Dental management in the Intensive Care Unit in the treatment of toxic epidermal necrolysis associated with phenytoin: case report / Manejo odontológico na Unidade de Terapia Intensiva no tratamento da necrólise epidérmica tóxica associada a fenitoína: relato de caso

ABSTRACT Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are adverse reactions, usually medicine-related, in which extensive and painful mucocutaneous lesions are formed. Oral lesions are characterized by the presence of erosions and ulcers. The treatment usually requires intensive care in a hospital setting, where the Dentist is essential in the multidisciplinary team, as he will act in the treatment of oral lesions. In this article, a 26-year-old female patient was reported, who presented TEN-compatible mucocutaneous lesions associated with the drug Phenytoin and required admission to a Teaching Hospital. The treatment consisted of the immediate suspension of the Phenytoin use and systemic therapy with corticosteroids and immunosuppressants. Oral lesions were monitored by the hospital dentistry team. The procedures adopted were: oral hygiene with 0.12% non-alcoholic Chlorhexidine Digluconate solution, hydration of the oral tissue with an essential fatty acid, application of frozen chamomile tea, and prescription of topical Triamcinolone Acetonide. Also, low-level laser therapy sessions (red and infrared) were performed. Oral lesions evolved with significant improvement. In this case, the importance of the Dentist in the multi-professional team is demonstrated. Although there is no protocol, the procedures used in the present case can be an alternative to therapies used in SSJ and TEN oral lesions.

RESUMO A Síndrome de Stevens Johnson (SSJ) e a Necrólise Epidérmica Tóxica (NET) são reações adversas, geralmente medicamentosas. Na pele, formam-se máculas que se tornam pápulas, vesículas e bolhas, além de eritema extenso e desprendimento da epiderme. As lesões bucais caracterizam-se pela presença de erosões e úlceras dolorosas. O tratamento geralmente é realizado em âmbito hospitalar, com necessidade de cuidados intensivos, onde o Cirurgião-dentista é fundamental na equipe multiprofissional, pois atuará na terapêutica das lesões bucais. Neste artigo foi relatado o caso de uma paciente do sexo feminino, de 26 anos, a qual apresentou lesões mucocutâneas compatíveis com NET associadas ao medicamento Fenitoína e necessitou de internamento em um Hospital Universitário. O tratamento realizado foi a suspensão imediata do fármaco desencadeador, além de terapia sistêmica com corticóide e imunossupressor. As lesões bucais foram acompanhadas pela equipe de odontologia hospitalar. As condutas adotadas foram: higiene bucal com solução de Digluconato de Clorexidina 0,12% não alcoólica, hidratação da mucosa bucal com Ácido Graxo Essencial, aplicação de raspas de chá de gelo de camomila e prescrição de Triancinolona Acetonida tópica. Além disso, foram realizadas sessões de laserterapia de baixa intensidade (vermelho e infravermelho). As lesões bucais evoluíram com melhora significativa. A paciente permaneceu internada durante 22 dias, sendo 12 em Unidade de Terapia Intensiva. Este caso demonstra a importância do Cirurgião-dentista na equipe multiprofissional. Apesar de não haver um protocolo para as lesões bucais, as condutas utilizadas no presente caso podem ser uma alternativa de terapêuticas utilizadas em lesões de SSJ e NET.

Stevens-Johnson syndrome and herpes simplex reactivation associated with paracetamol use. Case report / Síndrome de Stevens-Johnson con reactivación de herpes simple asociado a la ingesta de paracetamol. Reporte de caso

Abstract Introduction: Stevens-Johnson syndrome (SJS) is a rare disease that affects the skin, as well as the oral, ocular, and urogenital mucous membranes. This condition is caused by drug reactions in more than 50% of cases. Case presentation: A 20-year-old male visited the emergency service of a tertiary care hospital of Popayán due to a 10-day history of asthenia, adynamia, fever (without objective measurement records), cough with scarce whitish sputum, and headache that improved with paracetamol treatment. However, his condition worsened in the last two days, and he developed hyporexia, pharyngeal pruritus, vesicles, and blisters on the corners of the mouth, the oral mucosa, the trunk, and limbs, as well as breathing difficulty and odynophagia, prompting him to seek medical treatment at the ER. At first, disseminated herpes simplex, systemic lupus erythematosus and SJS were suspected, but based on clinical and laboratory findings, the patient was finally diagnosed with SJS with herpes simplex reactivation associated with the use paracetamol. Consequently, the administration of this drug was stopped and management with acyclovir and methylprednisolone was started. The patient progressed satisfactorily and was discharged 10 days after beginning the new treatment, and his general condition was optimal during follow-up appointments. Conclusion: The occurrence of SJS may be associated with the oral administration of paracetamol; nevertheless, its use is not discouraged due to its great overall benefits. In this sense, given that paracetamol is an over-the-counter drug widely used in Colombia, recognizing the clinical manifestations of SJS is essential to provide adequate management and avoid complications in cases such as the one reported here.

Resumen Introducción. El síndrome de Stevens-Johnson (SJS) es una enfermedad poco común que afecta la piel y las mucosas oral, ocular y urogenital; además, en más del 50% de los casos es producida por reacciones a medicamentos. Presentación del caso. Hombre de 20 años quien asistió al servicio de urgencias de un hospital de tercer nivel de Popayán (Colombia) por un cuadro clínico de 10 días de evolución consistente en astenia, adinamia, fiebre no cuantificada, tos con escasa expectoración blanquecina y cefalea, sintomatologia que mejoraba con el uso de paracetamol; sin embargo, la condición del paciente empeoró en los últimos dos días, presentando hiporexia, prurito en faringe, vesículas y ampollas en comisuras labiales, mucosa oral, tronco y extremidades, además de dificultad respiratoria y odinofagia, razón por la cual acudió al servicio. En principio de sospechó de herpes simple diseminado, lupus eritema-toso sistêmico, y SJS; sin embargo, con base en los hallazgos clínicos y de laboratorio se confirmó el diagnóstico de SJS con reactivación de herpes simple asociado a la ingesta de paracetamol, por lo que se suspendió este medicamento y se inició manejo con aciclovir y metilprednisolona. El paciente tuvo una evolución satisfactoria y fue dado de alta a los 10 días del inicio del nuevo tratamiento y en las citas de control su condición general era óptima. Conclusiones. El desarrollo de SJS puede estar asociado al consumo de paracetamol; sin embargo, su uso no se desaconseja gracias a sus grandes bondades y beneficios generales. En este sentido, dado que el paracetamol es un medicamento de venta libre y uso extenso en Colombia, es indispensable reconocer las manifestaciones clínicas del SJS para poder dar un manejo adecuado y evitar complicaciones en casos como el aquí reportado.

Reacciones cutáneas adversas graves en la infancia / Severe adverse skin reactions in the childhood

RESUMEN Las reacciones cutáneas a drogas son cada vez más frecuentes en edades pediátricas, con un alto impacto en la salud de los niños. Pueden manifestarse en formas muy disímiles, desde un exantema transitorio hasta cuadros graves con afectación multisistémica potencialmente fatales. En la presente revisión se hace énfasis en las farmacodermias graves en la infancia, con el objetivo de promover el conocimiento por parte del personal médico para facilitar su diagnóstico y tratamiento oportuno. Se desarrolló una búsqueda en la Biblioteca Virtual de Salud de Infomed y en Google: se revisaron 28 trabajos científicos sin limitación de año y país, 24 de ellos pertenecen a los últimos cinco años y de estos 17 a los últimos tres. El dominio de los elementos para el diagnóstico precoz y las opciones terapéuticas son indispensables para elegir la conducta adecuada frente a estas reacciones cutáneas graves y disminuir la morbimortalidad por estas afecciones (AU).

ABSTRACT Skin reactions to drugs are increasingly common at pediatric ages, with a high impact on children's health. They can appear in very dissimilar forms, from a transient rash to serious pictures with potentially fatal multisystem involvement. This review focuses on severe pharmacodermies in the childhood, with the aim of promoting medical staff knowledge to facilitate their timely diagnosis and treatment. A search was led in the Infomed Virtual Health Library and in Google: 28 scientific papers were reviewed without limitation of year and country, 24 of them belong to the last five years and from these 17 to the last three. Mastery of the elements for early diagnosis and therapeutic options are indispensable to choose the appropriate behavior against these serious skin reactions and to decrease morbidity and mortality due to these conditions (AU).

Toxic epidermal necrolysis associated with anticonvulsants / Necrólisis epidérmica tóxica asociada a anticonvulsivantes

ABSTRACT Background and Objectives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as a continuum of the same process. TEN or Lyell Syndrome is the most severe form. Both entities involve an acute mucocutaneous blistering reaction associated with systemic inflammation. Materials and Methods: We present a case of a young woman who developed TEN following concomitant treatment with valproate, lamotrigine, and phenobarbital. Despite the extensive mucocutaneous detachment (over 90%), prognostic evaluation was favorable (SCORTEN score 2; probability of survival 88%), and this patient evolved satisfactorily. Five days after admission, valproate was reinitiated without any subsequent adverse reaction. Results: Causality evaluation identified both lamotrigine and phenobarbital as "very probable" (ALDEN score = 6) causes and valproate as "very unlikely" (ALDEN score = 0) cause of TEN. Conclusions: SJS and TEN are true life-threatening medical emergencies. This case emphasizes the importance of early diagnosis and treatment, including the discontinuation of the causative agent, which can be lifesaving.

RESUMEN Antecedentes y objetivos: El síndrome de Stevens-Johnson (SSJ) y la necrólisis epidérmica tóxica (NET) se consideran un continuum del mismo proceso. La NET o síndrome de Lyell es la forma más grave. Ambas entidades implican una reacción ampollosa mucocutánea aguda asociada con inflamación sistémica. Materiales y métodos: Presentamos el caso de una mujer joven que desarrolló NET posterior al tratamiento concomitante con valproato, lamotrigina y fenobarbital. A pesar del extenso desprendimiento mucocutáneo (más del 90%), la evaluación pronóstica fue favorable (puntuación SCORTEN 2; probabilidad de supervivencia 88%), y esta paciente evolucionó satisfactoriamente. Cinco días después de su ingreso, se reinició el valproato sin ninguna reacción adversa posterior. Resultados: La evaluación de causalidad identificó tanto la lamotrigina como el fenobarbital como causas "muy probables" (puntuación ALDEN = 6) y el valproato como causas "muy improbables" (puntuación ALDEN = 0) de NET. Conclusiones: El SJS y la NET son verdaderas emergencias médicas potencialmente letales. Este caso enfatiza la importancia del diagnóstico y tratamiento tempranos, incluida la interrupción del agente causal, lo cual puede salvar la vida del paciente.

Necrólisis epidérmica tóxica en una adolescente diagnosticada de trastorno bipolar II y tratada con lamotrigina / Toxic epidermal necrolysis in an adolescent with Bipolar Disorder II and treated with Lamotrigine

Resumen La necrólisis epidérmica tóxica es una enfermedad cutánea severa, la mayoría de las veces desencadenada como reacción adversa a medicamentos, con alta morbilidad y mortalidad. La lamotrigina, junto a otros medicamentos estabilizadores del ánimo, constituye la causa medicamentosa más frecuente de esta complicación, consistente en necrosis y esfacelo de la epidermis y mucosas en más del 30 % de la superficie corporal, con la consecuente pérdida de líquidos y electrolitos, respuesta inflamatoria sistémica, susceptibilidad a infecciones y hasta sepsis, además de posibles secuelas ominosas. En la actualidad, el diagnóstico de trastorno bipolar se hace con mayor frecuencia, incluyendo al grupo etario de niños y adolescentes, pero tal proceso diagnóstico se caracteriza por dificultades y controversias en mayor cuantía que otros diagnósticos psiquiátricos. Ello obliga a una meticulosa elucubración diagnóstica y selección farmacológica, con pleno conocimiento de las moléculas del arsenal medicamentoso para, en caso de prescripción de lamotrigina, establecer una escrupulosa psicoeducación al paciente y sus familiares además de un seguimiento estricto y cercano. A propósito del caso de una adolescente diagnosticada de trastorno bipolar II, que recibió lamotrigina durante un episodio depresivo pero con un esquema posológico inadecuado, y desarrolló necrólisis epidérmica tóxica, revisamos y comentamos la literatura correspondiente. Concluimos en que es preciso extremar las precauciones al decidir el uso de lamotrigina para minimizar el riesgo de este severo efecto adverso.

Toxic epidermal necrolysis is a severe skin disease, most often triggered as an adverse drug reaction, with high morbidity and mortality. Lamotrigine, together with other mood stabilizer drugs, constitutes the most frequent drug that causes this complication, which consists of necrosis and detachment of the epidermis and mucosa in more than 30% of the body surface, with the consequent loss of water and electrolytes, systemic inflammatory response, susceptibility to infections and even sepsis, in addition to other possible ominous sequelae. Currently, the diagnosis of bipolar disorder is made more frequently, including the age group of children and adolescents, but such a diagnostic process is characterized by difficulties and controversies to a greater extent than other psychiatric diagnoses. This requires meticulous diagnostic process and pharmacological selection, with full knowledge of the molecules in the drug arsenal so, in case of lamotrigine prescription, it should be established scrupulous psychoeducation to the patient and their family members, as well as strict and close follow-up. A propos of the case of an adolescent girl diagnosed with bipolar II disorder, who received lamotrigine during a depressive episode but with an inappropriate posology, and developed toxic epidermal necrolysis, we reviewed and commented on the corresponding literature. We conclude that extreme caution is necessary when deciding the use of lamotrigine to minimize the risk of this severe adverse effect.

Reações cutâneas graves em crianças: como se comportam? / Severe cutaneous adverse reactions in children: how do they behave?

Introdução: As reações cutâneas graves a medicamentos (RCGM) compreendem um grupo de doenças caracterizadas por hipersensibilidade tardia a um ou vários tipos de fármacos. Por ser uma doença potencialmente fatal, o diagnóstico precoce, bem como o início do tratamento, são de suma importância. Objetivo: Analisar a evolução das RCGM em pacientes pediátricos acompanhados em dois hospitais da cidade de São Paulo, SP. Método: Trata-se de um estudo retrospectivo baseado na análise de prontuários de pacientes atendidos no período de 2002 a 2018 em dois hospitais da capital paulista. Resultados: Não houve diferença entre os sexos, prevaleceu a faixa etária dos adolescentes, e os medicamentos mais implicados com o desenvolvimento das lesões cutâneas foram os anticonvulsivantes, sendo os principais a carbamazepina e fenitoína, sem diferença entre eles, seguidos dos antibióticos betalactâmicos. No tratamento, todos os pacientes fizeram uso de corticoides sistêmicos e anti-histamínicos, sendo que oito pacientes também receberam imunoglobulina intravenosa e um recebeu ciclosporina. A taxa de mortalidade foi baixa e, em relação às complicações e sequelas, a autoimunidade foi a mais encontrada. Conclusão: Os casos de RCGM são eventos raros na faixa etária pediátrica, todavia de alta morbimortalidade e risco de sequelas. O diagnóstico e tratamento precoces contribuem para um melhor prognóstico, sendo de suma importância a identificação da medicação associada, bem como a retirada da mesma.

Background: Severe cutaneous adverse reactions (SCARs) comprise a group of diseases characterized by late hypersensitivity to one or more types of drugs. Because they are potentially fatal, early diagnosis and initiation of treatment are of paramount importance. Objective: To analyze the evolution of SCARs in pediatric patients followed up in two hospitals in São Paulo, SP, Brazil. Methods: This is a retrospective study based on the analysis of medical records of patients treated between 2002 and 2018 in two hospitals in the state capital. Results: There was no difference between sexes, and the age group of adolescents prevailed. Anticonvulsants were the drugs most implicated in the development of skin lesions, especially carbamazepine and phenytoin, with no difference between them, followed by betalactam antibiotics. During treatment, all patients used systemic corticosteroids and antihistamines; eight patients also received intravenous immunoglobulin and one received cyclosporine. The mortality rate was low, and regarding complications and sequelae, autoimmunity was the most commonly found. Conclusion: Cases of SCAR are rare events in the pediatric age group, but morbidity, mortality, and risk of sequelae are high. Early diagnosis and treatment contribute to a better prognosis, and identification of the associated medication as well as its withdrawal are extremely important.

Cese en el desarrollo radicular. Secuelas orales del Síndrome de Stevens-Johnson: Reporte de un caso / Cesse no desenvolvimento radicular. Sequelas orais da síndrome de Stevens-Johnson: relato de caso / Cease in root development. Oral sequelae of Stevens-Johnson Syndrome: A case report

Resumen El Síndrome de Stevens-Johnson (SSJ) y la Necrólisis Epidérmica Tóxica (NET) son enfermedades que se encuentran dentro del espectro de las reacciones adversas cutáneas severas. Estas enfermedades comprometen cavidad oral entre el 25% y 50% de los casos. Se presenta el caso de un paciente de 10 años de edad que sufrió unataque agudo de SSJ a la edad de 6 años. Con implicación de la cavidad oral debido a las secuelas que presenta mucosa oral, raíces cortas y poco desarrolladas en todos los órganos dentario sin otra causa atribuible en su historial médico, diferente al SSJ. La edad a la que se presentó la enfermedad y el estadio de desarrollo dental señalan como causa probable el ataque agudo de SSJ, lo que permite concluir que durante su curso ocurrió un daño en la vaina epitelial de Hertwig, provocando apoptosis y, en consecuencia, un cese en el desarrollo radicular.

Abstract Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are diseases that are within the spectrum of severe cutaneous adverse reactions. These diseases compromise the oral cavity in 25% to 50% of the cases. We present a case of a 10-year-old patient who suffered an acute attack of SJS at the age of 6 years. In the oral cavity, he presented sequelae in the oral mucosa, delayed root developed in all teeth, with no other cause attributable in its medical history, different to the SJS. The age at which the disease occurred and the stage of dental development point to that the acute SJS as a probable cause, which allows us to conclude that damage occurred in the Hertwig epithelial sheath during its course, causing apoptosis and, consequently, a cessation in root development.

Resumo A síndrome de Stevens-Johnson (SJS) e a Necrólise Epidérmica Tóxica (NET) são doenças que estão dentro do espectro de reações adversas cutâneas graves. Essas doenças comprometem a cavidade oral em 25% a 50% dos casos. É apresentado um caso de um paciente de 10 anos de idade que sofreu um ataque agudo de SJS aos 6 anos de idade. Na cavidade oral, apresentava sequelas na mucosa oral, raízes curtas e pouco desenvolvidas em todos os dentes, sem outra causa atribuível no seu histórico médico, diferente da SJS. A idade em que a doença ocorreu e o estágio do desenvolvimento dentário apontam o ataque agudo da SJS como causa provável, o que permite concluir que o dano ocorreu na bainha epitelial de Hertwig durante o seu curso, causando apoptose e, consequentemente, cesse no desenvolvimento radicular.

Síndrome de Steven Johnson: Caso clínico / Steven Johnson syndrome: Clinical case

Resumen El síndrome de Stevens-Johnson (SJS) y la necrosis epidérmica tóxica (TEN) son consideradas como un espectro de enfermedades cutáneas raras pero potencialmente fatales. Se presenta el caso de una mujer de 55 años, con antecedente de epilepsia secundario a angiomas cavernosos en manejo con fenitoína. Asistió por cuadro clínico de dos días de evolución consistente en edema facial asociado a rash pruriginoso en región toracocervical, disfagia y picos febriles no cuantificados. A la exploración física conhiperemia e inyección conjuntival, edema de labios superior e inferior con lesiones tipo aftas blanquecinas y dolorosas. En piel, exantema maculopapular, confluente y eritematoso que desaparece a la digitopresión, de predominio en tronco y extremidades superiores. Ante estos resultados se sugirió que la paciente estuviera cursando con un síndrome de Steven Johnson. Esta es una patología de baja prevalencia, generalmente secundaria a una reacción idiosincrática que en la mayoría de los casos se produce por medio de medicamentos como los antibióticos, anticonvulsivantes, antiinflamatorios no esteroideos, entre otros. El manejo se basa en un diagnóstico temprano, retiro de las sustancias causales, realizar una vigilancia estricta de líquidos corporales, temperatura, aislamiento y un adecuado cuidado de la piel. Dentro de las medidas farmacológicas se puede usar la inmunoglobulina intravenosa.

Summary Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) are considered as a spectrum of rare but potentially fatal skin diseases. The case of a 55-year-old woman with a history of epilepsy secondary to cavernous angiomas under management with phenytoin is presented. She attended for a clinical picture of two days of evolution consisting of facial edema associated with pruritic skin eruption in the thoracocervical region, dysphagia and unquantified feverish peaks. On physical examination with hyperemia and conjunctival injection, upper and lower lip edema with whitish and painful thrush-like lesions. In the skin, a maculopapular, confluent and erythematous rash that disappears when digitopressured, predominantly in the trunk and upper extremities. Given these results, it was suggested that the patient be studying with Steven Johnson syndrome. This is a pathology of low prevalence, generally secondary to an idiosyncratic reaction that in most cases occurs through medications such as antibiotics, anticonvulsants, non-steroidal anti-inflammatory drugs, among others. Management is based on early diagnosis, removal of causal substances, strict monitoring of body fluids, temperature, isolation and proper skin care. Within pharmacological measures, intravenous immunoglobulin can be used.

Síndrome de Lyell em 72% de superfície corporal / Lyell syndrome in 72% of the body surface

Introdução: A síndrome de Lyell é caracterizada por flictenas em mais de 30% de superfície corporal e acomete mucosas. Está relacionada à reação alérgica decorrente de medicamentos e apresenta alta mortalidade. Relato de caso: Mulher de 28 anos apresentou flictenas em 72% de superfície corporal após uso de diversos fármacos. Apesar da grande extensão, a paciente evoluiu de maneira satisfatória e recebeu alta sem sequelas. Conclusão: É fundamental o diagnóstico precoce, a estratificação e a conduta para reduzir a mortalidade da doença.

Introduction: Lyell's syndrome is characterized by phlyctenas on more than 30% of the body surface and by involvement of the mucous membranes. It is related to the allergic reaction resulting from medications and has a high mortality. Case report: A 28-year-old woman presented phlyctenas on 72% of the body surface after using various drugs. Despite a great extent, the patient progressed satisfactorily and was discharged without sequelae. Conclusion: Early diagnosis, stratification, and management are essential to reduce mortality from the disease.

Necrólisis cutánea - Casuística de 25 años / Cutaneous necrolysis - Casuistry of 25 years

Introducción: La Necrólisis Epidérmica es una reacción grave y potencialmente mortal, inducida sobre todo por fármacos. Incluye 2 cuadros que presentan similitudes histológicas, etio-patogénicas, pero difieren en el porcentaje de piel afectada: el Síndrome de Stevens-Johnson y la Necrólisis Epidérmica Tóxica. Existen cuadros intermedios entre ambas entidades. El objetivo del trabajo es comunicar las características clínico-epidemiológicas de los casos de Necrólisis Epidérmica registrados en el Hospital Nacional en un periodo de 25 años. Materiales y Métodos: Estudio retrospectivo, descriptivo de los casos de Necrólisis Epidérmica en sus variantes Síndrome de Stevens-Johnson, Necrólisis Epidérmica Tóxica y cuadros intermedios registrados en el Servicio de Dermatología del Hospital Nacional en el periodo 1992-2017. Resultados: Se registraron 46 casos, 27 de Stevens-Johnson, 15 de Necrólisis Epidérmica Tóxica y 4 intermedios. Los casos se observaron en ambos sexos por igual, con edad media de 26,7años (DE 20,69). Los fármacos involucrados con más frecuencia fueron los antibióticos en 16 casos (penicilina y derivados en primer lugar), los anticonvulsivantes (13 casos) predominando la difenilhidantoina y antiinflamatorios-analgésicos (11 casos). El tratamiento consistió en medidas generales en todos, corticoides sistémico en 21 casos y gammaglobulina en 3. La mortalidad global fue del 28%, llegando al 46% en la Necrólisis Epidérmica Tóxica. Conclusiones: La Necrólisis Epidérmica es un cuadro poco frecuente pero grave, con elevada mortalidad, causado principalmente por antibióticos y anticonvulsivantes en esta serie, al igual que en la mayoría de otras series

Introduction: Epidermal Necrolysis is a serious and potentially fatal reaction, induced mainly by drugs. It includes 2 clinical pictures that present histological, etio-pathogenic similarities, but differ in the percentage of affected skin: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. There are intermediate tables between both entities. The objective of the work is to communicate features epidemiological cases of Epidermal Necrolysis reported at a National Hospital in a period of 25 years. Materials and methods: Retrospective, descriptive study of the cases of Epidermal Necrolysis in its variants Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis and intermediate tables registered in the Dermatology Service of the National Hospital in the period 1992-2017. Results: 46 cases were registered, 27 of Stevens-Johnson, 15 of Toxic Epidermal Necrolysis and 4 intermediate. Cases were observed in both sexes equally, with an average age of 26.7 years (SD 20.69). The drugs most frequently involved were antibiotics in 16 cases (penicillin and derivatives in the first place), anticonvulsants (13 cases), predominantly diphenylhydantoin and anti-inflammatoryanalgesics (11 cases). The treatment consisted of general measures in all, systemic corticoids in 21 cases and gamma globulin in 3. The overall mortality was 28%, reaching 46% in Toxic Epidermal Necrolysis. Conclusions: Epidermal necrolysis is a rare but serious condition, with high mortality, caused mainly by antibiotics and anticonvulsants in this series, as in most other series.

Terapia de fotobiomodulação no tratamento das lesões orais da síndrome de Stevens-Johnson: relato de caso / Photobiomodulation therapy in the treatment of Stevens-Johnson syndro-me oral lesions: case report

Introdução: A Síndrome de Stevens-Johnson é uma reação rara que se manifesta por exantema que evolui para um extenso deslocamento epidérmico. Na maioria dos casos, há o surgimento de lesões dolorosas em mucosa oral, que podem reduzir a ingestão de nutrientes e dificultar a higiene bucal. A terapia de fotobiomodulação pode ter um importante papel antálgico, na modulação da inflamação e na cicatrização dessas lesões. Objetivo: Relatar um caso clínico no qual as lesões orais da Síndrome de Stevens-Johnson foram tratadas com o auxílio da terapia de fotobiomodulação. Relato de caso: Paciente de 17 anos, do sexo feminino, com diagnóstico de síndrome de Stevens-Johnson, apresentava-se com edema labial, úlceras orais, febre e odinofagia intensa, com tolerância apenas para dieta líquida. Encontrava-se com redução da ingestão alimentar, sem comunicação oral eficiente e com interrupção da higiene bucal devido a dor intensa. A terapia de fotobiomodulação foi utilizada no tratamento das lesões orais. Conclusão: A terapia de fotobiomodulação auxiliou na redução da dor, favoreceu a ingestão de alimentos por via oral e reduziu o tempo de cicatrização das lesões.

Introduction: Stevens-Johnson Syndrome is a rare reaction manifested by a rash that evolves into extensive epidermal dislocation. In most cases there are painful oral mucosal lesions that can reduce nutrient intake and make oral hygiene difficult. Photobiomodulation therapy can play an important antalgic role in modulating inflammation and healing these lesions. Objective: To report a clinical case in which Stevens-Johnson Syndrome oral lesions were treated with the aid of photobiomodulation therapy. Case Report: A 17-year-old female patient diagnosed with Stevens-Johnson syndrome presented with lip edema, oral ulcers, fever, and severe odynophagia, tolerating only a liquid diet. She experienced reduced food intake, no efficient oral communication, and an interruption of oral hygiene due to intense pain. Photobiomodulation therapy was requested to treat the oral lesions. Conclusion: Photobiomodulation therapy helped reduce pain, favored oral food intake, and reduced lesion healing time.

Reporte de un caso clínico de Síndrome de Stevens Johnson por uso de Lamotrigina / Report of a clinical case of Stevens Johnson Syndrome due to the use of Lamotrigine

El Síndrome de Stevens-Johnson (SJS) es una urgencia dermatológica rara pero potencialmente fatal que se diferencia de le necrólisis epidérmica tóxica en el porcentaje de desprendimiento de piel y que amerita tratamiento agresivo que incluya retiro de la medicación que provoca el síndrome, manejo de heridas, líquidos de reanimación, uso de inmunoglobulina y soporte nutricional temprano para impactar en el desenlace final. Entre los factores que se han correlacionado con un peor pronóstico se hallan la edad del paciente, alteraciones hematológicas como trombocitopenia, neutropenia y linfopenia, además de la alteración de la función renal. El caso que reportamos es el de un paciente masculino de 6 años con antecedentes de Trastorno del espectro autista y epilepsia manejado con ácido valpróico que ameritó cambio a lamotrigina por no conseguir el medicamento. El paciente desarrolló una faringoamigdalitis que se manejó con trimetoprim-sulfametoxazol y 4 días después de haber finalizado el antibiótico y 12 días después de haber iniciado la lamotrigina desarrolló el SJS; fue manejado en unidad de cuidados intensivos pediátricos con hidratación, uso de inmunoglobulina, antibióticos y curación de heridas con evolución favorable permitiendo egreso luego de 19 días

Stevens-Johnson Syndrome (SJS) is a rare but potentially fatal dermatological emergency that differs from toxic epidermal necrolysis in the percentage of skin detachment and that merits aggressive treatment that includes withdrawal of the medication that causes the syndrome, management of wounds, resuscitation fluids, use of immunoglobulin and early nutritional support to impact the final outcome. Among the factors that have been correlated with a worse prognosis are the patient's age, haematological alterations such as thrombocytopenia, neutropenia and lymphopenia, as well as impaired renal function. The case we report is a 6-year-old male child with a history of Autism Spectrum Disorder and epilepsy managed with valproic acid that warranted a change to lamotrigine for not getting the medication. The patient developed a pharyngotonsillitis that was managed with trimethoprim-sulfamethoxazole and 4 days after the antibiotic was finished and 12 days after starting lamotrigine he developed SJS; he was managed in pediatric intensive care unit with hydration, use of immunoglobulin, antibiotics and wound healing with favorable evolution allowing discharge after 19 days

Relato de caso e revisão da literatura de síndrome de Stevens-Johnson associada a episódio de Varicela / Case report and Steve-Johnson syndrome literature review associated with chickenpox episode

Objetivo: Descrição de caso de Síndrome de Stevens-Johnson (SSJ) associado a quadro de varicela e revisão da literatura em criança. Descrição do caso: Feminina, 6 anos, iniciou com prurido ocular seguido de lesões eritematosas pápulosas em tronco e febre de 38,5ºC. Atendida em unidade básica de saúde, sendo iniciado violeta, analgésicos e anti-histamínicos. No mesmo dia, houve aumento do número das lesões nas extremidades. Procurou atendimento terciário na cidade de origem; internada, iniciou antibióticoterapia e uso de antiinflamatórios devido a suspeita de varicela infectada. Dois dias depois, apresentou hiperemia conjuntival, edema de pálpebras e lesões vesiculares pruriginosas em extremidades, sendo recebida em hospital infantil em Criciúma com diagnóstico de varicela com infecção secundária. Apresentou linfonodomegalia em cadeia submandibular, odinofagia, mucosa oral com lesões descamativas e purulentas. Laboratório com leucocitose sem desvio à esquerda e hematúria. Hemocultura e urocultura negativas, demais exames sem alterações. Iniciou-se antibioticoterapia com oxacilina, aciclovir e tobramicina tópica em isolamento. Após três dias, com a hipótese de Síndrome de Stevens-Johnson, iniciou o tratamento de suporte, com melhora significativa do estado geral e remissão progressiva das lesões cutâneas.Comentários: A Síndrome de StevensJohnson (SSJ) e a Necrólise Epidérmica Tóxica (NET) constituem um espectro de reações cutâneas que afeta todas as idades. O diagnóstico é essencialmente clínico e não há critérios diagnósticos ou achados histopatológicos específicos. O tratamento mais eficaz e seguro ainda é o de suporte.

Objective: Case report of Stevens-Johnson syndrome (SJS) associated with Chickenpox disease and literature review in children. Case description: Female, 6 years old, started with ocular pruritus followed by papular erythematous lesions on the chest and fever (38.5ºC/101,3ºF). She was examined at a basic health unit, where it was prescripted gentian violet, analgesics and antihistamines. On the same day, there was an increase in the number of lesions at the extremities. She sought after a tertiary care in her city of origin; after hospitalization, it was started antibiotic therapy and anti-inflammatory drugs due to the suspicion of infected chickenpox. Two days later, she presented conjunctival hyperemia, eyelid edema and pruritic vesicular lesions at the extremities. Then, she was referred to a children's hospital in Criciúma with the diagnosis of Chickenpox with secondary infection. She presented submandibular lymphadenopathy, odynophagia and scaly and purulent lesions in the oral mucosa. The laboratory showed leukocytosis without left shift, hematuria, negative blood culture and uroculture; other exams without further alterations. Antibiotic therapy with oxacillin, aciclovir and topical tobramycin was started in isolation. After three days, with the hypothesis of SJS, it was started a supportive treatment, with significant improvement of the general state and progressive remission of the cutaneous lesions.Comments: Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEC) constitute a spectrum of cutaneous reactions that affects all ages. The diagnosis is essentially clinical and there are no specific diagnostic criteria or histopathological findings. The most effective and safe treatment still is support.

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.

Síndrome de Stevens-Johnson y necrólisis epidérmica tóxica en una lactante / Stevens-Johnson Syndrome and toxic epidermic necrolysis in an infant girl

Se describe el caso clínico de una lactante de 6 meses de edad, residente en el municipio de Palma Soriano, de la provincia de Santiago de Cuba, con antecedente de buena salud, atendida por su médico de familia por presentar lesiones en la piel. En esta ocasión se le diagnosticó impétigo contagioso, para lo cual fue tratada con azitromicina; al séptimo día presentó fiebre de 40 °C y nuevas lesiones en la cara, el tronco, así como en los miembros inferiores y superiores, con ampollas y costras, de manera que fue ingresada en el Hospital Pediátrico de su municipio, con el diagnóstico de síndrome mano-boca-pie. Se le indicó tratamiento sintomático y 24 horas después comenzó a presentar toma del estado general, inflamación de las mucosas bucal, conjuntival, vulvar y anal, además de zonas eritematosas y húmedas, que se extendían por casi toda la superficie corporal. El signo de Nikolski resultó positivo y se corroboró que la paciente presentaba síndrome de Stevens Johnson y necrólisis tóxica epidérmica, pero evolucionó favorablemente

The case report of a 6 months infant girl from Palma Soriano municipality, in Santiago de Cuba who had a history of good health is described. She was assisted by her family doctor due to lesions in the skin. In this occasion she was diagnosed impetigo contagiosa, reason why she was treated with azitromicin; at the seventh day she had fever (40 °C) and new lesions in the face, trunk, as well as in the lower and upper members, with blisters and scabs, so that she was admitted in the Pediatric Hospital of her municipality, with the diagnosis of hand-mouth-foot syndrome. She was indicated symptomatic treatment and 24 hours later her general state began to decline, inflammation of the oral, conjuntival, vulvar and anal mucous, besides erithematous and humid areas that extended for almost the whole body surface. Nikolski sign was positive and it was corroborated that the patient presented the Stevens Johnson syndrome and epidermic toxic necrolysis, but she had a favorable clinical course

Síndrome de Stevens-Johnson asociado al tratamiento con metotrexato de la leucemia linfoblástica aguda: a propósito de un caso / Stevens-Johnson Syndrome associated with methotrexate treatment for acute lymphoblastic leukemia: a case report

La necrólisis epidérmica tóxica y el síndrome de StevensJohnson son enfermedades mucocutáneas raras que están asociadas a una evolución prolongada y a un desenlace potencialmente mortal. Principalmente están inducidas por fármacos y las tasas de mortalidad son muy elevadas. Aunque la piel es la más comprometida, también pueden estar afectados múltiples aparatos o sistemas como el cardiovascular, pulmonar, gastrointestinal y urinario. En este artículo, describimos el caso de un paciente con síndrome de Stevens-Johnson asociado al tratamiento con metotrexato, quien desarrolló insuficiencia cardíaca aguda y hemorragia gastrointestinal además de las manifestaciones en la piel. El paciente recibió un tratamiento satisfactorio con metilprednisolona e inmunoglobulina por vía intravenosa y continuó la quimioterapia con metotrexato.

Toxic epidermal necrolysis and Stevens-Johnson syndrome are rare mucocutaneous diseases which are associated with a prolonged course and potentially lethal outcome. They are mostly drug induced and mortality rates are very high. Although mostly skin is involved, multiple organ systems such as cardiovascular, pulmonary, gastrointestinal, and urinary systems may be affected. Here, we report a case of StevensJohnson Syndrome associated with methotrexate treatment who developed acute cardiac failure and gastrointestinal hemorrhage beside skin findings. He had been treated with intravenous immunglobulin and methylprednisolone succesfully and continued chemotherapy with methotrexate treatment again.

Necrólisis epidérmica tóxica y lupus eritematoso sistémico / Toxic epidermal necrolysis and systemic lupus erythematosus

Resumen El lupus eritematoso sistémico con manifestación tipo necrólisis epidérmica tóxica es una entidad descrita recientemente y cada vez aparecen más reportes en la literatura. Se describe el caso de una paciente de 15 años con lupus eritematoso sistémico quien presentó una necrólisis epidérmica tóxica extensa, cuyas lesiones iniciales eran tipo eritema multiforme. Se discute el caso a la luz de la literatura actual sobre esta nueva entidad.

Abstract Toxic epidermal necrolysis (TEN)-like systemic lupus erythematosus is a recently described entity and more cases are being published in the literature today. We describe the case of a 15-year old patient with systemic lupus erythematosus who developed TEN that initially started with erythema multiforme (EM)-like lesiones. We discuss this case according to the published literature on this new entity.

Desenlaces en pacientes operados con queratoprótesis boston tipo 1 en un centro oftalmológico especializado en la ciudad de Medellín / Outcomes in patients with boston type 1 keratoprosthesis in a specialized center in Medellín

Objetivo: reportar las características y descenlaces de pacientes con queratoprótesis Boston tipo 1. Diseño: estudio observacional descriptivo de corte transversal. Método: se incluyeron los pacientes operados con queratoprótesis Boston tipo 1 residentes en Colombia, mayores de 17 años, sin queratoprótesis previas y que tuviera más de 6 meses de seguimiento, para un total de 39 ojos de 39 pacientes. La cirugía se indicó en los casos con o sin queratoplastia previa que tuvieran agudeza visual (AV) igual o inferior a cuenta dedos y déficit de limbo bilateral o vascularización corneal profunda en más de 3 cuadrantes. Se recolectó y analizó la información de la historia clínica en forma retrospectiva y prospectiva. Las variables principales fueron: agudeza visual, retención del dispositivo y complicaciones. Se realizó un análisis univariado presentando los resultados en frecuencias absolutas y porcentajes para las variables categóricas y para las variables cuantitativas como promedio y desviación estándar (DE) si tienen distribución normal o como mediana y rango intercuartil si no tienen distribución normal. Resultados: La mediana de seguimiento fue de 53.5 meses (rango 6 meses a 10 años). La mayoría de pacientes (66.7%) habían tenido previamente queratoplastia penetrante. El diagnóstico de base fue en 53.8% condiciones no inflamatorias yen 46.2% condiciones inflamatorias. La AV preoperatoria en todos los ojos estaba entre percepción de luz y cuenta dedos; posterior a la cirugía el 97.4% tuvieron mejoría de la AV. Para el fi nal del seguimiento la AV permaneció mejor que la preoperatoria en 53.8%, fue igual a esta en 17.94% y peor que esta en 28.2%. Hubo necrosis periprostética en 28.2%, y 38.5% tuvieron recambio del dispositivo. Al final del seguimiento 89.7% de los pacientes tenían una queratoprótesis in-situ. Conclusión: la queratoprótesis Boston tipo 1 es una opción viable para mejorar la AV en pacientes con trasplantes de córnea fallidos o en quienes se prevé alta tasa de fallo o rechazo de este. En la gran mayoría de los pacientes se logra mejoría inicial significativa de la AV, la cual se mantiene al final del seguimiento en más de la mitad de ellos. Refinamientos progresivos en la técnica quirúrgica y el manejo postoperatorio disminuirán las complicaciones que llevan a la perdida visual progresiva

Purpose: to report characteristics and outcomes in patients with Boston type 1 keratoprosthesis. Design: observational descriptive cross-sectional study. Method: Patients with Boston type 1 queratoprosthesis who were living in Colombia, 17 years or older, without previous keratoprosthesis and with more than 6 months follow up were included, for a fi nal of 39 eyes from 39 patients. Surgery was indicated for those with or without previous keratoplasty who had visual acuity (VA) of counting fi ngers or worse and bilateral limbal stem cell deficiency or deep corneal vascularization in more than 3 quadrants. Information was collected retrospectively and prospectively from medical registries and was analyzed. Main variables were: visual acuity, device retention and complications. An univariate analysis was conducted and results are presented in absolute frequencies and percentages for categoric variables and for quantitative variables in average and standard deviation (SD) if they have normal distribution and in median and interquartile range if they do not have normal distribution. Results: Median follow up was 53.5 months (range 6 months to 10 years). Most patients (66.7%) had previous penetrating keratoplasty. Baseline diagnoses were in 53.8% non-infl ammatory conditions and in 46.2% inflammatory conditions. Preoperative VA ranged between light perception and counting fingers in all eyes; aft er surgery 97.4% had improvement in VA. By the end of follow up VA remained better than preoperative in 53.8%, was the same as preoperative in 17.94% and worse than preoperative in 28.2%. There was periprosthetic necrosis in 28.2%, and 38.5% had keratoprothesis exchange. At the end of follow up 89.7% of patients had a keratoprosthesis in-situ. Conclusion: Boston type 1 keratoprosthesis is a viable option to improve VA in patients with previously failed corneal transplants or in cases that anticipate bad prognosis for keratoplasty. In most patients there was a signifi cant initial improvement in vision, and more than half of them retained better than initial VA on their last follow up. Further refinements in surgical technique and postoperative care would decrease complications that lead to progressive visual loss.

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.