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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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
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spelling Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish PatientsMontero-Menárguez, JSamaranch, VASanz, LPRuiz-Villaverde, RArias-Santiago, SGarcía, MLGenao, DRFerrán, MSchneller-Pavelescu, LFerreiro, CRDíaz, RRPityriasis rubra pilarisBiologic agentsErythrodermaIntroduction: 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/).ELSEVIER ESPANA2024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fisabio.portalinvestigacion.com/publicaciones/18606Actas Dermo-SifiliograficasISSN: 00017310ISSNe: 15782190reponame:r-FISABIO. Repositorio Institucional de Producción Científicainstname:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)Españolinfo:eu-repo/semantics/openAccessoai:fisabio.fundanetsuite.com:p186062026-06-11T12:45:17Z
dc.title.none.fl_str_mv Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
title Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
spellingShingle Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
Montero-Menárguez, J
Pityriasis rubra pilaris
Biologic agents
Erythroderma
title_short Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
title_full Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
title_fullStr Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
title_full_unstemmed Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
title_sort Pityriasis Rubra Pilaris: A Multicentric Case Series of 65 Spanish Patients
dc.creator.none.fl_str_mv 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
author Montero-Menárguez, J
author_facet 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
author_role author
author2 Samaranch, VA
Sanz, LP
Ruiz-Villaverde, R
Arias-Santiago, S
García, ML
Genao, DR
Ferrán, M
Schneller-Pavelescu, L
Ferreiro, CR
Díaz, RR
author2_role author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Pityriasis rubra pilaris
Biologic agents
Erythroderma
topic Pityriasis rubra pilaris
Biologic agents
Erythroderma
description 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/).
publishDate 2024
dc.date.none.fl_str_mv 2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
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status_str publishedVersion
dc.identifier.none.fl_str_mv https://fisabio.portalinvestigacion.com/publicaciones/18606
url https://fisabio.portalinvestigacion.com/publicaciones/18606
dc.language.none.fl_str_mv Español
language_invalid_str_mv Español
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv ELSEVIER ESPANA
publisher.none.fl_str_mv ELSEVIER ESPANA
dc.source.none.fl_str_mv Actas Dermo-Sifiliograficas
ISSN: 00017310
ISSNe: 15782190
reponame:r-FISABIO. Repositorio Institucional de Producción Científica
instname:Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
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reponame_str r-FISABIO. Repositorio Institucional de Producción Científica
collection r-FISABIO. Repositorio Institucional de Producción Científica
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