Pityriasis rubra pilaris type 1 spontaneously resolving after 20 years

Abstract

Pityriasis rubra pilaris (PRP) is an uncommon, idiopathic, papulosquamous eruption. We report a longitudinal study of a patient with PRP type 1 who was treated with retinoid therapy for 9 years and whose symptoms resolved spontaneously after 20 years. There are no data in the literature on the disease course of PRP type 1 persisting beyond the usual 3 years. This case highlights both the extreme chronicity of PRP and the possibility of remission after many years of active disease.

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

“…These findings clearly suggest that PRP, while in some cases a readily self-limiting condition, can persist well beyond the anticipated 2–3 years course in other cases. 11 In fact, the natural history of this seems to be more variable than previously realized. This has obvious implications when counseling newly diagnosed patients.…”

Treatment of refractory adult‐onset pityriasis rubra pilaris with TNF‐alpha antagonists: a case series

“…5,6 PRP type I is considered a self-limiting disease, albeit with spontaneous resolution after an average of 3 years and occasionally with long protracted course up to 20 years. 7 In type II PRP, which runs on a chronic course, long-term etanercept treatment for 12 months maintained persistent clearing of skin lesions, with a disease-free interval of 2 months after drug-withdrawal. While effective in controlling disease manifestations (erythroderma) in both types of PRP, etanercept treatment did not change the chronic course of type II PRP.…”

“…Long‐term follow‐up is necessary because of possible relapse in 20% of PRP type I cases, risk of a chronic course and evolution to circumscribed PRP and psoriasis 5,6 . PRP type I is considered a self‐limiting disease, albeit with spontaneous resolution after an average of 3 years and occasionally with long protracted course up to 20 years 7 . In type II PRP, which runs on a chronic course, long‐term etanercept treatment for 12 months maintained persistent clearing of skin lesions, with a disease‐free interval of 2 months after drug‐withdrawal.…”

A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with TNF‐antagonists

“…Specifically only in seven cases was a skin biopsy taken, with most of the earlier reported cases having no histological evidence of PRP. Secondly, we know that 80% of PRP individuals clear within 3 years 2 although a single case of spontaneous resolution after 20 years has been reported 19 . There is therefore the possibility that in some of the cases reported, the resolution of disease may not necessarily be attributable to treatment, but rather represent spontaneous resolution.…”