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Pityriasis Rubra Pilaris - Causes, Symptoms and Treatment


Definition

Pityriasis rubra pilaris was first described in 1828 by Tarral and was named by Besnier in 1889. It can be characterize by rare skin disorder that present with reddish-orange colored scaling patches with well defined borders. It may cover the entire body or just parts of the body such as the elbows and knees, palms and soles. It is an erythematosquamous eruption characterized by the association of palmoplantar keratoderma, follicular plugging and erythematous perifollicular papules which may progress to plaques or erythroderma. Initially, the disorder is characterized by elevated erythematous spots on the skin. These spots grow and become connected, producing red plaques over large areas. The inherited form starts early in childhood with persistent long-term symptoms into adulthood. Although most people who develop PRP are over age 50, individuals of any age, race, and nationality can be affected. Women and men seem to be equally affected.

Causes

  1. The cause of pityriasis rubra pilaris is unknown. Although some condition are looking to help in causing this disease.
  2. Sometimes minor burns rashes and infections may cause this disease. The infection may be by viral, fungus or yeast.

Symptoms

  1. The familial form of pityriasis rubra pilaris typically begins in early childhood and has an autosomal dominant inheritance pattern.
  2. Patients with extensive disease may develop ectropion. Patients have also reported blurred vision and dryness.
  3. In this disease there may occur orange-red or salmon-colored scaly plaques with sharp borders, which may expand to involve the entire body.
  4. Nail changes include distal yellow-brown discoloration, subungual hyperkeratosis, longitudinal ridging, nail plate thickening, and splinter hemorrhages.
  5. The palms and soles are usually involved and become diffusely thickened and yellowish.
  6. PRP most often starts on the head, neck and upper trunk as a red scaly rash. Often there is a solitary lesion but within a few weeks multiple patches appear and they join together to form groups of reddish-orange lesions.
  7. Individuals of any age, race, and nationality can be affected. Women and men seem to be equally affected.

Treatment

  1. Treatment is exceedingly difficult and empiric. Disease may be ameliorated but almost never cured
  2. Classic forms of the disease resolve slowly over 3 yr, while nonclassic forms persist. Scaling may be improved with emollients or 12% lactic acid under occlusive dressing, followed by topical corticosteroids.
  3. Topical corticosteroids may provide some patient comfort, but they are believed to have little long-term therapeutic effect.
  4. Topical applications of bland emollients are recommended. Lac-Hydrin is particularly helpful.
  5. Accutane is the treatment of choice in adult-onset pityriasis rubra pilaris. Etretinate has also been reported to be effective.
  6. Vitamin a is very helpful especially when it is associated with vitamin E.
  7. Photochemotherapy: This involves the ex vivo exposure of leukapheresed peripheral blood mononuclear cells to UVA in the presence of DNA-intercalating agent such as 8-MOP and subsequent reinfusion of the treated cells.
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Pityriasis Rubra Pilaris
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