No es psoriasis todo lo que parece <span class="so-article-trans-title" dir="auto"> Translated title: Not all that seems is psoriasis </span>

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Abstract

Resumen La pitiriasis rubra pilaris (PRP) es una enfermedad cutánea crónica caracterizada por pápulas foliculares, placas anaranjadas que dejan zonas de piel respetada e hiperqueratosis palmoplantar; su edad de presentación y su clínica son muy variables. Se trata de una enfermedad poco frecuente que puede ser mal diagnosticada de otras dermatosis con apariencia similar. Su tratamiento no está bien establecido y a veces puede resultar poco exitoso. Presentamos el caso de una niña de siete años con una clínica típica de PRP y realizamos una breve revisión de esta patología.

Translated abstract

Abstract Pityriasis rubra pilaris (PRP) is a chronic skin disease characterized by follicular papules, orange plaques that leave islands of non-affected skin, and palmoplantar hyperkeratosis, being its age and clinical presentation very variable. It is a rare disease that can be misdiagnosed with other dermatoses of similar appearance. Treatment is not well established and can be unsuccessful. We present the case of a 7-year-old girl with a typical PRP and expose a brief review of this pathology.

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A Review on Pityriasis Rubra Pilaris.

Vanessa Cui-Lian Chong, Wei-Sheng Chong, Hazel H Oon … (2018)

Pityriasis rubra pilaris (PRP) is an idiopathic, papulosquamous inflammatory dermatosis. It is characterized by hyperkeratotic follicular papules coalescing into orange-red scaly plaques, islands of sparing, and palmoplantar keratoderma. PRP can be subdivided into six clinical subtypes according to Griffiths' classification, based on age of onset, disease extent, prognosis, and other associated features. The sixth subtype of PRP occurs in individuals affected by HIV infection, and retroviral screening in all de novo cases of PRP is advised. Other reported associations include various infections, autoimmunity, drugs, and malignancies, although the true significance of these is still unclear. The genetic basis for familial cases, most commonly categorized under the fifth subtype, has been mapped to gain of function mutations in the caspase recruitment domain family, member 14 (CARD14) gene. Treatment of PRP remains a challenge to this day due to a paucity of high-quality evidence. Therapeutic regimens have been guided mostly by case reports and case series, with the mainstay of treatment being oral retinoids. Recently, biologics have emerged as a promising treatment for PRP. We present a review of the clinicopathologic features, pathogenesis, associated disorders, and treatment of PRP, with an emphasis and critical appraisal of the existing literature on the latter.

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Pityriasis rubra pilaris: algorithms for diagnosis and treatment.

S Roenneberg, T Biedermann (2018)

Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages and also children. The clinical appearance of PRP is highly variable, as is the individual prognosis. Therefore, stratifying PRP into six disease subtypes represents a first step to personalized medicine for this rare inflammatory skin disease. The next step should be to associate specific therapeutic strategies with these subtypes of PRP. However, no randomized, controlled trials on the treatment of PRP have been performed. Consequently, the actual treatment algorithm for PRP will be based on clinical experience, small case series and case reports. The majority of published evidence is on type I PRP, whereas the treatment experience for other clinical types of PRP is still sparse and has to be gained. Nevertheless, it is now time to start developing valid algorithms as a basis for the diagnosis and treatment of PRP based on the data available. This review makes use of algorithms developed in psoriasis and atopic eczema and puts together recent insights into the pathogenesis, diagnosis and treatment experience of PRP. The innovative intention of this appraisal is to develop a structured algorithm for PRP treatment that should be further developed going forward.

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Pityriasis Rubra Pilaris in a 3-Year-Old Male.

Stephen Stacey, Steven Novek, Craig Maddox (2016)

A 3-year-old male presented with progressive pruritic red-orange plaques across most of his body with erythema, desquamation, and fissuring of the hands and feet. He was diagnosed with classic juvenile (type III) pityriasis rubra pilaris (PRP) and treated with oral isotretinoin for 6 months. His skin findings resolved quickly during the treatment period, with residual postinflammatory hypopigmentation resolving within a year. PRP is rare in pediatric patients and standard recommended treatment algorithms for this population are not currently available. Diagnostic and treatment guidelines for PRP are based almost exclusively on case reports or case series, most of which focus on adult patients. The presentation, evaluation, and management of PRP are discussed.

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Journal

Journal ID (publisher-id): pap

Title: Pediatría Atención Primaria

Abbreviated Title: Rev Pediatr Aten Primaria

Publisher: Asociación Española de Pediatría de Atención Primaria (Madrid, Madrid, Spain )

ISSN (Print): 1139-7632

Publication date (Print and electronic): December 2020

Volume: 22

Issue: 88

Pages: 403-406

Affiliations

[2] Alzira. Valencia orgnameHospital de la Ribera orgdiv1Servicio de Pediatría España

[1] Alzira. Valencia orgnameHospital de la Ribera orgdiv1Servicio de Dermatología España

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Publisher ID: S1139-76322020000500016 Publisher ID: S1139-7632(20)02208800016

SO-VID: af6c78c5-9f61-41d2-9444-2d1fbe4fa629

License:

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

No es psoriasis todo lo que parece Translated title: Not all that seems is psoriasis

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Most cited references 5

A Review on Pityriasis Rubra Pilaris.

Pityriasis rubra pilaris: algorithms for diagnosis and treatment.

Pityriasis Rubra Pilaris in a 3-Year-Old Male.

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