Pityriasis rubra pilaris as the initial presentation of renal cell carcinoma?

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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“…[9][10][11][12][13][14] The successful treatment of PRP with antiretroviral drugs in HIV-positive patients can be especially placed as a standard therapeutic measure. 15 Anecdotal reports suggest that autoimmune diseases 16,17 and neoplasias [18][19][20][21][22][23] can trigger PRP. In a minority of patients, PRP is inherited in an autosomal-dominant fashion.…”

Section: Methodsmentioning

confidence: 99%

Pityriasis rubra pilaris: algorithms for diagnosis and treatment

Roenneberg

Biedermann

2018

Acad Dermatol Venereol

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Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages and also children. The clinical appearance of PRP is highly variable, as is the individual prognosis. Therefore, stratifying PRP into six disease subtypes represents a first step to personalized medicine for this rare inflammatory skin disease. The next step should be to associate specific therapeutic strategies with these subtypes of PRP. However, no randomized, controlled trials on the treatment of PRP have been performed. Consequently, the actual treatment algorithm for PRP will be based on clinical experience, small case series and case reports. The majority of published evidence is on type I PRP, whereas the treatment experience for other clinical types of PRP is still sparse and has to be gained. Nevertheless, it is now time to start developing valid algorithms as a basis for the diagnosis and treatment of PRP based on the data available. This review makes use of algorithms developed in psoriasis and atopic eczema and puts together recent insights into the pathogenesis, diagnosis and treatment experience of PRP. The innovative intention of this appraisal is to develop a structured algorithm for PRP treatment that should be further developed going forward.

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“…Reported associations of PRP with myasthenia gravis, coeliac sprue, myositis, and inflammatory arthritis raise the possibility of an autoimmune aetiology 7–10 . Other reported associations include HIV infection, and internal malignancies such as renal cell, bronchogenic and hepatocellular carcinomas 1,2,11 …”

Section: Discussionmentioning

confidence: 99%

“…Pityriasis rubra pilaris (PRP) is a rare idiopathic dermatosis, characterized by orange–red waxy keratoderma, and follicular papules on an erythematous base which can coalesce to form large red to orange–red plaques and, when severe, erythroderma with distinctive ‘islands of sparing’. PRP is associated with autoimmune diseases, HIV infection, and internal malignancies, but only infrequently with kidney diseases 1,2 . We report a case of PRP with concomitant membranous nephropathy (MN), which resolved spontaneously with resolution of the dermatosis.…”

Section: Introductionmentioning

confidence: 97%

A case of pityriasis rubra pilaris associated with membranous nephropathy

Lin¹,

Maurice²,

Cross

2011

Aust J Dermatology

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Pityriasis rubra pilaris (PRP) is a rare idiopathic dermatosis which may be associated with autoimmune diseases, HIV infection, and internal malignancies. Its association with renal diseases is, however, much less recognized. We report a case of PRP with associated membranous nephropathy (MN), which resolved spontaneously with resolution of the dermatosis. This is only the second reported association between PRP and MN of which we are aware. Further reports of such an association will strengthen the evidence for the two conditions being linked and may thereby shed light on the pathogenesis of both PRP and MN.

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Pityriasis rubra pilaris as the initial presentation of renal cell carcinoma?

Cited by 18 publications

References 6 publications

Systemic therapies of pityriasis rubra pilaris: a systematic review

Systemic therapies of pityriasis rubra pilaris: a systematic review

Pityriasis rubra pilaris: algorithms for diagnosis and treatment

A case of pityriasis rubra pilaris associated with membranous nephropathy

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