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., Amat Samaranch, V., Puig Sanz, L., Ruiz-Villaverde, R., Arias-Santiago, S., Larrea García, M., Ruiz Genao, D., Ferrán, M., Schneller-Pavelescu, L., Romero Ferreiro, C., Rivera Díaz, R.
Tipo de recurso: artículo
Fecha de publicación:2024
País:España
Institución:Universidad Francisco de Vitoria
Repositorio:DDFV. Repositorio Institucional de la Universidad Francisco de Vitoria
Idioma:inglés
OAI Identifier:oai:ddfv.ufv.es:10641/7814
Acceso en línea:https://hdl.handle.net/10641/7814
Access Level:acceso abierto
Palabra clave:Biologic agents
Erythroderma
Pityriasis rubra pilaris
Pathology and Forensic Medicine
Histology
Dermatology
Multicenter Study
Journal Article
Yes
yes
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.