(Juvenile) Pityriasis rubra pilaris

Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, Delhi, India. in
International Journal of Dermatology (Impact Factor: 1.31). 05/2006; 45(4):438-46. DOI: 10.1111/j.1365-4632.2006.02666.x
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Available from: Virendra N Sehgal, Aug 27, 2014
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    • "The etiology of PRP is unknown. Both familial and acquired forms of the disease have been described [7,910]. PRP has been reported in association with HIV infection [11-13], malignancy [4] and Down’s syndrome [14]. "
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    ABSTRACT: Pityriasis rubra pilaris (PRP) is a rare, chronic erythematous squamous disorder of unknown etiology. It has been found in association with several autoimmune diseases, including thyroiditis, myositis, myasthenia gravis and vitiligo. Herein we report a case of systemic sclerosis in a patient with classic adult pityriasis rubra pilaris. A 38 year old woman with classic adult type 1 pityriasis rubra pilaris (PRP) developed progressive skin thickening of the trunk, face, upper and lower extremities after 2 years of PRP treatment with topical emollients and steroids. Clinical examination and immunological findings were consistent with SSc. Co-existence of these two rare conditions is documented for the first time.
    Full-text · Article · Aug 2010 · Pan African Medical Journal
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    ABSTRACT: RESUMEN Antecedentes: la pitiriasis rubra pilaris es una afección de causa desconocida, poco frecuente, que se distingue por pápulas foliculares, placas eritematoescamosas, o ambas. La evolución es variable, crónica y recidivante, por lo general, benigna. Objetivo: determinar las características clínicas y epidemiológicas de los casos de pitiriasis rubra pilaris. Material y método: se consultaron los archivos de los últimos diez años de la clínica de dermatología pediátrica del Centro Dermatológico Pascua para analizar las características epidemiológicas y clínicas de estos casos, y se evaluaron las siguientes variables: edad de inicio, edad al momento del diagnóstico, sexo, manifestaciones clínicas al momento del diagnóstico (clasificación de Griffiths) y antecedentes heredo-familiares relacionados. Resultados: de 2,790 expedientes analizados, se encontraron 44 de pacientes con diagnóstico clínico de pitiriasis rubra pilaris, con una prevalencia de 0.016 y una incidencia de 4.4. De éstos, 66% fueron hombres y 34% mujeres (relación 2:1). La edad predominante fue de uno a cuatro años. En 33 casos (75%) se encontraron manifestaciones clínicas congruentes con el tipo III. Solamente en un paciente hubo antecedentes heredo-familiares relevantes para la enfermedad. Conclusiones: la pitiriasis rubra pilaris es una enfermedad poco frecuente en la población de estudio, predomina en hombres, la evolución en general es benigna y de curación espontánea. Palabras clave: pitiriasis rubra pilaris, hiperqueratosis, alteraciones de la queratinización. ABSTRACT Background: Pityriasis rubra pilaris (PRP) is an entity with unknown etiology, not frequent, characterized by the presence of follicular papu-les and/or erythemato-squamous plaques. The course is variable, chronic, and recurrent; generally benign. There are many contradictions between epidemiological data reported in literature, therefore we decided to design this retrospective, transversal, and descriptive study. Objective: To analyze the files of the Centro Dermatológico Pascua for the PRP cases and to evaluate the clinical features along with the epidemiology. Material and methods: The last 10 years' files have been consulted searching for PRP cases based on clinical features: The next va-riables were looked for: age at onset, age at diagnosis, gender, clinical features at diagnose (Griffiths' classification), and related familiar background. Results: From the total of patients (2,790), 44 met with PRP diagnosis, with a prevalence of 0.016 and an incidence of 4.4. From the 44 patients, 66% were males, and 34% females (relation 2:1). The most frequent age of presentation was between one and four years (16 cases, 36.4%). In 33 cases (75%) we found clinical features of the type III. Familiar background was found in only one patient. Conclusions: PRP is an entity with low frequency in our study population, most common in male patients and generally has a benign and auto-limited evolution.
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    Full-text · Article · Nov 2006 · International Journal of Dermatology
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