolve within a period of three years, though it may last for longer as well. (10)

Atypical Adult-Onset Pityriasis Rubra Pilaris: This type of disorder is also known to start in adulthood. However, the symptoms are known to last for over 20 years. (11)

Classical Juvenile Onset Pityriasis Rubra Pilaris: This form of pityriasis rubra pilaris begins in childhood, and the symptoms usually go away within a year. However, they may come back later. (12)

Circumscribed Juvenile Onset Pityriasis Rubra Pilaris: This disorder also begins in childhood, just before the child reaches puberty. It is known to commonly affect the soles of children’s feet, palms of their hands, the elbows, and the knees. The symptoms are likely to go away during the teenage years.

Atypical Juvenile Onset Pityriasis Rubra Pilaris: This condition is sometimes a genetic condition and passed on through the family. It can develop during early childhood, but it can also be present at birth. The symptoms of this type of disorder ten to last for life.

HIV-associated Pityriasis Rubra Pilaris: This type of pityriasis rubra pilaris is associated with the human immunodeficiency virus (HIV) and is very difficult to treat. (13,14)

What are the Causes of Pityriasis Rubra Pilaris?

The exact underlying cause of pityriasis rubra pilaris remains unknown. However, it is believed that a combination of genetic predisposition, environmental factors, and other unknown causes play a significant role in determining who develops the disease. In many cases, pityriasis rubra pilaris simply occurs for no reason. Most cases of pityriasis rubra pilaris are also not inherited, but inherited pityriasis rubra pilaris has been found to be more severe.

Classical adult-onset pityriasis rubra pilaris is believed to be linked with underlying skin cancer, though how often skin cancer occurs with this type of pityriasis rubra pilaris is not known. However, if you are diagnosed with classical adult-onset pityriasis rubra pilaris, it is common for doctors to also check for skin cancer. (15)

According to early research by the National Organization for Rare Disorders, pityriasis rubra pilaris could occur because of a problem in the manner in which the body processes vitamin A. However, more research is still needed to firmly establish this theory. (16) It was earlier also believed that a deficiency of vitamin A could be related to Pityriasis Rubra Pilaris, but the theory continues to lack sufficient evidence. At the same time, treatment of people with pityriasis rubra pilaris with vitamin A has not been effective. (17)

The Genetic and Rare Diseases Information Center believes that pityriasis rubra pilaris could also be associated with an immune system response. Again, more research is needed to confirm this. (18)

What are the Symptoms of Pityriasis Rubra Pilaris?

The symptoms of pityriasis rubra pilaris can vary from person to person. However, Pityriasis Rubra Pilaris typically causes red, pink, or orangish-red scaly patches to appear on your skin. These patches are commonly itchy, and you may develop these scaly patches either only on some parts of the body or throughout the body. The patches most commonly tend to affect the:

  • Knees
  • Elbows
  • Feet
  • Hands
  • Ankles

The skin of the palms of your hands and the soles of your feet are also affected and may turn red and become thickened. In some forms of Pityriasis Rubra Pilaris, it is possible for the scaly patches to eventually spread all over the body.

It is essential to notice the pre-onset signs of pityriasis rubra pilaris, which usually begin with dandruff and a crusty scalp. There can also be a limited number of red patches or scaling of the skin. For example, you may develop a dime-sized red spot on your forehead or another part of the body. The duration of pre-onset symptoms varies from person to person. At some time, though, you will need to get it checked by a doctor. This usually happens if the red spot doubles in size within a period of less than two weeks.

Depending on how quickly the inflammation advances, a doctor will see symptoms that include red, pink, or orangish-red scaly patches on the skin. These patches are commonly itchy, and initially, these scaly patches only appear on some parts of the body. These scaly patches typically appear on the knees, elbows, hands, feet, and ankles, while the skin of the soles and palms may also turn red or waxy. The skin may also become thickened and take on a classic orangish hue. Over time, the scaly patches will spread throughout the body. (19)

In places where the skin has thickened, cracks may develop, which can eventually become painful and make it difficult to walk and use one’s hands. Nails may also become thickened, or they may be discolored, ridged, cluttered with debris underneath the free edge, and may start shedding. (20) Hair may also begin to shed drastically, either due to the disease itself or due to some of the treatments that are prescribed. Protein loss, fluid imbalance, and heat intolerance can also occur when the rash starts to spread throughout the body.

How Does Pityriasis Rubra Pilaris Get Inherited?

As mentioned above, pityriasis rubra pilaris can be inherited if it runs in your family. You may inherit this disease if one of your parents passes the gene that causes this condition to you. It is also possible that your parent is a carrier of the gene, meaning they have the gene, but they do not have the disease. If one of your parents is a carrier of the pityriasis rubra pilaris gene, there is a 50 percent chance that you will also inherit the same gene. However, it is possible that you may not go on to develop pityriasis rubra pilaris even if you have inherited the gene. The gene associated with pityriasis rubra pilaris is the CARD14 gene, and mutations caused in this gene are believed to determine who develops the disease. (21,22,23,24)

How is Pityriasis Rubra Pilaris Diagnosed?

Pityriasis rubra pilaris is often misdiagnosed for other more commonly occurring skin conditions like psoriasis. It can sometimes also be misdiagnosed as less common diseases, including pityriasis rosea and lichen planus. Psoriasis is characterized by scaly, itchy patches of skin that are usually red. However, unlike pityriasis rubra pilaris, psoriasis is more easily diagnosed and successfully treated. Pityriasis rubra pilaris, though, might not get diagnosed until the scaly, itchy patches fail to get better or respond to psoriasis treatment. (25)

If your doctor suspects that you have pityriasis rubra pilaris, they are likely to do a skin biopsy in order to make a confirmed biopsy. (26,27)

Treatment of Pityriasis Rubra Pilaris

There is no cure for pityriasis rubra pilaris, but there are several treatments that can help you manage the symptoms. Your doctor is likely to prescribe or more of these treatments:

  • Topical Creams That Contain Lactic Acid Or Urea: These ointments are applied directly to the skin.
  • Oral Retinoids: Common examples include acitretin or isotretinoin, which are derivatives of vitamin A. This helps slow down the growth and shedding of the skin cells.
  • Oral Vitamin A: This treatment can help some people, but only when prescribed in very high dosage. Retinoids are known to be more effective and also more frequently used than vitamin A.
  • Immunosuppressants: These are oral medications that work by suppressing the immune system. Azathioprine and cyclosporine are prime examples of immunosuppressants used for treating pityriasis rubra pilaris.
  • Methotrexate: This type of oral drug that may be used if treatment with retinoids doesn’t work.
  • Biologics: Biologics care intravenous or injectable medications that have an impact on your immune system. Biologics used for treating pityriasis rubra pilaris include etanercept, adalimumab, and infliximab.
  • Ultraviolet Light Therapy: This is commonly prescribed in combination with a drug that makes you less sensitive to the sun, known as psoralen, and a retinoid. (28)

Are There Any Complications of Pityriasis Rubra Pilaris?

Pityriasis rubra pilaris is an itchy and uncomfortable condition. The symptoms of the disease can decrease over time, even if the rash looks like it is getting worse. Pityriasis rubra pilaris is not usually known to cause many complications.

However, the rash associated with pityriasis rubra pilaris can sometimes cause other issues like ectropion. (29) Extropian is a condition in which the eyelid turns out, leaving the surface of the eye exposed. Pityriasis rubra pilaris can sometimes also cause problems with the mouth’s lining, leading to pain and irritation.

Over a period of time, pityriasis rubra pilaris can even lead to keratoderma. This condition causes the soles of the feet and skin of the hands to become very thick, forming deep cracks in the skin known as fissures.

Some people with pityriasis rubra pilaris become sensitive to light and also have trouble in controlling their body temperature or sweating when it’s hot.

Is It Possible To Prevent Pityriasis Rubra Pilaris?

It is not possible to prevent this condition because both the onset and cause are unknown. If you suspect that you have pityriasis rubra pilaris, you should consult your doctor. Starting a treatment that works for you at the earliest will help you get relief from your symptoms.

Remember that finding the proper treatment that works for you may also take some time, and it is a process of trial and error. It is also important to find an effective treatment because many people end up developing more than one type of pityriasis rubra pilaris over the course of the disorder.

Conclusion

Depending on the type of pityriasis rubra pilaris you have, your symptoms may go away, or they may continue to persist. If you have been diagnosed with classical adult-onset pityriasis rubra pilaris, your symptoms are likely to last for a couple of years or less and then probably not return.

The symptoms of the other types of pityriasis rubra pilaris tend to be more long-lasting, though the proper treatment can make the symptoms manageable and less noticeable.

Pityriasis rubra pilaris is a very rare type of skin disease that is characterized by persistent inflammation and shedding of the skin, hair, and even nails. It may affect just some parts of the body or the entire body. The disease onset can also be sudden, meaning it can start at any time in your life. Although there is no cure for the disease, various treatments can help alleviate the symptoms, giving you a good quality of life.

There are several types of treatment for pityriasis rubra pilaris that include oral, topical, and injectables. Ultraviolet light therapy can also help in some cases. The earlier your condition is diagnosed, the faster you can start the treatment to help get relief from your symptoms.

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