PITYRIASIS RUBRA PILARIS

 

PITYRIASIS RUBRA PILARIS

 

DEFINITION:

A chronic cutaneous disorder characterized by small follicular papules, disseminated scaling patches, and palmoplantar hyperderatosis.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • Type II (first 5 years of life); Type I (51-55 years)
  • risk factors:
    • familial - autosomal dominant (Type II)
      • chrom.#: ?
      • gene: ?
    • M = F

PATHOGENESIS:

1. Background

  • unknown etiology and attempts to link this disorder with a defect in vitamin A metabolism have not been definitive

2. Types

Type I

  • classic adult form
  • makes up 60% of cases
  • good prognosis with 80% involuting after 3 years

Type II

  • juvenile-onset (3 forms)
  • make up 40% of cases
  • poorer prognosis for involuting

CLINICAL FEATURES:

1. Cutaneous Manifestations

1. Prodrome

  • insidious onset with diffuse scaling and erythema of the scalp

2. Primary Rash

  • a firm acuminate, tiny papule, reddish-brown in colour topped by a central keratotic (horny) plug pierced by a vellus hair
  • masses of these papules coalesce to form large erythematous sharply demarcated plaques within which islands of normal skin can be found
  • initially found on the sides of the neck, the trunk, and on the extensor surfaces of the extremities but then extensive areas become involved which look like exaggerated goose-flesh and feel like a nutmeg grater
  • rash takes on an orange salmon-yellow colour
  • may be pruritic and the Koebner phenomenon may be present, i.e., new onset of lesions at the site of trauma

3. Others

  • thick hyperkeratosis of the palms and soles with a tendency to fissure (may get a sandal-like plantar hyperkeratosis)
  • nails - dull, rough, thickened, brittle, striated, but pitting is not evident

INVESTIGATIONS:

1. Skin Biopsy

  • follicular hyperkeratosis
  • inflammatory changes in the dermis

MANAGEMENT:

1. Rash

  • topical bland emollients
  • isotretinoin (Accutane) in Type I

 

 

Pediatric Database - PITYRIASIS RUBRA PILARIS

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