Chapter 4: Papulosquamous Disorders

Pityriasis Rubra Pilaris

What is it?

PRP is an uncommon chronic inflammatory disorder that can be seen in both adults and pediatric patients. It can mimic psoriasis but has a distinct morphology with orange-red follicular papules that join together (coalesce) into large plaques. The cause is unknown.

What does it look like?

Small follicular papules that coalesce to form disseminated yellowish-pink scaly plaques with characteristic islands of sparing. The palms and soles develop yellowish thick waxy scales that can fissure.

How is it treated?

Similar to psoriasis, except for phototherapy which can flare the condition. Most cases resolve in 2-3 years, but systemic therapy might be required to control symptoms.  Acitretin is one of the most effective therapies.

 

Image 4.10: PRP: Erythematous follicular papules coalescing into plaques with scale and islands of sparing

 

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Pediatric Dermatology Copyright © 2023 by Dr. Wingfield E. Rehmus, MD, MPH; Dr. Jamie Phillips; Dr. Lisa Flegel; Dr. Saud Alobaida; and Hannah Podoaba is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, except where otherwise noted.

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