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Pityriasis Rubra Pilaris

What is Pityriasis Rubra Pilaris ?

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. The term pityriasis rubra pilaris (PRP) has been applied somewhat loosely to a group of patients showing in varying degrees more-or-less circumscribed follicular keratosis, palmoplantar keratoderma and erythroderma.

Pityriasis rubra pilaris is a rare, chronic cutaneous disorder characterized by: (1) Follicular, conical, pinkish papules covered with scales or horny plugs with hair curled on top. These papules are usually found on the back of the fingers and hands. (2) Later, generalized erythema and scaling develop. The eruption rarely becomes universal. The trunk, neck and extremities are commonly involved. The etiology is unknown but an inherited metabolic defect may be the underlying cause. The condition may respond to large doses of vitamin A; this may have to be supplemented with thyroid or corticosteroids.

Pityriasis rubra pilaris is a chronic papulosquamous skin disease of unknown etiology, characterized clinically by redness, scaling patches, small follicular papules, palmoplantar hyperkeratosis. Symptoms can start very suddenly, and may affect the scalp with heavy dandruff, the face with erythematous eruptions which spread quickly from the upper half of the body to involve another portions.The initial manifestations are nonspecific and may be confused with other common dermatoses. 

What causes pityriasis rubra pilaris ?

Classical PRP affects the sexes equally and occurs at any age from early childhood to the eighth decade. There are, however, two peaks: the highest incidence is between 40 and 60, but there is a second smaller peak in the first decade. It is not certain that the juvenile and adult diseases are identical. The cause of pityriasis rubra pilaris is not known. It is not an infection and cannot be passed on to others. 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.

What are the symptoms of pityriasis rubra pilaris?

  • erythema with occasional islands of normal skin.
  • follicular papules on the dorsa of the fingers.
  • hyperkeratosis
  • parakeratosis around follicular openings
  • mild inflammatory infiltrate in the papillary dermis
  • yellow-coloured hyperkeratosis on the palms and soles
  • spontaneous resolution usually occurs in 6 months to 2 years

Pityriasis Rosea

These have medallion like lesions on back with scales pointing towards the center. It has the herald patch. It is prominently found on ribs and are distributed there.

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