Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients

Abstract

Introduction: PRP is a rare entity of unknown etiopathogenesis. Lack of management guidelines makes it a challenge for clinicians.

Objective: To add our experience to increase evidence about PRP.

Methods: We performed a retrospective, descriptive and multicentric study of 65 patients with PRP, being the largest European case series of patients with PRP.

Results: PRP was more frequent in male patients with an average age of 51 years, but erythrodermic forms presented in older patients (average age 61 years). Six (75%) paediatric patients and ten (60%) non-erythrodermic adults controlled their disease with topical corticosteroids. On the contrary, 26 (68%) erythrodermic patients required biologic therapy as last and effective therapy line requiring an average of 6.5 months to achieve complete response.

Conclusion: Our study showed a statistical difference in terms of outcome and response to treatment between children or patients with limited disease and patients who develop erythroderma.

Keywords: Biologic agents; Eritrodermia; Erythroderma; Pitiriasis rubra pilaris; Pityriasis rubra pilaris; Tratamiento biológico.