Article

(Juvenile) Pityriasis rubra pilaris

Dermato-Venereology (Skin/VD) Centre, Sehgal Nursing Home, Delhi, India. in
International Journal of Dermatology (Impact Factor: 1.23). 05/2006; 45(4):438-46. DOI: 10.1111/j.1365-4632.2006.02666.x
Source: PubMed
0 Followers
 · 
132 Views
  • Source
    [Show abstract] [Hide abstract]
    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.
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous disorder of unknown etiology. Studies on this condition from our region are lacking. To describe the clinical and histopathological findings as well as response to treatment of all patients diagnosed with PRP at the American University of Beirut Medical Center between 1995 and 2010 and compare our findings with those published in the literature. Pityriasis rubra pilaris was diagnosed in 32 patients (16 males, 16 females). Age of onset ranged between 2.5 and 70 years. The majority of patients (n=15) were classified as type I (47%) followed by type III (n=9, 28%) and type IV (n=6, 19%). Based on treatment responses, retinoids appear to be very effective in our population as all patients treated with isotretinoin or acitretin had excellent response. In addition to checkerboard alternating orthokeratosis/parakeratosis, which was observed in 31 cases, interesting features, including the presence of follicular plugging in all 21 cases in which follicles were available for examination, eosinophils in 12 cases, and focal acantholysis in three cases were observed. Features of patients with PRP in our study are generally comparable to those published in the literature, with minor differences. Microscopically follicular plugging, in addition to checkerboard alternating orthokeratosis/parakeratosis, may serve as clues to PRP diagnosis. The presence of eosinophils and focal acantholysis, observed in a few cases, should not exclude PRP diagnosis.
    International journal of dermatology 04/2014; 53(4):434-9. DOI:10.1111/j.1365-4632.2012.05811.x · 1.23 Impact Factor
  • Piel 01/2013; 28(1):20–30. DOI:10.1016/j.piel.2012.05.002