An Unusual Cluster of Circumscribed Juvenile Pityriasis Rubra Pilaris Cases

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.

A novel presentation of juvenile pityriasis rubra pilaris with possible involvement of bacterial superantigens

“…The recent history of GABHS infection and the early resolution with no recurrences during the long follow-up period supported the diagnosis of postinfectious PRP, as reported in the literature [8,9]. However, our cases also showed some peculiarities: the stereotypical clinical presentation with exclusive involvement of the face, genitals and palmoplantar area, the previous cutaneous manifestations (palmoplantar erythema and diffuse rash) and the concomitance of other skin signs (acral lamellar peeling, strawberry tongue) [9].…”

Systemic therapies of pityriasis rubra pilaris: a systematic review

“…Overall, 743 records were identified and 182 studies were considered eligible for analysis (Figure ).…”

Pityriasis rubra pilaris

“…Etiológiája és patogenezise nem tisztázott. Hátterében számos ok felvetôdik, mint felsô légúti Streptococcus fertôzés, amely a szuperantigének szerepére utalhat (3,4), malignus tumor (5), A-hypovitaminosis, esetleg hypothyreosis. A pajzsmirigy hormon elégtelenség esetén a karo-tin A-vitaminná alakulása gátolt, ami felelôs lehet a bôrtünetek kialakulásáért (6).…”