Management of refractory pityriasis rubra pilaris: challenges and solutions

Abstract

Pityriasis rubra pilaris (PRP) is a rare chronic inflammatory papulosquamous skin disease. Its clinical presentation and evolution is very variable. The most frequent clinical features are follicular papules, progressing to yellow-orange erythroderma with round small areas of normal skin and the well-demarcated palmoplantar keratoderma. Actually, six different types of PRP have been described based on clinical characteristics, age of onset, and prognosis. The pathogenesis is still unknown, and treatment can be challenging. Available treatments are mainly based on case reports or case series of clinical experience because no controlled randomized trials have never been performed because of the rarity of the condition. Traditional systemic treatment consists in retinoids, which are actually considered as first-line therapy, but refractory cases that do not respond or relapse after drug interruption do exist. In recent years, numerous reports have demonstrated the efficacy of new agents such as biological drugs. This article is an overview on available therapeutic options, in particular for refractory forms of PRP.

Successful treatment of type I pityriasis rubra pilaris with ixekizumab

“… 4 Historically, because of similarities clinically and histopathologically with psoriasis, PRP has been treated with oral retinoids as first-line therapy with methotrexate as an alternative, and more recently the use of biologic agents has been reported. 5 , 6 , 7 However, the assumptions regarding the natural course of PRP and its response to treatment with traditional therapies for psoriasis have been questioned. In a retrospective case study of 100 patients with PRP, Ross et al 8 found that only 26% of patients were correctly diagnosed at the time of presentation, diagnosis was delayed by 29 months on average, and that 72% had persistent clinical findings, with persistence of 58 months on average and up to 300 months.…”

The management and genetic background of pityriasis rubra pilaris: a single‐centre experience

“…Recently, biological therapies, such as TNFalpha blocking agents, IL-23 and IL-17 inhibitors may also represent therapeutic options. 29,30 Ustekinumab acting on the NF-jB pathway may be effective in cases of PRP with a gainof-function mutation in CARD14. 5 Systemic corticosteroids are considered to be ineffective and not used in PRP and psoriasis in general.…”

Pityriasis rubra pilaris – eine seltene entzündliche Dermatose mit vielen Facetten

“…tiger Therapie zu erwarten [43]. Die für einen Therapieeffekt erforderlichen Dosen der Retinoide resultieren nicht selten in mukokutanen Nebenwirkungen oder Haarverlust, die zum frühzeitigen Abbruch der Therapie führen können.…”

“…Eine kürzlich veröffentlichte systematische Analyse der Literatur der letzten 50 Jahre konnte ein relativ gutes Ansprechen auf Methotrexat bei überwiegend guter Verträglichkeit belegen [46]. Es werden Dosen zwischen 5 bis 25 mg wöchentlich (mit Folsäuresupplementation am Folgetag) eingesetzt [43].…”