Pityriasis Rubra Pilaris - Causes, Symptoms and Treatment

Definition

Pityriasis rubra pilaris is a mildly itchy chronic skin disorder characterized by the association of palmoplantar keratoderma, follicular plugging and erythematous perifollicular papules which may leads to plaques or erythroderma and is usually present with reddish-orange coloured scaling patches with well defined borders. It may cause persistent general inflammation and scaling of the skin. The disease may affect entire body or just restrict to some parts such as the elbows and knees, palms and soles. The disease often has a sudden onset.

The Disease generally produces elevated spots on the skin. These spots grow and become connected, producing red plaques over large areas containing dry plugged pores .It is found to be having association with abnormal metabolism of vitamin A. It could take as long as one to three years for an individual to fully recover from this skin disorder. It may occur in any race, and both male and female are equally affected.

Types of Pityriasis Rubra Pilaris:-

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

Symptoms:-

The possible symptoms of pityriasis rubra pilaris are as:-

• Pityriasis Rubra Pilaris usually starts as a patchy rash on the scalp, face or chest and then multiple patches appear and join together.
• The rash can be itchy in its early stages.
• The rash is of orange-red color and the the palms and soles get thickened.
• Shivering may occur if the rash is angry and widespread and loses a great deal of heat.
• Rough, dry plugs can be felt within the rash.
• Some cases involve hair loss also as a consequence of the disease.
• There may be splitting of thick skin on the palms and soles and become painful.
• The itching is usually severe at first and later it becomes less irritating.
• Swelling of the legs, feet and other parts of the body may be the early symptoms of the disease.

Causes:-

The exact cause of pityriasis rubra pilaris is not known, but several investigations suggest that some of the causes may be :

• Sometimes minor burns rashes and infections may trigger pityriasis rubra pilaris.
• Fungal infection may also be a cause of the disease.
• Pityriasis Rubra Pilaris is generally sporadic, but in some forms it may be partially inherited.
• Chances of Recurrences are generally rare.

Treatment:-

The main effective treatments for pityriasis rubra pilaris are as:-

• The best ever known treatment is Accutane or Soriatane pills.
• Topical medications such as emollients are may be used to relieve dryness and cracking of the skin.
• acitretin or isotretinoin may also be useful if the case is severe .
• Steroid creams and ointments soothe but probably useful in removing rash from the skin.
• Pruritus is commonly associated with the rash and can be treated conservatively with calamine lotion or oral antihistamine therapy.
• Greasy applications : such as soft white paraffin, helps in reducing the splitting of the thickened palms and soles.
• Treated with a derivative of vitamin A known as acitretin also useful in Pityriasis rubra pilaris but the treatment has some severe side effects.
• Methotrexate a strong drug used for suppressing the immune system may be used as a treatment for pityriasis rubra pilaris, but it too has many side effects.