Last reviewed by mvhs13 on August 13th, 2018.
What is Pityriasis Rubra Pilaris?
Pityriasis rubra pilaris is a group of rare and chronic skin disorder characterized by unvarying inflammation of the skin associated with shedding. The disease can cover the entire skin surface of the body or it can cover only some parts of the body.
Pityriasis rubra pilaris is also known by other names such as Lichen ruber acuminatus, Devergie’s disease and Lichen ruber pilaris. The identity of Pityriasis rubra pilaris became a controversy when the credit of first description and naming of the disease was given to Marie-Guillaume-Alphonse Devergie in the year 1856 although Devergie did not recognize the first case of Pityriasis rubra pilaris. The first case of the disease was first recognized in the year 1828 by Claudio Tarral but the term Pityriasis rubra pilaris was given by Besnier in the year 1889 who clearly described the disease.
Pityriasis rubra pilaris is a rare disease involving the skin which is not a life-threatening disease. On the other hand, the disease can be painful and disabling including an unappealing effect on cosmetic aspect that can affect the quality of life. It can affect people globally or without racial predilection and equally affecting both men and women. Pityriasis rubra pilaris can occur during early childhood or may occur during adulthood.
Pityriasis Rubra Pilaris Symptoms
Pityriasis rubra pilaris is characterized by the development of patches on the skin that can cover the entire skin surface or parts of the body such as in the elbows, knees, palm of the hands and soles of the feet. The patches have well-defined borders and with reddish-orange or reddish-brown discoloration with about pinhead in size. The disease is also characterized by severe flaking of the skin accompanies with itchiness and thickening bumps that grows around the hair follicle. Pityriasis rubra pilaris can cover the entire body although it often spares other parts of the body and is termed as “islands of sparing”.
Pityriasis rubra pilaris is sometimes mistaken for another skin disorder particularly with Psoriasis. The onset of symptoms however, is grouped according to the areas of development and such signs and symptoms of Pityriasis rubra pilaris include the following:
- Skin exhibit scaly patches with sharp borders with reddish-orange or reddish-brown discoloration. The patches may later extend to cover the other areas of the skin while the palm of the hands and sole of the feet may have thickening patches in yellowish color or termed as palmoplantar keratoderma. Patients with palmoplantar keratoderma often experience painful fissures and may also have pruritus.
- Nails also have changes in Pityriasis rubra pilaris. A yellowish-brown discoloration in the distal end of the nails may occur, including nail thickening and longitudinal ridging.
- Eyes may also experience changes which include changes or problems with vision or the patient may complain of a blurry vision.
- Mucus membrane also exhibits changes due to Pityriasis rubra pilaris. The patient may complain of irritation and pain in and around the mouth.
The exact cause of Pityriasis rubra pilaris remains unclear and the onset is often sporadic. There are cases where Pityriasis rubra pilaris exists as an inherited disease but most cases are not. The disease can be partially inherited where there is a 50% chance that an offspring can acquire the disease from their parents who carried the defective gene. However, not all children with parents carrying the defective gene can automatically suffer from Pityriasis rubra pilaris.
Pityriasis rubra pilaris is classified into different forms according to the onset and these include the following:
- Classic adult pityriasis rubra pilaris is the most common form which accounts for 50% of all cases. This form is often associated with underlying malignancy of the skin although this form has a good prognosis amongst other forms of Pityriasis rubra pilaris. The onset is acute and the remission is 3 years in about 80% of patients.
- Atypical adult pityriasis rubra pilaris accounts for about 5% of all cases of the disease. Patients with this form often suffer from eczematous component and atypical adult pityriasis rubra pilaris has a long duration usually about 20 years.
- Classic juvenile pityriasis rubra pilaris occurs within the first two years of life and with remission that takes place faster than the classic adult type usually within a year. Classic juvenile pityriasis rubra pilaris is believed to occur following an incidence of acute infection.
- Circumscribed pityriasis rubra pilaris accounts for about 25% of pityriasis rubra pilaris cases and occurs in children before reaching the puberty stage. The skin patches are often confined in the palm of the hands, sole of the feet, elbows and knees. The long term outcome of this type of pityriasis rubra pilaris is unclear but the disease usually improves during the late teens.
- Atypical juvenile pityriasis rubra pilaris usually occurs at birth or during the early childhood and is sometimes acquired in an inherited pattern and is chronic. It often affects the palm of the hands and the sole of the feet.
- HIV-associated pityriasis rubra pilaris occurs in patients suffering from HIV. This type of pityriasis rubra pilaris is resistant to standard treatment but is often responsive in antiretroviral therapy.
Pityriasis rubra pilaris has no specific laboratory test to confirm the disease. The disease is often mistaken for Psoriasis although the difference is that Psoriasis is treated easily and often successful whereas pityriasis rubra pilaris often fails to respond to treatment or is rather difficult to treat. Confirmation of diagnosis of pityriasis rubra pilaris is usually based on the relationship between the histologic findings and clinical findings. A skin biopsy is the usual diagnostic procedure used to determine the onset of the disease. A small sample of the skin is usually taken to be examined under the microscope.
Histologic findings are normally evaluated to rule out other skin disorders similar to pityriasis rubra pilaris. The suspicion for pityriasis rubra pilaris is sometimes raised when the skin disease fails to respond to standard treatment such as application of creams and ointments, pills and ultraviolet light therapy. A skin biopsy is specifically asked to be checked for evidence of pityriasis rubra pilaris.
Pityriasis rubra pilaris has no specific treatment and is instead geared towards the relief of symptoms. Treatment is rather complicated due to the many types or forms of the disease. The treatment is then based on the type of pityriasis rubra pilaris including the age and overall health condition of the patient. Pityriasis rubra pilaris generally persist all throughout life while those that exist sporadically generally resolve over a period of time.
Topical medications proved to be helpful in the relief of symptoms such as dryness of the skin. Emollients such as white soft paraffin can be used to relieve the fissures in the palm of the hands and sole of feet. Other treatment for pityriasis rubra pilaris includes pills such as Accutane or Soriatane which are found to be effective. Severe cases of pityriasis rubra pilaris are usually treated with acitretin or isotretinoin.
Is Pityriasis Rubra Pilaris Contagious?
Pityriasis rubra pilaris is not a contagious disease neither it is an infectious disease. It is therefore cannot be acquired through close contact with a person afflicted with the disease. The disease is not a serious disease that can be potentially life threatening although the feature of the disease can be disturbing and unappealing in terms of the cosmetic aspect.