Eritema multiforme, una rara presentación de exantema en población pediátrica: a propósito de un caso / Erythema multiforme, a rare occurrence of exanthema in the pediatric population: on the subject of a case

El Eritema multiforme (EM) o eritema polimorfo es una enfermedad aguda de la piel de naturaleza inmunológica con o sin compromiso de mucosas, que puede comportarse como crónica recurrente. Se presenta con lesiones cutáneas en diana distintivas, a menudo acompañado de úlceras o bullas en mucosas (oral, genital u ocular). Entre sus formas clínicas se distingue: una forma menor caracterizado por un síndrome cutáneo leve y su forma mayor que se manifiesta como una afectación cutánea con daño mucoso marcado. Entre sus principales diagnósticos diferenciales se encuentran el Síndrome de Stevens-Johnson (SSJ) y Síndrome de Lyell (Necrólisis epidérmica tóxica (NET)). Tiene una incidencia estimada < 1%, siendo su forma mayor levemente más frecuente que su forma menor (0.8-6 por millón/año). Puede darse a cualquier edad, presentando un peak de incidencia entre los 20 y 30 años, predominando ligeramente el sexo masculino con una proporción 3:2, sin predilección racial. Su presentación en edad pediátrica es rara, más aún en la primera infancia. En esta población es más frecuente el EM menor recurrente. En el presente texto se reporta un caso de EM en población pediátrica como una rara forma de presentación exantemática, abordado en el Servicio de Pediatría del Complejo Asistencial Dr Victor Rios Ruiz (CAVRR)en la ciudad de Los Ángeles, Chile en el presente año.

Erythema multiforme (EM) also known as polymorph erythema is an acute skin disease of immunological nature with or without mucous membrane involvement, which may behave as chronic recurrent. It presents with distinctive targets like skin lesions, often together with ulcers or bullae in mucous membranes (oral, genital or ocular). Among its clinical forms are: a minor form characterized by a mild skin syndrome and its major form that manifests as a skin disease with marked mucosal damage. Among its main differential diagnoses are Stevens-Johnson Syndrome (SJS) and Lyell Syndrome (Toxic Epidermal Necrolysis (TEC)). It has an estimated incidence < 1%, with its major form being slightly more frequent than its minor form (0. 8-6 per million/year). It can occur at any age, presenting a peak incidence at the age between 20 and 30 years, with a slight predominance of males with a 3:2 ratio, without racial predilection. Its presentation in pediatric age is rare, even more so in early childhood. Minor recurrent EM is more common in this population. This paper reports a case of EM in the pediatric population as a rare form of exanthematic presentation, addressed at the Department of Pediatrics of the Complejo Asistencial Victor Rios Ruiz (CAVRR) in the city of Los Angeles, Chile this year.

Conjuntivitis cicatrizal recalcitrante: Tratamiento de nueve pacientes con rituximab / Recalcitrant cicatrizing conjunctivitis. Treatment of nine patients with rituximab

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients

Abstract: Background: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term 'Epidermal Necrolysis' has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for 'Epidermal Necrolysis' cases that still needed validation in acquired immunodeficiency syndrome. Objective: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. Methods: Retrospective cohort study of AIDS and non-AIDS 'Epidermal Necrolysis' cases admitted to a Brazilian reference center from 1990-2014. Results: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. Study Limitations: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. Conclusion: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.

Síndrome de Stevens-Johnson en edad pediátrica / Stevens Johnson Syndrome of a Pediatric Age Case

El síndrome de Stevens-Johnson (SSJ) es una enfermedad inflamatoria aguda, originada por una reacción de hipersensibilidad, secundaria a ingesta de medicamentos o infecciones, que afecta a la piel y las membranas mucosas produciendo lesiones características del síndrome, causadas por apoptosis y posterior necrosis de los queratinocitos; su forma más severa es la necrolisis epidérmica tóxica, que constituye junto al SSJ un espectro de la misma enfermedad, compartiendo aspectos etiológicos, patogenéticos, histológicos y terapéuticos que ponen en peligro la vida del paciente. La afección se caracteriza por una súbita erupción morfológicamente variable, acompañada de estomatitis y oftalmia.En el presente trabajo se presenta el caso de un niño de 8 años de edad,con diagnóstico clínico de síndrome de Stevens-Johnson, con manifestaciones cutáneas, oculares y de la mucosa oral, que iniciaron posterior a ingesta de ibuprofeno, se mantuvo con un protocolo de cuidados que incluyeron soporte de oxígeno, antibioticoterapia, analgesia, corticoides, nebulizaciones, limpieza de lesiones con solución salina, sin debridación y lubricante oftálmico; tras 8 días dehospitalización el paciente evoluciona satisfactoriamente sin complicaciones durante su estancia hospitalaria.

The Stevens-Johnson syndrome (SJS) is an acute inflammatory disease caused by a hypersensitivity reaction, secondary to medication intake or infections, that affects skin and mucous membranes producing characteristic wounds of the syndrome, caused by apoptosis and subsequent necrosis of keratinocytes; the major form of this disease, is toxic epidermal necrolysis, wich together with SJS is a spectrum of the same disease, sharing etiological pathogenetic, histological and therapeutic aspects, that endanger the patient's life. The affection is characterized by a sudden morphologically varying rash, accompanied bystomatitis and ophthalmic injure. In this work we show an 8 year old patient with a clinical diagnosis of Stevens-Johnson syndrome, involving skin, eye and oral mucosa manifestations, which began after the intake of ibuprofen , it was mantained with a protocol care based on oxygen support, antibiotic therapy, analgesia, corticosteroids, sprays, cleansing wounds with saline solution without debridement and ophthalmic lubricant, after 8 days of hospitalization, our patient has a satisfactory evolution without acute complications during their time at the hospital.

Cutaneous drug reactions in patients admitted to the dermatology unit at the University Hospital of the West Indies, Kingston, Jamaica / Reacciones cutáneas medicamentosas en pacientes ingresados en la Unidad de Dermatología del Hospital Universitario de West Indies, Kingston, Jamaica

OBJECTIVE: Cutaneous reactions are among the most common adverse reactions to drugs. The purpose of this study is to examine the aetiology and outcome of cutaneous drug reactions among patients admitted to the Dermatology Ward at the University Hospital of the West Indies. SUBJECTS AND METHODS: This was a retrospective study looking at all patients who were admitted with a diagnosis of a cutaneous drug eruption from January 1, 1997 to December 31, 2005. Data included patient demographics, date of admission to hospital, duration of hospitalization and a detailed drug history including any previous episodes of drug sensitivity. All drugs reportedly ingested by the patients up to three months prior to their cutaneous reaction were documented and the period of time between drug ingestion and the appearance of skin lesions was also noted. Clinical diagnosis, co-morbidities, histopathological diagnosis, final outcome and all ensuing disabilities were noted. The data retrieved were collated and analyzed using SPSS 12.0. RESULTS: The results showed a female to male ratio of 2.2:1. The categories of drugs most commonly implicated were antimicrobials followed by anti-epileptic drugs and nonsteroidal anti-inflammatory drugs. The most common form of drug eruption requiring admission was the exanthematous drug eruption followed by erythema multiforme, toxic epidermal necrolysis and Stevens-Johnson syndrome. CONCLUSION: In general, the causative agents identified and the types of drug eruptions were similar to those found in previous studies. However, the anti-epileptic drugs, phenytoin and carbamazepine, ranked among the most commonly implicated drugs which differ significantly from other studies.

Las reacciones cutáneas se hallan entre las reacciones adversas más comunes frente a los medicamentos. El propósito de este estudio fue examinar la etiología y la evolución clínica de las reacciones cutáneas medicamentosas entre pacientes ingresados a la sala de dermatología en el Hospital Universitario de West Indies. MÉTODOS: Este es un estudio retrospectivo que pasa revista a todos los pacientes que fueron ingresados con diagnóstico de erupción cutánea desde el 1ero. de enero de 1997 al 31 de diciembre de 2005. RESULTADOS: Los resultados mostraron una proporción hembra-varón de 2.2:1. Las categorías de los medicamentos más frecuentemente implicados fueron los antimicrobianos, seguidos por los medicamentos antiepilépticos y los antiinflamatorios no esteroideos. La forma más común de erupción que requirió ingreso a causa de medicamentos, fue la erupción exantemática medicamentosa seguida por el eritema multiforme, la necrólisis epidérmica tóxica, y el síndrome de Stevens-Johnson. CONCLUSIÓN: En general, los agentes causativos identificados y los tipos de erupciones medicamentosas, fueron similares a los hallados en estudios previos. Sin embargo, los antiepilépticos conocidos como fenitoína y carbamazepina, estuvieron entre los medicamentos más comúnmente implicados, presentándose en tal sentido una diferencia significativa con los otros estudios.

Transplante de membrana amniótica em casos agudos graves de queimadura ocular química e síndrome de Stevens-Johnson / Amniotic membrane transplantation for severe acute cases of chemical ocular burn and Stevens-Johnson syndrome

OBJETIVO: Investigar o potencial terapêutico do transplante de membrana amniótica nos casos agudos graves de queimadura ocular química e síndrome de Stevens-Johnson. MÉTODOS: Foram analisados retrospectivamente os prontuários de oito pacientes, com um total de dez olhos, submetidos a transplante de membrana amniótica para tratamento de queimadura ocular química e síndrome de Stevens-Johnson na fase aguda entre janeiro de 1999 e maio de 2008 no Departamento de Oftalmologia da UNIFESP. Dados referentes a sexo, idade, grau da queimadura, etiologia, olho acometido, achados oftalmológicos, extensão da membrana amniótica, operações adicionais, tempo em dias entre a lesão e a cirurgia, acuidade visual antes e depois da cirurgia, defeito epitelial em dias, complicações e tempo de seguimento em meses foram coletados. RESULTADOS: A idade média dos pacientes foi de 35,7 ± 23,04 anos, dos quais seis eram homens e dois eram mulheres. Três pacientes (quatro olhos) apresentaram síndrome de Stevens-Johnson e cinco pacientes (seis olhos) apresentaram queimadura ocular química. O defeito epitelial foi cicatrizado em média de 27,8 ± 4,7 dias (variando de 20 a 35 dias). Todos os pacientes evoluíram com deficiência límbica em seguimento médio de 7,8 ± 2,8 meses (variando entre cinco e doze meses) e quatro olhos desenvolveram simbléfaro. CONCLUSÕES: Os resultados sugerem que o transplante de membrana amniótica representa um aditivo que pode ser realizado nos casos agudos graves de queimadura ocular química e síndrome de Stevens-Johnson com a finalidade de promover a epitelização e suprimir a inflamação e suas consequências, se comparado a outros trabalhos que trataram casos semelhantes com terapia medicamentosa somente. Por outro lado, não é capaz de evitar a deficiência límbica nesses casos, que futuramente necessitarão de transplante de células-tronco do limbo ou outras cirurgias para correção da superfície ocular.

PURPOSE: To study the therapeutic potential of amniotic membrane transplantation in cases of severe acute chemical ocular burn and Stevens-Johnson syndrome. METHODS: We retrospectively reviewed the records of eight patients, with a total of ten eyes, submitted to amniotic membrane transplantation for treatment of ocular chemical burns and Stevens-Johnson syndrome in the acute phase between January 1999 and May 2008 in the Ophthalmology Department of UNIFESP. Data relating to sex, age, degree of chemical burns, etiology, affected eye, ophthalmological findings, extension of amniotic membrane, surgeries, additional time between the injury and surgery in days, visual acuity before and after surgery, epithelial defect healing (days), complications and time of follow-up in months were collected. RESULTS: The average age of patients was 35.7 ± 23.04 years, with six men and two women. Three patients (four eyes) had Stevens-Johnson syndrome and five patients (six eyes) had chemical ocular burn. The epithelial defect was healed at an average of 27.8 ± 4.7 days (ranging from 20 and 35 days). All patients presented limbal stem cell deficiency in a median follow-up of 7.8 ± 2.8 months (ranging from six and twelve months) and four eyes developed symblepharon. CONCLUSIONS: The results suggest that the amniotic membrane transplantation represents an additive that can be carried out in the serious cases of ocular chemical burn and Stevens-Johnson syndrome with the finality of promoting the epithelialization and abolishing the inflammation and its consequences, if compared with other studies that treated similar cases with medical therapy only. On the other hand, it is not possible to avoid the limbic deficiency in these cases, which in the future will need limbal stem cell transplantation or other surgeries for correction of the ocular surface.

Necrólise epidérmica tóxica (Síndrome de Lyell)? Apresentaçäo de um caso clínico / Necrólise epidérmica tóxica. Síndrome de Lyell. Hipersensibilidade a drogas

Os autores fazem uma breve revisäo de literatura e apresentam o caso clínico de uma paciente do sexo feminino, de 73 anos de idade, acometida pela necrólise epidérmica tóxica (NET) ou síndrome de Lyell. A paciente foi internada no Hospital Nossa Senhora da Saúde na cidade de Diamantina/MG. A evoluçäo médica é descrita, bem como o protocolo para o tratamento dessa enfermidade. Uma vez que o tratamento dessa síndrome exige a participaçäo de uma equipe multidisciplinar, um cirurgiäo-dentista deveria ser incluído nessa equipe para avaliar as condiçöes bucais do paciente, visto que é uma entidade clínica com graves manifestaçöes na mucosa bucal

Exantemas febriles no infecciosos / Non infectious febrile rash

Exantema es una erupción de la piel con lesiones de distinto tipo, configuración y disposición. El diagnóstico diferencial para pacientes febriles con exantema es extenso. Las posibles causas son enfermedades infecciosas, drogas, enfermedades dermatológicas, inmunológicas y/o neoplásicas. Una historia detallada y un examen físico cuidadoso pueden ser esenciales para hacer un diagnóstico correcto. La historia debe incluir el sitio de comienzo, porcentaje y dirección de extensión, presencia o ausencia de prurito y relación temporal con la fiebre. En este artículo se revisarán diagnósticos no infecciosos de exantemas febriles