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An unusual cluster of circumscribed juvenile pityriasis rubra pilaris cases. Pediatr Dermatol 2014;31(2):138-45

Date

01/25/2014

Pubmed ID

24456130

DOI

10.1111/pde.12260

Scopus ID

2-s2.0-84895930935   4 Citations

Abstract

Circumscribed juvenile pityriasis rubra pilaris (PRP) is an uncommon dermatosis. We describe the unusual clustering of circumscribed juvenile PRP cases in our pediatric dermatology clinic in 2011. A retrospective chart review was done of patients presenting during the summer of 2011 with classic findings of circumscribed juvenile PRP. Clinical data including past medical and family history, presenting symptoms, infectious disease history and evaluation, biopsy results, and management were recorded. Seven patients, ages 5 to 19 years, all had strikingly similar skin findings of pink to hyperpigmented, well-defined, scaly papules and plaques on their elbows, knees, dorsal hands, ankles, and Achilles tendons. Four of the seven also had palmoplantar involvement. Four were sibling pairs and the other three were unrelated. Streptococcus pyogenes infection was suspected as a trigger in four of the patients. The unusual clustering of this uncommon disease, along with the occurrence in two sibling pairs, suggests that a genetic susceptibility unmasked by an infectious agent may play a role in its pathogenesis.

Author List

Martin KL, Holland KE, Lyon V, Chiu YE

Authors

Yvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin
Kristen E. Holland MD Associate Professor in the Dermatology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Biopsy
Child
Child, Preschool
Female
Humans
Male
Pityriasis Rubra Pilaris
Retrospective Studies
Wisconsin
Young Adult