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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.
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.
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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