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Pityriasis Rubra Pilaris - Causes, Symptoms and Treatment

 

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Pityriasis rubra pilaris is an uncommon skin condition. The cause of pityriasis rubra pilaris is unknown. Sometimes minor burns rashes and infections seem to trigger it. It is not really a single condition, but rather a group of unusual eruptions that cause red scaly patches containing dry plugged pores. It may cover the entire body, or just the elbows and knees. Pityriasis rubra pilaris is a rare erythematosquamous cutaneous eruption with characteristic follicular papules that is associated with abnormal metabolism of vitamin A. Pityriasis rubra pilaris 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 outlook in pityriasis rubra pilaris depends on its type. The acquired form usually shows a spontaneous or gradual remission of symptoms within several years although long-term symptoms may continue for years. The inherited form starts early in childhood with persistent long-term symptoms into adulthood. Treatment helps, but cannot be guaranteed to clear either of the two most common types.

Pityriasis rubra pilaris affects persons of all ages, races, and nationalities. The disease is rare but no exact incidence has been reported. The disease can run over weeks, months, or even years,in which lesions appearing and disappearing continually. Women are more frequently affected. The 2 most common forms of the disease are juvenile classic, characterized by autosomal dominant inheritance and childhood onset, and adult classic, characterized by no apparent inheritance and adult onset. Pityriasis Lichenoides can affect all individuals equally. The eruption is usually generalized, affecting chiefly the trunk, and sparing sun-exposed surfaces. Cracks may develop which can be painful and make walking and using the hands difficult. The nails may become thickened and discoloured at the free nail edge and may show linear black streaks. 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.

Causes of Pityriasis rubra pilaris

The cause of pityriasis rubra pilaris is not known. It is not an infection and cannot be passed on to others. Sometimes Pityriasis rubra pilaris is suspected only after the usual creams, pills and even ultraviolet light treatments used for skin conditions have no effect. Pityriasis rubra pilaris mostly affects adults over 40, but some children are also affected. Sometimes minor burns rashes and infections seem to trigger it.

Common causes and risk factors of Pityriasis rubra pilaris:

  • Highly contagious.
  • Fungal infection.
  • Sometimes minor burns rashes and infections.
  • Weather condition.

Signs and Symptoms of Pityriasis rubra pilaris

Pityriasis rubra pilaris most often starts as a patchy rash on the scalp, face or chest. The efflorescence of new lesions spreads rapidly, and after three to eight weeks they usually disappear spontaneously. Rough, dry plugs can be felt within the rash. The itching is usually severe at first, and then later is not as bad as you would think considering how bad the rash looks. Cracks may develop which can be painful and make walking and using the hands difficult. The nails may become thickened and discoloured at the free nail edge and may show linear black streaks. The hair may thin considerably.

Sign and symptoms may include the following :

  • Skin redness.
  • Lesions of the face.
  • Fever.
  • Seborrhea of the scalp.
  • Pink swellings.

Treatment 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. Topical medications such as emollients may be used to relieve symptoms of dryness and cracking. In severe cases acitretin or isotretinoin may be useful. Methotrexate has been of limited value.

Treatment may include:

  • Accutane or Soriatane pills are the best treatment.
  • Potent topical steroids may have a use if only a few lesions are present.
  • Corticosteroids creams and ointments can be used to control the skin rash.
  • Treated with a derivative of vitamin A known as acitretin also useful in Pityriasis rubra pilaris but the treatment has some severe side effects.
  • Topical medications such as emollients may be used to get relive from dryness and cracking.