Treatment of pityriasis rubra pilaris type I: a systematic review

Engelmann, C.; Elsner, Peter; Miguel, Diana

doi:10.1684/ejd.2019.3641

Cited by 17 publications

(25 citation statements)

References 142 publications

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“…Topische Therapien werden bei lokalisierten Formen sowie bei disseminierten Formen in Kombination mit einer Systemtherapie eingesetzt [2]. Die Anwendung von mittel‐ bis hochpotenten topischen Kortikosteroiden, Vitamin‐D‐Derivaten sowie Calcipotriol, Dithranol, Calcineurin‐Inhibitoren, Salicylsäure und topischen Retinoiden wird beschrieben [2, 3]. Die Daten über den Einsatz von UVA‐ und UVB‐Lichttherapien sind kontrovers.…”

Section: Kommentarunclassified

“…Die Daten über den Einsatz von UVA‐ und UVB‐Lichttherapien sind kontrovers. Sowohl erfolgreiche als auch negative Effekte sind in der Literatur zu finden [2, 3].…”

Section: Kommentarunclassified

“…Bei fehlender Wirksamkeit werden Therapieversuche als „off‐label‐Use“ vorgenommen. Cyclosporin A hat sich in einzelnen Fallberichten als wirksam erwiesen [3, 4]. Methotrexat (MTX) ist eine weitere Therapieoption bei PRP.…”

Section: Kommentarunclassified

“…Eine retrospektive Studie hat ein positives Ansprechen auf MTX bei einigen Patienten mit PRP Typ‐I gezeigt [5]. Die Anwendung von Azathioprin, Apremilast und Antibiotika wie Benzylpenicillin wird in einzelnen Fällen als erfolgreich beschrieben [2, 3].…”

Section: Kommentarunclassified

“…In der Literatur werden mehrere Fallberichte und Fallserien über den Einsatz von Biologika bei therapierefraktärer PRP dargestellt [2, 3]. Anti‐TNF‐alpha Biologika wie Adalimumab, Infliximab und Etarnecept zeigten sich effektiv in der PRP‐Behandlung [6].…”

Section: Kommentarunclassified

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Therapieresistente Pityriasis rubra pilaris erfolgreich therapiert mit Adalimumab

Lima

Pahnke

Schliemann

et al. 2021

J Deutsche Derma Gesell

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Section: Kommentarunclassified

“…Die Daten über den Einsatz von UVA‐ und UVB‐Lichttherapien sind kontrovers. Sowohl erfolgreiche als auch negative Effekte sind in der Literatur zu finden [2, 3].…”

Section: Kommentarunclassified

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Therapieresistente Pityriasis rubra pilaris erfolgreich therapiert mit Adalimumab

Lima

Pahnke

Schliemann

et al. 2021

J Deutsche Derma Gesell

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Guselkumab for Pityriasis Rubra Pilaris and Dysregulation of IL-23/IL-17 and NFkB Signaling

Velasco,

Shao,

Cutler

et al. 2024

JAMA Dermatol

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ImportanceThere is no US Food and Drug Administration–approved treatment for pityriasis rubra pilaris (PRP), and it is common for patients to fail to experience improvement with several systemic options. Involvement of interleukin (IL) 23 suggests a potential therapeutic target.ObjectiveTo determine whether guselkumab, an IL-23p19 inhibitor, provides clinical improvement for participants with PRP and better understand gene and protein dysregulation in PRP.Design, Setting, and ParticipantsThis single-arm, investigator-initiated nonrandomized trial was conducted from October 2019 to August 2022 at a single-center academic university with participants from 8 states in the US. In total, 14 adults with moderate to severe PRP were enrolled; 12 completed the trial. Age-matched and sex-matched healthy controls provided skin and blood for proteomic and transcriptomic studies. The primary outcome was observed at 24 weeks, and additional follow-up occurred at 36 weeks.InterventionGuselkumab is a fully human immunoglobulin G1 λ monoclonal antibody that selectively binds and inhibits the p19 subunit of IL-23. Subcutaneous injections were given at the US Food and Drug Administration–approved dosing schedule for psoriasis over a 24-week period.Main Outcomes and MeasuresThe primary outcome was the mean change in the Psoriasis Area Severity Index (PASI) score at week 24. Secondary outcomes included pruritus, Dermatology Life Quality Index score, clinical response at week 36, and association with transcriptomics and proteomics expression.ResultsA per-protocol analysis was performed for the cohort of 4 female and 8 male patients who had a mean (SD) age of 56.5 (18.7) years. The mean improvement in PASI score, pruritus, and Dermatology Life Quality Index score was 61.8% (P &lt; .001), 62.3% (P = .001), and 60.2% (P &lt; .001), respectively. Nine participants (75%) achieved a 50% improvement in PASI. Among these clinical responders, at week 36, 8 of 9 achieved PASI75, and 6 of 9 achieved PASI90. No participants had pathogenic CARD14 gene variations. There was 1 serious adverse event that was not associated with the study drug. Proteomics and gene expression profiles identified dysregulation of a predominance of inflammatory pathways (such as T helper 17 and nuclear factor κ B) in participants with PRP who later responded well to treatment with guselkumab and stronger dysregulation of keratinocyte development pathways in individuals who did not respond to guselkumab.Conclusion and RelevanceThe results of this nonrandomized trial suggest that guselkumab has efficacy in treating refractory moderate to severe adult PRP.Trial RegistrationClinicalTrials.gov Identifier: NCT03975153

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Pityriasis Rubra Pilaris

Conrad

2024

Rook's Textbook of Dermatology

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Pityriasis rubra pilaris represents a group of rare inflammatory skin disorders of unknown aetiopathology sharing common clinical and histopathological features. The disease is characterised by the appearance of hyperkeratotic follicular papules, which tend to coalesce to diffuse pink or red plaques, and a yellowish palmoplantar keratoderma. It may progress to erythroderma with characteristic distinct areas of uninvolved skin, the so‐called ‘islands of sparing’. Based on its age of onset, clinical presentation and prognosis, pityriasis rubra pilaris has been classified into distinct subtypes.

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Treatment of pityriasis rubra pilaris type I: a systematic review

Cited by 17 publications

References 142 publications

Therapieresistente Pityriasis rubra pilaris erfolgreich therapiert mit Adalimumab

Therapieresistente Pityriasis rubra pilaris erfolgreich therapiert mit Adalimumab

Guselkumab for Pityriasis Rubra Pilaris and Dysregulation of IL-23/IL-17 and NFkB Signaling

Pityriasis Rubra Pilaris

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