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Juvenile pityriasis rubra pilaris
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Juvenile pityriasis rubra pilaris

Contributors: Teri Greiling MD, PhD, Vivian Wong MD, PhD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

This summary discusses pityriasis rubra pilaris in children. Pityriasis rubra pilaris in adults is addressed separately.

Juvenile pityriasis rubra pilaris (PRP) is an inflammatory disorder affecting cornification with onset in childhood. PRP is usually acquired, but there are heritable forms. It can present in 3 clinical forms: type III (classical), type IV (circumscribed), and type V (atypical). Overlap between these subtypes is common in children.

In classical / type III (10% of all cases), there is a fairly rapid onset of extensive, confluent, scaling, pink-orange plaques in association with keratoderma of the hands and feet. Age of onset may be at any time during childhood, with a peak around age 6. Half of all cases resolve without therapy in less than 6 months, although many take longer to resolve and 10%-20% may become chronic.

Circumscribed / type IV (25% of all cases) primarily involves the extensor knees and elbows with palmoplantar keratoderma. It is the most common pediatric subtype. Onset is most commonly at age 3-9 years. The rate of spontaneous resolution is similar to classical juvenile / type III PRP.

Atypical / type V (less than 5% of all cases) is present from birth or in the first few years of life. It may be inherited in an autosomal dominant pattern due to activating mutations in CARD14

Codes

ICD10CM:
L44.0 – Pityriasis rubra pilaris

SNOMEDCT:
238620000 – Classical juvenile pityriasis rubra pilaris

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Psoriasis is the closest clinical mimic of PRP. Psoriasis has a more adherent scale, and larger lesions do not have areas of normal skin within them.
  • Keratosis pilaris is usually limited to the extensor extremities.
  • Wong-type dermatomyositis – presents with features of both Juvenile dermatomyositis and PRP
  • Erythrokeratodermia variabilis
  • Erythroderma
  • Drug eruption general overview
  • Atopic dermatitis
  • Contact dermatitis (Irritant contact dermatitis or Allergic contact dermatitis )
  • Sézary syndrome (rare in children; see Cutaneous T-cell lymphoma)
  • Type V / atypical juvenile PRP can be confused with Lamellar ichthyosis. Scleroderma of childhood-like changes of hands and feet may be rarely observed.
  • CARD14-associated papulosquamous eruption (CAPE) can present with classical PRP-like features, psoriasis-like features, or atypical PRP-like features.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:05/08/2023
Last Updated:05/22/2023
Copyright © 2024 VisualDx®. All rights reserved.
Juvenile pityriasis rubra pilaris
A medical illustration showing key findings of Juvenile pityriasis rubra pilaris (Widespread) : Confluent configuration, Erythroderma, Follicular configuration, Palms and soles, Scaly plaque, Widespread distribution
Clinical image of Juvenile pityriasis rubra pilaris - imageId=1787397. Click to open in gallery.  caption: 'A close-up of well-demarcated orange-pink plaques with fine scale.'
A close-up of well-demarcated orange-pink plaques with fine scale.
Copyright © 2024 VisualDx®. All rights reserved.