Pityriasis rubra pilaris: algorithms for diagnosis and treatment

Abstract

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.

Systemic therapies of pityriasis rubra pilaris: a systematic review

“…A recent review recommends a treatment algorithm starting with retinoids (preferably isotretinoin) as first‐line therapy, immunosuppressive treatment (e.g. MTX) as second‐line therapy, and treatment with biologicals (or apremilast in case of concomitant malignancy) as third‐line therapy . In our systematic review, patients naïve to TNFα antagonists showed a better response to TNF inhibitors than patients who had previously been treated with a TNFα antagonist; a similar phenomenon has been reported for other chronic inflammatory diseases such as psoriasis and psoriatic arthritis .…”

“…However, chronic non‐remitting disease courses have been described , highlighting the need for effective long‐term control in these patients. Various therapies comprising topical application of corticosteroids, phototherapy, classic systemic treatments such as retinoids or immunosuppressive drugs, and most recently biologicals have been described with various effects . However, large clinical trials of high methodological quality are lacking due to the rarity of the disease, and treatment is based mainly on empirical data.…”

“…Several reviews have collected scientific evidence on the effectiveness of different therapies . However, the review processes were mostly performed in a non‐systematic manner and/or with no quantitative analysis .…”

Systemische Therapien bei Pityriasis rubra pilaris: eine systematische Übersicht

“…Es sind jedoch auch chronische, nicht remittierende Krankheitsverläufe beschrieben worden , was die Notwendigkeit einer wirksamen langfristigen Kontrolle bei den betroffenen Patienten unterstreicht. Es wurden mehrere unterschiedlich erfolgreiche Therapien beschrieben, darunter die lokale Anwendung von Corticosteroiden, Phototherapie, klassische Systemtherapien mit Retinoiden oder Immunsuppressiva und seit Kurzem Biologika . Größere methodisch hochwertige klinische Studien liegen jedoch wegen der Seltenheit der Krankheit nicht vor, sodass sich die Behandlung hauptsächlich auf empirische Daten stützt.…”

“…Die wissenschaftlichen Belege für die Wirksamkeit verschiedener Therapien wurden in verschiedenen Übersichtsarbeiten zusammengestellt . Der Review‐Prozess wurde jedoch meist auf nicht‐systematische Weise und/oder ohne quantitative Analyse vorgenommen .…”