Paraneoplastische Pityriasis rubra pilaris 
bei metastasiertem Adenokarzinom ohne lokalisierbares Primum

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%

Systemic therapies of pityriasis rubra pilaris: a systematic review

Kromer

Sabat

Celis

et al. 2018

J Deutsche Derma Gesell

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“…In most of these cases, an improvement and clearance of the dermatosis was obtained after tumor removal [5][6][7][8]10]. In our case, the patient had PRP leading to the discovery of a benign cerebral meningioma; noticeably, a dramatic regression of the eruption was observed after surgical removal of the tumor.…”

mentioning

confidence: 67%

“…The initial diagnosis was photo-induced drug eruption due to sitagliptin. [5][6][7][8][9][10]. No improvement was noted and the rash spread, forming follicular lesions coalescing into erythematosquamous and itchy plaques on the face, trunk, upper and lower limbs ( figure 1C).…”

mentioning

confidence: 99%

Pityriasis rubra pilaris and transitional meningioma: a fortuitous association?

Quenan

Kaya

Laffitte

2015

European Journal of Dermatology

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“…Weiterhin wird auch über das paraneoplastische Auftreten einer Pityriasis rubra pilaris z. B. bei Bronchialkarzinom [9], hepatozellulärem Karzinom [9] und metastasiertem Adenokarzinom ohne bekannten Primarius [8] berichtet.…”

Section: Diskussionunclassified