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Pityriasis rubra pilaris

Synonym(s) -
Prevalence <1 / 1 000 000
Inheritance Autosomal dominant
or Not applicable
Age of onset Infancy
  • L44.0
  • C0032027
  • D010916
  • 10035116


Disease definition

Pityriasis rubra pilaris is a rare chronic papulosquamous disorder of unknown etiology characterized by small follicular papules, scaly red-orange patches, and palmoplantar hyperkeratosis, which may progress to plaques or erythroderma. Although most of the cases are sporadic and acquired, a familial form of the disease exists.

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