Afecciones perianales no infecciosas, no neoplásicas, del adulto / Noninfectious, non-neoplasic adult perianal affections

Las enfermedades perianales del adulto, de carácter no infeccioso y no neoplásico, son un motivo de consulta poco frecuente. Se caracterizan por la variedad de su etiología y de su sintomatología clínica, y plantean dificultad en el diagnóstico y en la terapéutica. El objetivo del presente trabajo es abordar una patología que plantea la necesidad de una intervención interdisciplinaria. Se incluyen consideraciones anatomopatológicas, clínicas y terapéuticas. (AU)

Noninfectious, non- neoplasic perianal affections are uncommon diseases. They are characterized by the variety of the etiology and clinical symptomatology, posing difficulty in diagnosis and therapeutics. The objective of this paper is to address a pathology that raises the need for interdisciplinary intervention anatomopathological, clinical and therapeutic considerations are included. (AU)

Necrolisis epidérmica tóxica: un paradigma de enfermedad crítica / Toxic epidermal necrolysis: a paradigm of critical illness

RESUMEN La necrolisis epidérmica tóxica es una reacción cutánea adversa de tipo inmunológico secundaria en la mayor parte de los casos a la administración de un fármaco. La necrolisis epidérmica tóxica, el síndrome de Steven Johnson y el eritema exudativo multiforme forman parte del mismo espectro de enfermedad. La mortalidad de la necrolisis epidérmica tóxica es alrededor del 30%. La fisiopatología de la necrolisis epidérmica tóxica es semejante en muchos aspectos a la de las quemaduras dérmicas superficiales. La afectación mucosa del epitelio ocular y genital se asocia con secuelas graves si no se trata de forma temprana. Se acepta en general que los pacientes con necrolisis epidérmica tóxica son tratados mejor en unidades de grandes quemados, donde existe experiencia en el manejo de enfermos con pérdida cutánea extensa. El tratamiento es de soporte, eliminación y cobertura con derivados biosintéticos de la piel de las zonas afectadas, tratamiento de la afectación mucosa, y tratamiento inmunosupresor específico. De los tratamientos ensayados sólo se usa actualmente en la mayor parte de los centros la inmunoglobulina G y la ciclosporina A, aun cuando no existe evidencia sólida para recomendar ningún tratamiento específico. Entre los aspectos particulares del tratamiento de esta enfermedad se encuentra la prevención de secuelas relacionadas con la formación de sinequias, los cuidados oculares para prevenir secuelas graves que pueden conducir a la ceguera, y el tratamiento específico inmunosupresor. Un mejor conocimiento de los principios del manejo de la necrolisis epidérmica tóxica llevará a un mejor manejo de la enfermedad, a una mayor supervivencia y una menor prevalencia de las secuelas.

ABSTRACT Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.

Abordagem cutânea na necrólise epidérmica tóxica / Cutaneous approach in toxic epidermal necrolysis

Introdução: Necrólise epidérmica tóxica é uma erupção mucocutânea aguda grave, geralmente induzida por medicamentos, associada a alta taxa de morbidade e mortalidade. Os cuidados com as lesões mucosas e cutâneas e a abordagem multidisciplinar são muito importantes para o prognóstico e sequelas futuras. Objetivos: Discutir os principais aspectos dessa síndrome por meio da revisão de literatura, ilustrada por um caso clínico. Métodos: Revisão de literatura utilizando bases de dados on-line PubMed e Scielo. Incluímos artigos em língua inglesa, portuguesa, francesa e espanhola, e ilustração com caso clínico pediátrico. Termos procurados foram "toxic epidermal necrolysis", "Stevens-Johnson overlap", ''necrólise epidérmica tóxica'', ''síndrome Stevens-Johnson''. Resultados: Apresentamos dados para guiar o manejo de pacientes com necrólise epidérmica tóxica para cirurgiões plásticos, pediatras, intensivistas, dermatologistas e emergencistas. O caso tratado teve evolução favorável, sem sequelas cutâneas. Conclusão: O alto nível de suspeição é imprescindível para um diagnóstico e estratificação de risco adequados e instituição precoce de medidas de suporte, e o tratamento deve ser realizado por uma equipe multidisciplinar treinada para reduzir sequelas e mortalidade.

Introduction: Toxic epidermal necrolysis is a severe acute mucocutaneous condition usually induced by drugs associated with a high rate of morbidity and mortality. The care of the mucous lesions and skin and a multidisciplinary approach are very important for the prognosis and future sequelae. Objectives: To discuss the main aspects of this syndrome through a literature review illustrated by a clinical case. Methods: Review of the literature using the PubMed and SciELO online databases was performed. Articles in English, Portuguese, French, and Spanish were included and illustrated with a pediatric clinical case. The keywords used were as follows: "toxic epidermal necrolysis," "Stevens-Johnson overlap," "necrólise epidérmica tóxica," and "síndrome Stevens-Johnson." Results: We presented data to guide the management of patients with toxic epidermal necrolysis for plastic surgeons, pediatricians, intensivists, dermatologists, and emergency physicians. The case treated had a favorable disease course without sequelae. Conclusion: A high level of suspicion is necessary for an adequate diagnosis and risk stratification, and early support measures and treatment should be performed by a multidisciplinary team trained to minimize damage and mortality.

Necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae / Toxic epidermal necrolysis associated with acute infection by Mycoplasma pneumoniae

El eritema multiforme, el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica representan diferentes manifestaciones de un mismo espectro de graves reacciones cutáneas idiosincrásicas a fármacos y, en menor medida, están asociados a agentes infecciosos. De estos últimos, Mycoplasma pneumoniae es uno de los más frecuentes. Se presenta el caso de una niña de 5 años, con una necrólisis epidérmica tóxica asociada a infección aguda por Mycoplasma pneumoniae, que comenzó con un cuadro febril acompañado de un exantema generalizado y compromiso de todas las mucosas. Se obtuvo serología IgM positiva para Mycoplasma pneumoniae y una biopsia inicial compatible con eritema multiforme mayor. La paciente fue tratada con corticosteroides, gammaglobulina intravenosa, plasmaféresis y estrictos cuidados para la prevención de sobreinfección y posibles secuelas. Después de 31 días de internación fue dada de alta hospitalaria.

Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis represent different manifestations of the same spectrum of severe idiosyncratic cutaneous reactions to drugs and to a lesser extent are associated with infectous agents. Among these, Mycoplasma pneumoniae is one of the most frequent. We report the case of a female patient aged 5 years, with a toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection, which begins with a fever accompanied by a generalized rash with involvement of the mucous membranes. IgM serology for Mycoplasma pneumoniae was positive and initial biopsy was compatible with erythema multiforme major. The patient was treated with corticosteroids, intravenous immunoglobulin, plasmapheresis and strict care to prevent superinfection and sequels. After 31 days of hospitalization the patient was discharged from hospital.

Steven Johnson Syndrome / Síndrome de Steven Johnson

Ulcerative vesiculobullous disorders are common in Dermatology and Oral Medicine. Diagnosis of these conditions is pretty effortless if they give a classic appearance as described in the literature. Steven Johnson syndrome is one such condition which is a type of erythema multiforme. It is a disorder involving mucous membrane, skin and even the multiple organs in severe form. Multiple etiologies persist so be acquainted with the cause and prohibit the root is crucial. But still drugs are considered to be common cause. Early management is vital as complications are serious for this condition even leading to death. We hereby report a typical case with classic appearance of Steven Johnson syndrome.

Doenças ulcerativas vésicobolhosas são comuns em Dermatologia e Medicina Oral. O diagnóstico destas condições é bastante fácil se apresentar uma aparência clássica, tal como descrito na literatura. Steven Johnson é um tipo de eritema multiforme. É uma desordem envolvendo as mucosas, pele e até múltiplos órgãos de forma grave. Múltiplas etiologias são apontadas como causa. Mas ainda os medicamentos são considerados como causas comuns. O tratamento precoce é vital, pois complicações são graves para essa condição, podendo levar à morte. Vimos por meio deste relatar um caso típico, com aparência clássica de Síndrome de Steven Johnson.

Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica / Stevens-Johnson syndrome and toxic epidermal necrolysis

El síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica son toxicodermias graves que forman parte de un mismo espectro de enfermedad y que, pese a su baja frecuencia, se asocian con una gran morbimortalidad. Aunque su fisiopatología, clasificación y manejo son controvertidos, se sabe que la apoptosis masiva de queratinocitos por diferentes vías se encuentra implicada. No deben confundirse con el eritema multiforme y hasta el momento no se ha demostrado la seguridad y eficacia de los tratamientos, excepto de las medidas de soporte que son el pilar del manejo. A continuación se presenta una revisión de la literatura sobre el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica.

Reacciones cutáneas adversas a anticonvulsivantes y estabilizadores del ánimo / Adverse cutaneous reactions to anticonvulsants and mood stabilizers

Anticonvulsivantes y estabilizadores del ánimo principalmente el ácido valproico, lamotrigina y carbamazepina, poseen una alta incidencia de reacciones adversas a medicamentos (RAM) severas, como eritema multiforme, Síndrome Stevens- Johnson y necrolisis epidérmica tóxica, asociadas. Existen signos de alarma para su sospecha diagnóstica precoz, que permiten indicar la temprana suspensión del fármaco sospechoso e iniciar la terapia de soporte únicas medidas que han demostrado una clara disminución en la mortalidad. La inmunoglobulina G intravenosa se recomienda por su seguridad, sin embargo, su rol en disminuir la mortalidad es contradictorio. Los corticoides no han demostrado cambios en la mortalidad comparados con la terapia de soporte exclusiva. Se ha intentado mantener el tratamiento con lamotrigina, por sus cualidades terapéuticas, pese a la aparición de RAM cutáneas. De hecho, en estudios recientes en pacientes que han desarrollado RAM leves a este producto se ha demostrado un éxito de reexposición de 85 por ciento-87 por ciento mediante una lenta titulación de la dosis.

Anticonvulsants and mood stabilizers mainly valproic acid, lamotrigine and carbamazepine are medications that have a high incidence of severe adverse drug reactions (ADRs), such erythema multiforme, Stevens- Johnson syndrome and toxic epidermal necrolysis. Early diagnosis based in systemic and cutaneous alarm signs have been described, allowing premature discontinuation of suspected drugs and start supportive therapy; these are the only measures that have that have shown clear reduction in mortality. The use of intravenous immunoglobulin G is recommended for their safety, but studies regarding their role in reducing mortality are conflicting. Corticosteroids have not proved changes in mortality compared with exclusive supportive care. Due to therapeutic quality Lamotrigine is used despite the incidence of ADRs. In fact in recent studies patients with mild ADRs to this drug have shown between 85 percent-87 percent of success, when patients are re-exposed through a slow increasing in dosage.

Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica: experiencia clínica en el Hospital Nacional Guillermo Almenara Irigoyen, Lima - Perú / Stevens-Johnson Syndrome and toxic epidermal necrolysis: clinical experience at the Hospital Nacional Guillermo Almenara Irigoyen, Lima-Peru

Objetivo: Describir las características clínicas de un grupo de pacientes diagnosticados de síndrome de Stevens-Johnsons (SSJ) y necrólisis epidérmica tóxica (NET). Material y métodos: Estudio retrospectivo, transversal y descriptivo realizado en el Hospital Nacional Guillermo Almenara Irigoyen entre junio de 2004 y mayo del 2007. Se revisó las historias clínicas de los casos hospitalizados, recogiendo la información sobre drogas responsables, tratamiento y resultado final. Resultados: Se estudiaron siete pacientes, cinco con diagnóstico de NET, uno con SSJ y uno con síndrome SSJ-NET. Cinco pacientes fueron mujeres y dos varones, con una edad media de 47 años.Los fármacos asociados fueron los anticonvulsivantes (fenitoína y lamotrigina) en tres pacientes, metamizol en dos, cotrimoxazol en uno y fluconazol también en uno. La mucosa oral fue afectada en todos los casos, la mucosa genital en cuatro y la mucosa conjuntival en tres. El tratamiento de elección fueron los corticoides en seis pacientes y se inicio de forma rápida en cuatro, evolucionando con mínimas complicaciones y solo uno de ellos presento secuelas oculares. Solo hubo un deceso en este grupo. Conclusiones: El SSJ y la NET en nuestros casos fueron causados principalmente por anticonvulsivantes y metamizol, fueron tratados en su mayoría con corticoides sistémicos. Drogas de uso creciente como fluconazol y lamotrigina también deben considerarse entre los medicamentos de riesgo.

Exploiting the igloo principle and greenhouse effect to regulate humidity and temperature.

BACKGROUND: Toxic epidermal necrolysis can be fatal and nursing care with careful monitoring of temperature and humidity can improve survival rate. We adapted the greenhouse and igloo principle using a common hood to monitor the temperature and humidity. METHODS: A small heater with a regulator was placed in a mini hood and temperature was recorded inside the uncovered hood and hood covered with green cloth and aluminium foil separately. The regular hood was placed over a volunteer and the temperature was measured inside the open hood and hood covered with green cloth and aluminium foil separately. The relative humidity was also monitored using Zeal mercury dry--wet bulb hygrometer. RESULTS: Temperature increase was most marked in the foil-covered hood followed by cloth-covered hood, both with the heater and the volunteer. Similarly, in the volunteer study, the humidity was best maintained inside the aluminium foil-covered hood. CONCLUSION: We recommend the use of regular hood with suitable cover to monitor the humidity and temperature of patients with toxic epidermal necrolysis.

Achieving asepsis of banana leaves for the management of toxic epidermal necrolysis.

BACKGROUND: Banana leaf is used in many centers in India during the care of patients with toxic epidermal necrolysis (TEN) and other extensive blistering disorders. Sepsis is an important cause of death in TEN patients and use of banana leaf may be a source of such infection. AIMS: We conducted this study to detect the bacterial flora of the banana leaf and to examine various methods of rendering the leaf aseptic. METHODS: Five pieces of banana leaf, 2 x 2 cm in size, were cultured separately in blood agar as follows: One piece was heated over a flame and one was soaked in boiling water and one was autoclaved. Methylated spirit was applied over one piece and ignited. One piece was placed on the media, 'as is.' The Petri dishes were incubated examined after 48 h. RESULTS: All the pieces except the autoclaved specimen of the leaf grew coagulase-negative staphylococci (CONS) when aseptic precautions were not maintained and aerobic spore bearers when all aseptic measures were subsequently instituted during the procedure. CONCLUSION: We recommend measures to prevent possible transmission of bacterial infection by the leaf. Autoclaved and aseptically handled banana leaves may be used to reduce chance of infection in the treatment of TEN.

Efficacy of low dose intravenous immunoglobulins in children with toxic epidermal necrolysis: an open uncontrolled study.

BACKGROUND: High dose intravenous immunoglobulins (IVIG) have emerged as a promising new therapy for treating the rare but potentially fatal drug reaction toxic epidermal necrolysis (TEN). Experimental in vitro studies support the view that IVIG can block the fas-fas ligand mediated apoptosis in TEN. METHODS: Ten pediatric patients of TEN were treated with IVIG (0.05 - 0.1 gm/kg/day) along with antibiotics and supportive care. RESULTS: Patients with 67% of mean body surface area of involvement showed an average of 2.1 days for arrest of progression of lesions and 8.1 days for complete reepithelization. There was no mortality. CONCLUSIONS: Low dose IVIG appears to be a safe and effective treatment for TEN in children. Randomized trials are needed to further evaluate the efficacy of IVIG and compare it with other therapeutic modalities.

Uso de inmunoglubolina en el manejo del Síndrome de Stevens-Johnson. Reporte de un caso / Use of immunoglubolin in the handling of the Stevens-Johnson Syndrome. Report of a case

El uso de inmunoglobulina humana se ha descrito en el tratamiento de pacientes con síndrome de Stevens-Johnson. La inmunoglobulina bloquea la apoptosis de los keratinocitos inducida por la interacción del complejo Fas-FasL. No hay estudios clínicos controlados que recomienden el uso rutinario, la dosis y la eficacia de este tratamiento para el síndrome de Stevens-Johnson. Se describe el caso de un paciente de 18 años con un Síndrome de Stevens Johnson secundario a tetraciclinas, que se trató exitosamente con inmunoglobulina intravenosa. Descriptores: Inmunoglobulina, Síndrome Stevens-Johnson.

Eritema multifome - atualizações / Erythema multiforme - an update

O eritema multiforme ou polimorfo é um processo inflamatório agudo que apresenta lesões cutâneo-mucosas características. Representa uma enfermidade de interesse para Odontologia, uma vez que, por vezes, a boca pode ser a única área afetada. O objetivo deste trabalho é apresentar uma revisão da literatura destacando os aspectos relacionados à etiologia, características clínicas e histológicas, diagnóstico, tratamento e prognóstico da referida condição patológica

Síndrome de Stevens-Johnson / Syndrome of Stevens Johnson

Es una variante severa del Eritema Multiforme (EM), consistente en una reaccion de hipersensibilizacion que afecta la piel y las membranas mucosas. Adicionalmente pueden quedar implicados algunos otros organos.(au)