A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF-antagonists

Authors


  • Conflict of interest
    Dr Catherine Smith is invited speaker and receives grant/research support, not related to this study, from Abbott, Janssen-Cilag, Schering-Plough, Serono, Wyeth and Pfizer.

  • Funding source
    The authors acknowledge financial support from the Department of Health via the UK National Institute for Health Research (NIHR) Comprehensive Biomedical Research Centre award to Guy’s & St Thomas’ NHS Foundation Trust in partnership with King’s College London and King’s College Hospital NHS Foundation Trust.

G. Petrof. E-mail:gabriela.petrof@kcl.ac.uk

Abstract

Background  Adult pityriasis rubra pilaris (PRP) type 1 is a rare chronic papulosquamous disorder with clinical and histological parallels with psoriasis. Treatment is challenging and recent case reports suggest a potential role for tumour necrosis factor (TNF) antagonists.

Objectives  Our objective was to systematically review the literature for evidence of efficacy of TNF antagonists in the treatment of adult PRP.

Methods  We performed a systematic search of the Cochrane library, EMBASE, Pubmed and MEDLINE databases. We defined diagnosis of PRP, classified clinical response and whether this was clearly attributed to TNF-antagonists. We also reviewed disease, treatment duration and follow up.

Results  Sixteen articles were selected for detailed review. From these, 12 articles (13 cases) met our predefined criteria and were included in the systematic review. The authors identified two more cases from their personal archive. A total of 15 evaluable cases were included for analysis. Twelve showed complete response (CR) (80%) to TNF-antagonists with a mean time to maximal response of 5 months. In 10 of the CR cases (83%) this was clearly attributable to TNF antagonist therapy.

Conclusion  These data indicate that TNF-antagonists may be of value in treating adult type 1 PRP refractory to other systemic agents but selective reporting bias, together with the lack of standard diagnostic criteria and established spontaneous resolution in PRP, prevent any firm recommendations on their place in management.

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