Paraneoplastic pityriasis rubra pilaris: case report and literature review

Abstract

Pityriasis rubra pilaris (PRP; MIM 173200) is an uncommon papulosquamous inflammatory dermatosis. Only a few cases of PRP associated with an underlying malignancy have been documented. We investigated a 59-year-old patient presenting with a fulminant form of PRP recalcitrant to systemic retinoid therapy, in whom the skin disease heralded a diagnosis of cholangiocarcinoma. We searched the MEDLINE database to find articles reporting on similar associations of PRP with malignancies. We identified 10 studies linking PRP and malignancies, but an association between PRP and cholangiocarcinoma has not yet been reported.

Systemic therapies of pityriasis rubra pilaris: a systematic review

“…Overall, 743 records were identified and 182 studies were considered eligible for analysis (Figure ).…”

“…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 .…”

Secukinumab in pityriasis rubra pilaris: A case series demonstrating variable response and the need for minimal clinical datasets

“… 1 It can be spontaneous in nature and associated with familial inheritance or as a paraneoplastic phenomenon. 1 , 2 PRP has a variable clinical presentation with Griffiths criteria classifying PRP into 5 subtypes based on age of onset, clinical course, and prognosis. 1 HIV-related PRP and paraneoplastic PRP have been proposed as additional subtypes.…”

“… 1 HIV-related PRP and paraneoplastic PRP have been proposed as additional subtypes. 2 The pathophysiology of PRP remains unclear but is proposed to be triggered by a variety of as-yet-unidentified triggers of chronic inflammation. 3 Reported effective treatment with etanercept, adalimumab, ustekinumab, and secukinumab 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 implicate tumor necrosis factor α, interleukin (IL)-12/IL-23, and IL-17 5 , 12 as involved in the downstream inflammatory cascade, although the variability in response indicates some degree of disease heterogeneity within Griffiths subtypes.…”

Systemische Therapien bei Pityriasis rubra pilaris: eine systematische Übersicht

“…Über multizentrische Studien wurde nur in wenigen Publikationen berichtet . In vier von fünf Studien wurde die Behandlungsmodalität klar beschrieben , dagegen wurde nur bei 15 % ein eindeutig definiertes Ergebnismaß angewendet …”