Pseudoporfiria asociada a hemodialisis / Pseudoporfiria associated with hemodialysis

La pseudoporfiria es una dermatosis ampollar poco frecuente con similares caracter¡sticas cl¡nicas e histopatol¢gicas a las de la porfiria cut nea tarda pero con cifras normales de porfirinas. Se presenta el caso de una paciente con pseudoporfiria asociada a hemodi lisis que fue tratada con N-acetilciste¡na.

The pseudoporphyria is a bullous dermatosis rare with similar clinical and histopathologic features to the ofporphyria cutanea tarda but with normal numbers of porphyrins. The case of a patient is presented with pseudoporphtyria associated with hemodialysis who was treated with N-acetylcysteine.

Consenso peruano de diagnóstico y tratamiento de la micosis fungoides y síndrome de Sezary / A peruvian consensus for the diagnosis and therapy of mycosis fungoides and Sézary

Introducción: varias revisiones y guías de manejo de Micosis Fungoides (MF) y Síndrome Sézary (SS) han sido publicados. Sin embargo, estrategias terapeúticas para esta entidad varían entre instituciones y países. Existen pocos estudios fase III que permitan tomar decisiones en el tratamiento de MF/SS. Por tanto éste es frecuentemente determinado por las experiencias institucionales. Objetivo: con el objeto de sumarizar la evidencia disponible y revisar las mejores prácticas, es que se realizó un Consenso Peruano llevado a caboen Setiembre 2008 para establecer guías de diagnóstico y tratamientode MF/SS. Resultados: este artículo revisa los criterios clínicos, histopatológicos y terapéuticos de MF/SS.

Background: Several reviews and guidelines on the management of Mycosis Fungoides (MF) and Sézary syndrome (SS) have been published; however, treatment strategies for patients with MF/SS vary from institution to institution and among countries. There are few phase III trials to support treatment decisions for MF/SS and treatment is often determined by institutional experience. Objetive: In order to summarise the available evidence and reviewbest practices, Peruvian Consensus met in September 2004 to establish guidelines for the diagnosis and treatment of MF/SS. Results: This article reviews hystopatologic, clinic and therapeutic criteria of MF/SS.

Tratamiento del linfoma cutáneo de células T con bexaroteno a dosis bajas en combinación con fototerapia o interferon alfa: comunicación de ocho casos tratados en el Hospital Edgardo Rebagliati Martins, 2003-2007, Lima, Perú / Therapy for cutaneous T-cell lymphoma using low-dose bexarotene combined with phototherapy or alpha-interferon: Report of eight cases treated in Hospital Nacional Edgardo Rebagliati-Martins, Lima, Peru between 2003 and 2007

Introducción: bexaroteno es un rexinoide aprobado en el tratamiento de estadios tempranos y avanzados del linfoma cutáneo de células T (LCCT). Caso Clínico: el presente reporte de casos mostramos los resultados del empleo de bexaroteno en dosis bajas más fototerapia o Interferon alfa 9 millones en el tratamiento del LCCT. Ocho pacientes fueron tratados , cinco fueron Micosis fungoides, dos Linfoma Epidermotrópico CD8 agresivo y uno fue un Síndrome Sézary. La respuesta global fue del 62.5 por ciento (5/8) y la duración media de respuesta fue de 20 meses. El bexaroteno en dosis bajas en combinación a fototerapia o interferon alfa 2a puede ser efectivo en el tratamiento del LCCT.

Introduction: Bexarotene is a rexinoid compound that is approved for use in the therapy for early and advanced stage cutaneous T-cell lymphoma (CTCL).Clinical Cases: We present in this report the results of the use of low-dose bexarotene plus phototherapy or alpha-interferon, nine million units, in the treatment of CTCL. Eight patients were treated, five had mycosis fungoides, two had CD-8 epidermothropic aggressive lymphoma. The overall response rate was 62.5 per cent (5/8), and the mean duration of response was 20 months. Low-dose bexarotene combined with phototherapy or alpha-interferon may be effective in the treatment of CTCL.

Discoid lupus erythematosus in children: clinical, histopathologic, and follow-up features in 27 cases.

Among 27 pediatric patients with a clinicopathologic diagnosis of discoid lupus erythematosus (DLE), 15 had localized cutaneous lesions and 12 had disseminated lesions. During a mean follow-up period of 36 months, seven patients (26%) developed systemic lupus erythematosus (SLE). Four of these patients were less than 10 years of age. No correlation was found between localized and disseminated lesions and evolution to SLE. Three of four patients with a positive family history for rheumatoid disease developed SLE (p < 0.05). Hyperpigmentation was significantly more frequent (p < 0.04) in children less than 10 years of age. There was a female predominance of 5:1 among patients less than 10 years of age. Our findings suggest that onset of DLE prior to 10 years of age does not indicate a greater risk of developing SLE. The occurrence of localized or disseminated lesions does not seem to influence the outcome.

Nevo epidérmico verrucoso lineal variedad epidermolítica: reporte de un caso / Lineal verrucous epidermal nevus, epidermolitic variant: report of a case

Presentamos el caso de una niña de dos años de edad con el diagnóstico de Nevus epidérmico verrucoso lineal extenso en el hemicuerpo izquierdo; que en la biopsia de piel evidenció hallazgos de la variedad epidermolítica del nevo epidérmico verrucoso lineal.

Are infantile seborrheic and atopic dermatitis clinical variants of the same disease?

BACKGROUND: The relationship between infantile seborrheic dermatitis (ISD) and infantile atopic dermatitis (IAD) is controversial. METHODS: Ninety-six children aged 2-12 months diagnosed with atopic dermatitis and a comparable control group of healthy children were evaluated. Demographic data, personal history of ISD and personal or family history of atopy was considered in both groups. RESULTS: A personal history of ISD was found in 49% of IAD cases and in 17% of controls (P < 0.05). CONCLUSIONS: Our result and those of the literature do not demonstrate a relationship between ISD and IAD. However, a number of cases of AD have an ISD-like clinical picture. It is probable that ISD is a syndrome and not a disease.

Manifestaciones cutáneas del dengue. Reporte de 92 casos en el Hospitalde Apoyo III Sullana - MINSA durante la epidemia enero a junio del 2001 / Dengue's cutaneous manifestations. Report of 92 cases at Hospital de Apoyo III Sullana - MINSA during epidemic between january to june 2001

En el presente estudio se revisan las historias clínicas de 92 pacientes con diagnóstico de Dengue hospitalizados durante la epidemia del 2001 en el Hospital de Apoyo III Sullana-MINSA con el objetivo de determinar la frecuencia del compromiso cutáneo en pacientes con dengue y si su presencia correlaciona o no con su severidad y evolución. Las manifestaciones dermatológicas estuvieron presentes en 50 de 92 pacientes y fueron: erupción cutánea, prurito corporal, petequias, congestión ocular y mucositis. Dichas variables no correlacionan con la severidad o evolución del cuadro clínico.