A case of juvenile pityriasis rubra pilaris: could varicella be an aetiological agent?

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disorder. Treatment is challenging; the armamentarium consists of topical corticosteroids, phototherapy, classic systemic treatments such as retinoids or immunosuppressive drugs, and most recently biologicals. However, the relative effectiveness of therapies is unclear. Our objective was to review the published literature on systemic treatment of PRP. A systematic review was conducted on PubMed and the Cochrane Library up to 5 September 2017. Studies evaluating any systemic treatments of PRP (except for historical treatments) were included. Overall, 182 studies met the predefined inclusion criteria, and reported on 475 patients and 652 courses of treatment. 42.0 % (225/514) of all patients treated with retinoids achieved an excellent response (isotretinoin: 61.1 % [102/167], etretinate: 47 % [54/115], and acitretin: 24.7 % [43/174]) compared to an excellent response rate of 33.1 % (53/160) with methotrexate. Therapy with biologicals was successful in 51.0 % of patients (71/133) (ustekinumab: 62.5 % [10/16], infliximab: 57.1 % [28/49], etanercept: 53.3 % [16/30], and adalimumab: 46.4 % [13/28]). This review balances effectiveness, side effects, experience, and drug costs in order to suggest a treatment regimen starting with isotretinoin as first-line, methotrexate as second-line and biologicals as third-line treatment for this difficult-to-treat dermatosis.

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

Section: Resultsmentioning

confidence: 99%

“…Only a minority reported a multicenter setting . Four out of five studies described the treatment modality clearly , but only 15 % applied clearly defined outcome measures . Statistical analysis was rarely performed .…”

Section: Resultsmentioning

confidence: 99%

Systemic therapies of pityriasis rubra pilaris: a systematic review

Kromer

Sabat

Celis

et al. 2018

J Deutsche Derma Gesell

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“…The etiology of PRP remains largely elusive. Defects in keratinization and vitamin A metabolism, response to physical triggers such as trauma, autoimmune abnormalities, and a superantigen‐mediated process have all been proposed . Similar to psoriasis, PRP may develop as the result of a combination of genetic susceptibility and environmental triggers (13).…”

Section: Discussionmentioning

confidence: 99%

“…There have been many reports of PRP cases after trauma or infection . Historically, PRP has been known to occur in association with HIV infection, but more recently, other potential infectious triggers have been identified .…”

Section: Types Of Pityriasis Rubra Pilaris (Prp) and Their Featuresmentioning

confidence: 99%

An Unusual Cluster of Circumscribed Juvenile Pityriasis Rubra Pilaris Cases

Martin

Holland

Lyon

et al. 2014

Pediatric Dermatology

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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.

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“…Über multizentrische Studien wurde nur in wenigen Publikationen berichtet . In vier von fünf Studien wurde die Behandlungsmodalität klar beschrieben , dagegen wurde nur bei 15 % ein eindeutig definiertes Ergebnismaß angewendet …”

Section: Ergebnisseunclassified

Systemische Therapien bei Pityriasis rubra pilaris: eine systematische Übersicht

Kromer

Sabat

Celis³

et al. 2019

J Deutsche Derma Gesell

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Zusammenfassung Pityriasis rubra pilaris (PRP) ist eine seltene papulosquamöse Erkrankung. Die Behandlung ist schwierig; das therapeutische Arsenal umfasst topische Corticosteroide, Phototherapie, klassische Systemtherapien wie Retinoide oder Immunsuppressiva sowie, seit Kurzem, Biologika. Die relative Wirksamkeit der Behandlungen ist jedoch unklar. Unser Ziel war die Durchsicht der Literatur zur systemischen Behandlung der PRP. In PubMed und der Cochrane Library wurde eine systematische Recherche bis zum 5. September 2017 durchgeführt. Eingeschlossen wurden Studien, in denen systemische Behandlungen der PRP (ausgenommen historische Behandlungen) geprüft wurden. Insgesamt erfüllten 182 Studien die vorgegebenen Einschlusskriterien; sie enthielten Daten über 475 Patienten und 652 Behandlungszyklen. 42,0 % (225/514) aller mit Retinoiden behandelten Patienten sprachen hervorragend an (Isotretinoin: 61,1 % [102/167], Etretinat: 47 % [54/115] und Acitretin: 24,7 % [43/174]), verglichen mit einer hervorragenden Ansprechrate von 33,1 % (53/160) unter Methotrexat. Therapien mit Biologika waren bei 51,0 % der Patienten (71/133) erfolgreich (Ustekinumab: 62,5 % [10/16], Infliximab: 57,1 % [28/49], Etanercept: 53,3 % [16/30] und Adalimumab: 46,4 % [13/28]). In dieser Übersicht werden Wirksamkeit, Nebenwirkungen, Erfahrungen und Arzneimittelkosten gegeneinander abgewogen und es wird ein Behandlungsregime mit Isotretinoin als Erstlinien‐, Methotrexat als Zweitlinien‐ und Biologika als Drittlinienbehandlung vorgeschlagen.

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A case of juvenile pityriasis rubra pilaris: could varicella be an aetiological agent?

Cited by 10 publications

References 4 publications

Systemic therapies of pityriasis rubra pilaris: a systematic review

Systemic therapies of pityriasis rubra pilaris: a systematic review

An Unusual Cluster of Circumscribed Juvenile Pityriasis Rubra Pilaris Cases

Systemische Therapien bei Pityriasis rubra pilaris: eine systematische Übersicht

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