Pityriasis rubra pilaris


Pityriasis rubra pilaris

Description, Causes and Risk Factors:

Abbreviation: PRP.

Pityriasis rubra pilaris is an uncommon erythematous papulosquamous disorder characterized by erythroderma, palmoplantar keratoderma and follicular hyperkeratosis. Although often exhibited at clinical meetings because of its rarity and difficulty in management, its etiology remains unknown.

The cause of PRP is unknown and has long been a subject of debate. A prominent finding is epidermal over-activity, as the thymidine-labeling index is increased from an average normal of 3-27%. These findings may, however, represent epiphenomena to a more basic defect.

Main types:

    Classical adult onset.

  • Atypical adult onset.

  • Classical juvenile onset.

  • Circumscribed juvenile onset.

  • Atypical juvenile onset.

  • HIV-associated pityriasis rubra pilaris.

Both inherited (familial) and acquired form of pityriasis rubra pilaris have been reported in the medical literature. The inherited form starts early in childhood and is persistent throughout life, whereas the acquired type may occur at any age. The acquired form usually shows a gradual remission but some patients continue to have trouble for many years. The disease is rare but no exact incidence has been reported.

In most cases of pityriasis rubra pilaris, the cause of the condition is unknown. However, mutations in the CARD14 gene have been found to cause the familial form of the disorder in a few affected families. The CARD14 gene provides instructions for making a protein that turns on (activates) a group of interacting proteins known as nuclear factor-kappa-B (NF-?B). NF-?B regulates the activity of multiple genes, including genes that control the body's immune responses and inflammatory reactions. It also protects cells from certain signals that would otherwise cause them to self-destruct (undergo apoptosis).

The CARD14 protein is found in many of the body's tissues, but it is particularly abundant in the skin. NF-?B signaling appears to play an important role in regulating inflammation in the skin. Mutations in the CARD14 gene lead to over-activation of NF-?B signaling, which triggers an abnormal inflammatory response. Researchers are working to determine how these changes lead to the specific features of familial pityriasis rubra pilaris.

The exact prevalence is unknown.

Symptoms:

Pityriasis rubra pilariscauses pink, red, or orange-red scaly patches on your skin. The patches are usually mildly itchy. You may only have the scaly patches on parts of your body. The scaly patches most often occur on the elbows, knees, hands, feet, and ankles. The skin on the palms of your hands and the soles of your feet may also become red and thick. The scaly patches may eventually spread over your entire body, including your nails and eyes.

Diagnosis:

The different types of pityriasis rubra pilaris may look like psoriasis and are often mistaken for it. Your doctor will probably refer you to a skin specialist; to confirm the diagnosis a small piece of skin can be removed under a local anaesthetic and examined under the microscope. There is no blood test for pityriasis rubra pilaris.

Treatment:

Evaluation of the treatment for pityriasis rubra pilaris has been difficult because of its natural remitting course and the lack of a standardized assessment of severity. When reading treatment studies of pityriasis rubra pilaris, one must consider the possibility of erroneous inclusion of psoriasis in the study population, unless clearly defined clinicopathological diagnostic inclusion criteria for PRP was established.

    Applications to the skin are all that is needed if pityriasis rubra pilaris is mild:

  • Steroid creams and ointments soothe but probably do not alter the rash.

  • Greasy applications (emollients), such as soft white paraffin, reduce the splitting of the thickened palms and soles.

  • Medication by mouth may be needed if the pityriasis rubra pilaris is severe. These medications should only be undertaken by a dermatologist as monitoring is necessary

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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