EA94

ICD-11 MMS 2026-01

Pityriasis rubra pilaris

Description

Pityriasis rubra pilaris (PRP) is the name given to a group of clinically similar papulosquamous dermatoses of unknown aetiology. They initially present with erythematous, hyperkeratotic perifollicular papules, which tend to coalesce into plaques, but may progress to erythroderma, particularly in adults. The distribution, age of onset and speed of onset differ markedly between patients and these differences have been used to classify PRP into a number of clinically distinct subtypes.

Index terms

  • Pityriasis rubra pilaris
  • Devergie's disease
  • PRP - [pityriasis rubra pilaris]
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  • Pityriasis rubra pilaris, adult
  • Pityriasis rubra pilaris type I, classical adult
  • Pityriasis rubra pilaris type II, atypical adult
  • Pityriasis rubra pilaris, juvenile
  • Pityriasis rubra pilaris type III, classical juvenile
  • Pityriasis rubra pilaris type IV, circumscribed juvenile
  • Pityriasis rubra pilaris type V, atypical juvenile
  • HIV-associated pityriasis rubra pilaris

Exclusions from above levels

  • 1B72.0 – Bullous impetigo of the napkin area
  • 1F23.12 – Napkin candidosis
  • EH71 – Dermatoses precipitated by drug therapy
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  • EH76 – Dermatoses associated with specific classes of medication

Sibling codes

  • EA90 – Psoriasis
  • EA91 – Lichen planus
  • EA92 – Lichenoid dermatoses
  • EA93 – Pityriasis lichenoides
  • EA95 – Small plaque parapsoriasis

Foundation entity

Pityriasis rubra pilaris – http://id.who.int/icd/entity/2048594962

Part of: 14: Diseases of the skin