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Pityriasis Rubra Pilaris - Causes, Symptoms And Treatment of Pityriasis Rubra Pilaris

What is Pityriasis Rubra Pilaris ?

Pityriasis rubra pilaris is a skin disorder causing persistent general inflammation and scaling (exfoliation) of the skin. Pityriasis rubra pilaris (PRP) is the name given to a group of rare skin disorders that present with reddish-orange coloured scaling patches with well defined borders.

They may cover the entire body or just parts of the body such as the elbows and knees, palms and soles.The palms and soles are usually involved and become diffusely thickened and yellowish (palmoplantar keratoderma). Pityriasis rubra pilaris is often initially mistaken for another skin condition, usually psoriasis.

There are 5 types of Pityriasis Rubra Pilaris

• Classical adult
• Atypical adult
• Classical juvenile
• Circumscribed juvenile
• Atypical juvenile

What causes pityriasis rubra pilaris ?

The cause of pityriasis rubra pilaris is not known. It is not an infection and cannot be passed on to others. Pityriasis rubra pilaris mostly affects adults over 40, but some children are also affected. Sometimes minor burns rashes and infections seem to trigger it. There is no blood test for PRP. It is usually diagnosed when a dermatologist, suspecting the condition, does a biopsy and specifically asks it to be checked for PRP.

Sometimes Pityriasis rubra pilaris is suspected only after the usual creams, pills and even ultraviolet light treatments used for skin conditions have no effect.

What are the symptoms of pityriasis rubra pilaris ?

• PRP most often starts as a patchy rash on the scalp, face or chest.
• The rash has an orange-red color ("salmon") and the palms and soles become thickened. Rough, dry plugs can be felt within the rash.
• Thick skin on the palms and soles can split and become painful. Walking may be sore.
• The itching is usually severe at first, and then later is not as bad as you would think considering how bad the rash looks.

What does pityriasis rubra pilaris look like?

• Classical adult-onset PRP most often starts on the head, neck and upper trunk as a red scaly rash.
• The patches are dry and scaly, red with an orangey tinge, and have sharp edges. They can join together to cover large areas of skin, and occasionally people with pityriasis rubra pilaris become red all over.
• Rough, dry plugs can be felt within the patches and are due to plugged hair follicles, often most obvious on the backs of the fingers.
• The nails may become thickened and discoloured at the free nail edge and may show linear black streaks (splinter haemorrhages).

Pityriasis Rubra Pilaris Treatment

The value of treatment is difficult to assess, as the clinical course is so variable for each of the different types of PRP. In general, the inherited forms of PRP tend to persist throughout life whilst the sporadically acquired forms tend to resolve spontaneously eventually. Some the common treatment available for Pityriasis Rubra Pilaris are :-

• Greasy applications (emollients), such as soft white paraffin, reduce the splitting of the thickened palms and soles.
• Steroid creams and ointments soothe but probably do little to get rid of the rash.
• Topical steroids can help to reduce itching for the palms and soles 2% salicylic acid may help.
• The best treatment is Accutane or Soriatane pills. These are closely related "retinoid" medications. While these have many minor side effects, they do not usually cause any serious harm to the body.
• In severe cases acitretin or isotretinoin may be useful for Pityriasis Rubra Pilaris.
• Pityriasis rubra pilaris improves if it is treated with a derivative of vitamin A known as acitretin. However this carries with it a wide range of potentially serious side effects. Consult your doctor before taking any drugs.

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