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

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...

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Detalles Bibliográficos
Autores: Montero-Menárguez, J, Samaranch, VA, Sanz, LP, Ruiz-Villaverde, R, Arias-Santiago, S, García, ML, Genao, DR, Ferrán, M, Schneller-Pavelescu, L, Ferreiro, CR, Díaz, RR
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Institución:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
Repositorio:r-FISABIO. Repositorio Institucional de Producción Científica
OAI Identifier:oai:fisabio.fundanetsuite.com:p18606
Acceso en línea:https://fisabio.portalinvestigacion.com/publicaciones/18606
Access Level:acceso abierto
Palabra clave:Pityriasis rubra pilaris
Biologic agents
Erythroderma
Descripción
Sumario: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. (c) 2024 AEDV. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).