Epidemiologic, Clinicopathologic, Diagnostic, and Management Challenges of Pityriasis Rubra Pilaris

Ross, Nicholas; Chung, Hye Jin; Li, Qiaoli; Andrews, Jonathan; Keller, Matthew; Uitto, Jouni

doi:10.1001/jamadermatol.2016.0091

Cited by 74 publications

(111 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…PRP is a rare skin disease, and a standard therapeutic protocol does not exist 1 . Treatment options include topical corticosteroids, acitretin, methotrexate, cyclosporine, phototherapy, and tumor necrosis factor (TNF)-α blockers.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of type II pityriasis rubra pilaris with secukinumab

et al. 2016

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Successful treatment of type II pityriasis rubra pilaris with secukinumab

et al. 2016

View full text Add to dashboard Cite

“…In a case series of 100 patients, the three most commonly reported topical were emollients (90%), corticosteroids (76%) and urea‐containing emollients (68%). Among these, emollients (76%), corticosteroids (50%) and emollients containing salicylic acid (45%) were perceived by the patients to be most helpful . Pimecrolimus has been described to be an effective alternative to corticosteroids .…”

Section: Topical Treatmentmentioning

confidence: 99%

“…Systemic modalities that were rated as most helpful were retinoids (59%) and methotrexate (52%). Interestingly, although UV therapy was one of the most commonly used systemic therapies, only one in 13 patients found it helpful …”

Section: Uv Therapy and Systemic Treatment Options (Without Biologics)mentioning

confidence: 99%

Pityriasis rubra pilaris: algorithms for diagnosis and treatment

Roenneberg

Biedermann

2018

Acad Dermatol Venereol

View full text Add to dashboard Cite

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.

show abstract

“…Topical corticosteroids and emollients, systemic retinoids, methotrexate, cyclosporin A and phototherapy may be administrated with benefit in some PRP patients . Although PRP may resolve spontaneously in 1–3 years, in many cases it poses a therapeutic challenge as it is refractory to commonly utilized treatments . In such cases, the use of anti‐tumor necrosis factor (TNF) molecules in both sporadic and familiar PRP refractory to other therapeutic agents has been reported .…”

Section: Introductionmentioning

confidence: 99%

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

Napolitano

Lembo

Fania

et al. 2017

The Journal of Dermatology

View full text Add to dashboard Cite

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.

show abstract

Epidemiologic, Clinicopathologic, Diagnostic, and Management Challenges of Pityriasis Rubra Pilaris

Cited by 74 publications

References 18 publications

Successful treatment of type II pityriasis rubra pilaris with secukinumab

Successful treatment of type II pityriasis rubra pilaris with secukinumab

Pityriasis rubra pilaris: algorithms for diagnosis and treatment

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

Contact Info

Product

Resources

About