Ineffectiveness of tumor necrosis factor-α blockers and ustekinumab in a case of type IV pityriasis rubra pilaris

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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“…14 To our knowledge, there is only one report of the ineffectiveness of ustekinumab (and TNF blockers) in a type IV PRP case, possibly due to different signaling pathways involved in this type of PRP. 15 In the present study, we treated five PRP patients with 45 mg s.c. ustekinumab with an initial prompt reduction in erythema, follicular hyperkeratosis and scaling in all patients and, after three doses of the drug, four out of five cases (1-3 and 5) reached complete remission of skin lesions and showed no significant relapse of skin lesions in a 13-15-month follow-up period. Case 4 showed instead a significant improvement of skin lesions following ustekinumab treatment but had an overall partial response also with concurrent therapy, namely acitretin, for 6 weeks.…”

Section: Discussionmentioning

confidence: 96%

“…To our knowledge, there is only one report of the ineffectiveness of ustekinumab (and TNF blockers) in a type IV PRP case, possibly due to different signaling pathways involved in this type of PRP …”

Section: Discussionmentioning

confidence: 99%

Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases

Napolitano

Lembo

Fania

et al. 2017

The Journal of Dermatology

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Pityriasis rubra pilaris (PRP) is a rare, chronic, inflammatory skin disease of unknown etiology. Patients refractory to conventional therapies have been treated successfully with biologic drugs such as anti-tumor necrosis factor agents. Recently, a role of the interleukin-23/T-helper 17 axis in PRP has been described. Our objective was to assess the effectiveness of ustekinumab in five patients with adult-onset PRP refractory to conventional therapies. In the present study, four patients had type I and one patient type II adult-onset PRP. They were treated with three s.c. doses of ustekinumab at weeks 0, 4 and 16. Clinical response was evaluated monthly during treatment up to a 15-month follow-up period. All patients promptly showed a decrease in erythema, follicular hyperkeratosis and scaling. After three injections, complete remission of skin lesions was achieved in four out of five cases and a significant clinical improvement was shown in one case. To the best of our knowledge, this is the largest case series reported on ustekinumab treatment in PRP. Our results, in addition to previous studies from other groups, suggest that ustekinumab may be a possible first-line treatment for PRP patients refractory to conventional therapies.

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“…26 However, in 2015, a report of a 29-year-old female patient with type IV PRP demonstrated no clinical response after the third injection of 45 mg ustekinumab. 123 More recently, Schuster et al 124 published the case of a 67year-old patient with refractory type I PRP who responded to treatment with the IL-17A inhibitor secukinumab after 3 weeks of therapy and cleared after 8 weeks of treatment. Secukinumab also seems to be effective in patients with type II PRP, 125 suggesting its further off-label use in PRP.…”

Section: Treatment With Biologics and Small Moleculesmentioning

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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Ineffectiveness of tumor necrosis factor-α blockers and ustekinumab in a case of type IV pityriasis rubra pilaris

Cited by 12 publications

References 10 publications

Systemic therapies of pityriasis rubra pilaris: a systematic review

Systemic therapies of pityriasis rubra pilaris: a systematic review

Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases

Pityriasis rubra pilaris: algorithms for diagnosis and treatment

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