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Pityriasis rubra pilaris, type IV.

Jennifer Bragg1, Agnieszka Witkiewicz, Seth J Orlow

  • 1Department of Dermatology, New York University School of Medicine, USA.

Dermatology Online Journal
|January 13, 2006
PubMed
Summary

Pityriasis rubra pilaris (PRP) is a rare skin condition. A juvenile form of PRP in a 4-year-old girl showed improvement with UVB phototherapy, despite UV radiation potentially worsening the condition.

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Area of Science:

  • Dermatology
  • Pediatric Dermatology
  • Cutaneous conditions

Background:

  • Pityriasis rubra pilaris (PRP) is a rare, chronic disorder of keratinization.
  • Type IV PRP, or circumscribed juvenile PRP, affects prepubertal children and typically presents with localized lesions on extremities.
  • The course of Type IV PRP is unpredictable, and its relationship with ultraviolet (UV) radiation is complex.

Observation:

  • A 4-year-old girl presented with a 3-year history of well-demarcated, salmon-pink, hyperkeratotic plaques.
  • Lesions were symmetrically distributed on elbows, knees, ankles, and dorsal extremities.
  • Diffuse orange-pink palmoplantar keratoderma was also observed.

Findings:

  • Clinical and histological evaluation confirmed the diagnosis of pityriasis rubra pilaris, type IV (circumscribed juvenile).
  • Despite the known potential for UV radiation to exacerbate PRP, the patient demonstrated significant improvement.
  • Treatment with broad-band ultraviolet B (UVB) phototherapy was effective.

Implications:

  • This case highlights a potential therapeutic option for juvenile PRP, specifically UVB phototherapy.
  • It suggests that the response to UV radiation in PRP may be variable and warrants further investigation.
  • Understanding treatment responses in rare pediatric dermatoses like PRP is crucial for clinical management.

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