RESUMO

BACKGROUND: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a severe adverse drug reaction marked by delayed hypersensitivity reactions causing skin and systemic complications. DRESS diagnosis is challenging due to the variety of clinical presentations and symptom overlap with other conditions. The perioperative period in these patients requires precise pharmacological strategies to prevent complications associated with this syndrome. The treatment of DRESS induced by unfractionated heparin during cardiopulmonary bypass (CPB) surgery presents some challenges that must be considered when selecting an anticoagulant to avoid side effects. In this case, bivalirudin, a direct thrombin inhibitor, is indicated as an alternative to heparin in patients undergoing CPB. However, in contrast to heparin/protamine, there is no direct reversal agent for bivalirudin. CASE PRESENTATION: We report the case of an 11-year-old male diagnosed with native aortic valve endocarditis and thrombosis in his left lower extremity. During valvular replacement surgery, systemic unfractionated heparin was administered. Postoperatively, the patient developed fever, eosinophilia and pruritic rash. Warm shock and elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels followed, leading to the diagnosis of DRESS syndrome. Treatment with methylprednisolone resulted in complete resolution of symptoms. Seven years later, the patient was readmitted due to insufficient anticoagulation and a thrombus in the prosthetic aortic valve, presenting a recurrent DRESS episode due to the administration of unfractionated heparin, which was later replaced with low-molecular-weight heparin during hospitalization. Treatment with corticosteroids and antihistamines was initiated, resulting in the resolution of this episode. Ultimately, the patient required the Ross procedure. During this intervention the anticoagulation strategy was modified, unfractionated heparin was replaced with bivalirudin during the procedure and fondaparinux was administered during the postoperative period. This resulted in stable transaminases levels and no eosinophilia. CONCLUSION: The severity of DRESS Syndrome underscores the importance of early recognition, heightened monitoring, and a comprehensive approach tailored to each patient's needs. This particular case highlights the significance of this approach and may have a substantial clinical impact since it provides alternatives to heparin, such as bivalirudin and fondaparinux, in the anticoagulation strategy of CPB for patients who have a hypersensibility reaction to this medication; thus, enhancing clinical outcomes by minimizing risks linked to adverse drug reactions.

Assuntos

RESUMO

Stevens-Johnson syndrome is an infrequent condition affecting the skin and mucous membranes, it involves cutaneous detachment with high mortality without adequate treatment. We present the case of a 40-year-old male with a history of epilepsy treated with valproic acid and lamotrigine, previously diagnosed with dengue. Evaluation showed erythematous blisters on skin and mucosa with bleeding and desquamation, covering 10% of the body surface. The patient progressed favorably with the medical care received. Stevens-Johnson syndrome should be studied in association with arboviral diseases.

Assuntos

RESUMO

Unlike other adverse drug reactions, visceral organ involvement is a prominent feature of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and correlates with mortality. The aim of this study was to systematically review cases published in PubMed-indexed, peer-reviewed journals in which patients had renal injury during the episode of DRESS syndrome (DS). We found 71 cases, of which 67 were adults and 56% were males. Female sex was associated with higher mortality. Chronic kidney disease (CKD) was present in 14% of patients who developed acute kidney injury (AKI) during DS. In 21% of cases, the kidneys were the only visceral organ involved, while 54% of patients had both liver and kidney involvement. Eosinophilia was absent in 24% of patients. The most common classes of medication associated with renal injury in DS were antibiotics in 34%, xanthine oxidase inhibitors in 15%, and anticonvulsants in 11%. Among antibiotics, vancomycin was the most common culprit in 68% of patients. AKI was the most common renal manifestation reported in 96% of cases, while isolated proteinuria or hematuria was present in only 4% of cases. In cases with AKI, 88% had isolated increase in creatinine and decrease in glomerular filtration (GFR), 27% had AKI concomitantly with proteinuria, 18% had oliguria, and 13% had concomitant AKI with hematuria. Anuria was the rarest manifestation, occurring in only 4% of patients with DS. Temporary renal replacement therapy was needed in 30% of cases, and all but one patient fully recovered renal function. Mortality of DS in this cohort was 13%, which is higher than previously reported. Medication class, latency period, or pre-existing CKD were not found to be associated with higher mortality. More research, particularly prospective studies, is needed to better recognize the risks associated with renal injury in patients with DS. The development of disease-specific biomarkers would also be useful so DS with renal involvement can be easier distinguished from other eosinophilic diseases that might affect the kidney.

RESUMO

Introdução: A reação a medicamentos com eosinofilia e sintomas sistêmicos (DRESS) trata-se de uma doença grave, sendo sua gravidade relacionada ao grau de acometimento visceral, e sua taxa de mortalidade de cerca de 10%. Seu diagnóstico é desafiador, e a utilização do escore RegiSCAR como ferramenta facilita a formação deste diagnóstico. Objetivo: Analisar os aspectos clínicos, laboratoriais, evolução e classificação dos casos segundo o RegiSCAR dos pacientes internados no serviço de Alergia e Imunologia do Hospital do Servidor Público Estadual de São Paulo, com o diagnóstico de DRESS. Método: Trata-se de um estudo retrospectivo baseado na análise de prontuários de pacientes atendidos no período entre janeiro de 2006 a janeiro de 2020. Resultados: Neste estudo verificou-se maior prevalência do sexo feminino, e a DRESS acometeu principalmente adultos e idosos, tendo como comorbidades mais frequentes as doenças cardiovasculares. Dos sintomas clínicos, 69,2% dos pacientes apresentava febre, e a alteração laboratorial mais encontrada foi a presença de eosinofilia. A lesão cutânea mais frequente foi o exantema maculopapular, e os medicamentos, os anticonvulsivantes. O tempo prévio de uso do medicamento foi de 2,1 semanas, e todos os pacientes receberam corticoide sistêmico como tratamento principal, e 3 pacientes fizeram uso da imunoglubulina humana como tratamento adicional. A mortalidade foi de 7% na fase aguda, e 14% por causas secundárias. Conclusão: A DRESS é uma síndrome complexa grave e potencialmente fatal, cujo diagnóstico é desafiador. O uso do escore preconizado pelo RegiSCAR demonstrou ser importante auxílio na confirmação do diagnóstico e na diferenciação de outras doenças. A mortalidade encontrada destaca a gravidade da doença. Reconhecer e excluir a droga implicada e iniciar um tratamento precoce permite maior chance de sobrevida para estes pacientes.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a serious disease. Its severity is related to the degree of visceral involvement and its mortality rate is approximately 10%. Diagnosis is a challenge, although RegiSCAR scores can facilitate the process. Objective: To analyze clinical and laboratory data, clinical course, and classify cases according to RegiSCAR scores among patients diagnosed with DRESS who were admitted to the Allergy and Immunology service of the Hospital do Servidor Público Estadual de São Paulo. Method: This retrospective study analyzed the medical records of patients seen between January 2006 and January 2020. Results: There was a higher prevalence of women, with DRESS mainly affecting adults and older adults; cardiovascular diseases were the most frequent comorbidity. The most common clinical symptom was fever (69.2%), while the most common laboratory finding was eosinophilia. The most frequent skin lesion was maculopapular rash, and anticonvulsants were the main prescribed drug class. The drug was used for a mean of 2.1 weeks, and all patients received systemic corticosteroids as the main treatment. Human immunoglobulin was used as an additional treatment in 3 patients. Mortality was 7% in the acute phase and 14% due to secondary causes. Conclusion: DRESS is a severe, complex, and potentially fatal syndrome whose diagnosis is challenging. RegiSCAR scores helped confirm diagnosis and differentiate it from other diseases. The disease's mortality highlights its severity. Recognizing and excluding the implicated drug and initiating early treatment led to a greater chance of survival for these patients.

Assuntos

RESUMO

Resumen El síndrome de reacción a medicamentos con eosinofilia y síntomas sistémicos (DRESS, por sus siglas en inglés) es una respuesta de hipersensibilidad multisistémica poco frecuente inducida por uno o varios medicamentos que puede inducir una reacción adversa cutánea grave, la cual es difícil de diagnosticar y pone en peligro la vida del paciente si no es identificada y no se recibe tratamiento. Frecuentemente, se manifiesta como una erupción cutánea amplia, linfadenopatía, signos de afectación de órganos viscerales y alteraciones hematológicas, como leucocitosis, eosinofilia y, en ocasiones, linfocitosis atípica que se presentan de 2 a 8 semanas posterior a la administración del fármaco responsable. Los medicamentos responsables con mayor número de reportes son la fenitoína, la carbamazepina, el alopurinol y el abacavir. Se han identificado algunos alelos específicos del antígeno leucocitario humano (HLA) que se asocian a la hipersensibilidad de estos fármacos. La fisiopatología del síndrome de DRESS aún no se conoce por completo, generalmente se trata de una respuesta de hipersensibilidad mediada por células T, al interactuar con el receptor del complejo principal de histocompatibilidad en individuos con factores de susceptibilidad genética, como ocurre en otros cuadros de reacciones graves secundarias a la ingesta de fármacos. Los criterios del European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) son los más utilizados para su diagnóstico. El síndrome de hipersensibilidad inducido por fármacos (DiHS), el síndrome de Stevens-Johnson (SSJ), la necrólisis epidérmica tóxica (NET), y la pustulosis exantemática generalizada aguda (PEGA) deben considerarse ante cualquier exantema que aparezca posterior a la administración de cualquier fármaco. La terapia incluye la eliminación del agente causal lo antes posible, así como los corticosteroides sistémicos, los cuales son los pilares del tratamiento.. Los agentes ahorradores de esteroides, como la ciclosporina, las inmunoglobulinas intravenosas (IVIGs) y otros agentes inmunosupresores, se han utilizado con éxito para contribuir al tratamiento.

Abstract DRESS (drug reaction syndrome with eosinophilia and systemic symptoms) is a rare drug-induced multisystemic hypersensitivity response that can induce a severe cutaneous adverse reaction that is difficult to diagnose and treat. It frequently manifests as an extensive skin rash, systemic symptoms, lymphadenopathy, visceral organ involvement, and hematological alterations, mainly leukocytosis, eosinophilia, and sometimes atypical lymphocytosis that manifest 2 to 8 weeks after continuous administration of the responsible drug. The most prevalent drugs related with this syndrome are phenytoin, carbamazepine, allopurinol, and abacavir. Some specific human leukocyte antigen (HLA) alleles have been identified that are associated with hypersensitivity to these drugs. The pathophysiology of DRESS syndrome is not yet fully understood; the main hypothesis is a T-cell mediated hypersensitivity response when interacting with the major histocompatibility complex receptor in individuals with genetic susceptibility factors. The criteria of the European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) are the most commonly used for the diagnosis of DRESS syndrome. Drug-induced hypersensitivity syndrome (DiHS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP) should be considered for any rash that appears following the administration of any drug. Therapy of DRESS includes the elimination of the causative agent as soon as possible, as well as systemic corticosteroids which are the cornerstones of treatment. Steroid-sparing agents such as cyclosporine, intravenous immunoglobulins (IVIGs), and other immunosuppressive agents have been used successfully to contribute to treatment.

RESUMO

Drug induced liver injury (DILI) can be can be triggered by many medications including antituberculosis drugs. We present the case of a 37-year-old woman with a smear- positive pulmonary tuberculosis who started treatment with first-line antituberculosis drugs and 4 weeks later presented jaundice, somnolence and a morbilliform generalized rash with progressive neurologic deterioration which had a fatal outcome. Antituberculosis drugs can cause DILI in 2 to 28% of patients and drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) in 1.2%. Acute liver failure (ALF) can occur in 35% of patients with DILI with an overall mortality of 9.7%. If the ALF is unresponsive to medical treatment, liver transplantation has shown promising results and can avoid progression of complications. DILI can be a serious medical condition in patients receiving antituberculosis drugs. If ALF develops and is unresponsive to medical treatment, liver transplantation should be considered as the treatment of choice.

La injuria hepática inducida por fármacos (IHIF) puede ser desencadenado por varios medicamentos incluyendo fármacos anti tuberculosos. Presentamos el caso de una paciente mujer de 37 años con un frotis positivo para tuberculosis pulmonar quien inició tratamiento de primera línea y 4 semanas después, presentó ictericia, somnolencia y un exantema generalizado de tipo morbiliforme con deterioro neurológico progresivo y un desenlace fatal. Los fármacos anti tuberculosos pueden producir injuria inducida por fármacos en 2 a 28% de pacientes y síndrome de DRESS (reacción de sensibilidad a medicamentos con eosinofilia y síntomas sistémicos) en 1,2%. La falla hepática aguda (FHA) en pacientes con injuria hepática inducida por fármacos, puede presentarse en un 35% con una mortalidad del 9,7%. Si la FHA no responde a tratamiento médico, el trasplante hepático ha mostrado resultados positivos y evita la progresión de complicaciones. La IHIF puede ser una condición médica grave en pacientes que reciben medicamentos antituberculosos. Si se desencadena una FHA y no responde a tratamiento médico, debe considerarse con urgencia la posibilidad de trasplante hepático.

RESUMO

El síndrome de erupción medicamentosa con eosinofilia y síntomas sistémicos (drug reaction with eosinophilia and systemic symptoms, DRESS), también conocido como síndrome de hipersensibilidad inducida por medicamentos, es una reacción rara potencialmente mortal que causa una erupción grave y que puede provocar insuficiencia multiorgánica. Como con otras erupciones medicamentosas graves, los linfocitos T específicos para un medicamento tienen una función crucial en el síndrome DRESS. El modelo de hapteno/pro-hapteno, el modelo de interacción farmacológica y el modelo alterado de repertorio de péptidos son tres modelos diferentes desarrollados para describir la relación/interacción entre un medicamento o sus metabolitos y el sistema inmunitario. Analizamos nuestra experiencia con el tratamiento con ciclosporina en un caso de síndrome DRESS resistente a esteroides causado por ácido valproico en una niña y sus resultados clínicos, de laboratorio y de antígeno leucocitario humano (HLA).

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome, is a potentially life-threatening rare reaction that causes a severe rash and can lead to multiorgan failure. As in other severe drug eruptions, drug-specific T lymphocytes play a crucial role in DRESS. The hapten/pro-hapten model, pharmacological interaction model, and altered peptide repertoire model are three different models developed to describe the relationship/interaction between a medication or its metabolites and the immune system. We discuss our experience with cyclosporine treatment in a steroid-resistant DRESS syndrome caused by valproic acid in a girl, as well as her clinical, laboratory, and human leukocyte antigens (HLA) study results

Assuntos

RESUMO

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug-induced hypersensitivity syndrome, is a potentially life-threatening rare reaction that causes a severe rash and can lead to multiorgan failure. As in other severe drug eruptions, drug-specific T lymphocytes play a crucial role in DRESS. The hapten/pro-hapten model, pharmacological interaction model, and altered peptide repertoire model are three different models developed to describe the relationship/interaction between a medication or its metabolites and the immune system. We discuss our experience with cyclosporine treatment in a steroid-resistant DRESS syndrome caused by valproic acid in a girl, as well as her clinical, laboratory, and human leukocyte antigens (HLA) study results.

El síndrome de erupción medicamentosa con eosinofilia y síntomas sistémicos (drug reaction with eosinophilia and systemic symptoms, DRESS), también conocido como síndrome de hipersensibilidad inducida por medicamentos, es una reacción rara potencialmente mortal que causa una erupción grave y que puede provocar insuficiencia multiorgánica. Como con otras erupciones medicamentosas graves, los linfocitos T específicos para un medicamento tienen una función crucial en el síndrome DRESS. El modelo de hapteno/pro-hapteno, el modelo de interacción farmacológica y el modelo alterado de repertorio de péptidos son tres modelos diferentes desarrollados para describir la relación/interacción entre un medicamento o sus metabolitos y el sistema inmunitario. Analizamos nuestra experiencia con el tratamiento con ciclosporina en un caso de síndrome DRESS resistente a esteroides causado por ácido valproico en una niña y sus resultados clínicos, de laboratorio y de antígeno leucocitario humano (HLA).

Assuntos

RESUMO

A Síndrome de DRESS (do inglês, Drug Rash with Eosinophilia and Systemic Symptoms) é uma patologia rara que consiste em uma severa reação medicamentosa mediada por células T. O presente relato de caso retrata uma paciente do sexo feminino, 59 anos, que apresentou icterícia, febre não termometrada, acolia, colúria, mialgia, placas hipercrômicas e lesões pruriginosas. Referiu uso recente de alopurinol, paracetamol e nimesulida, apresentando melhora importante e espontânea após a suspensão das medicações. A extensão do tempo de exposição ao medicamento agressor ocasiona um maior período de internação e risco de mortalidade. Além disso, os dados restritos sobre a Síndrome de DRESS impõe desafios ao seu diagnóstico. Sendo assim, este estudo busca destacar a importância do diagnóstico clínico precoce, a suspensão do medicamento agressor e a instituição da terapêutica adequada para um prognóstico favorável

The Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare pathology that consists of a severe drug reaction mediated by T cells. The present case report depicts a female patient, 59 years old, who presented jaundice, non thermometered fever, acholia, choluria, myalgia, hyperchromic plaques and pruritic lesions. She mentioned recent use of allopurinol, paracetamol and nimesulide, showing significant and spontaneous improvement after discontinuation of medications. The extension of time of exposure to the offending drug causes a longer period of hospitalization and risk of mortality. In addition, the restricted data on DRESS Syndrome poses challenges to its diagnosis. Therefore, this study seeks to highlight the importance of early clinical diagnosis, suspension of the offending drug and the institution of appropriate therapy for a favorable prognosis

Assuntos

RESUMO

El síndrome de reacción a fármacos con eosinofilia y síntomas sistémicos es una reacción de hipersensibilidad a fármacos poco común, pero con una alta mortalidad, por ello se requiere un diagnóstico precoz y un manejo oportuno. Presentamos el caso de una mujer de 32 años con diagnóstico de epilepsia y trastorno esquizofreniforme orgánico, secundarios a encefalitis viral, y que ha recibido tratamiento con múltiples fármacos. Tres semanas después de añadir carbamazepina de liberación prolongada a su terapia habitual, la paciente presentó una erupción cutánea difusa tipo habón, edema facial, fiebre, linfadenopatía, leucocitosis con eosinofilia y elevación de las transaminasas. La administración de la carbamazepina fue suspendida, se administró antihistamínicos y glucocorticoides por vía oral, y la paciente mejoró.

The drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but highly lethal drug hypersensitivity reaction. Thus, it requires an early diagnosis and timely management. We present the case of a 32-year-old female patient with a diagnosis of epilepsy and organic schizophreniform disorder, secondary to viral encephalitis, who was treated with multiple drugs. Three weeks after the addition of extended-release carbamazepine to her usual therapy, the patient presented a diffuse welt-type skin rash, facial edema, fever, lymphadenopathy, leukocytosis with eosinophilia and elevated transaminases. Carbamazepine administration was discontinued, antihistamines and glucocorticoids were administered orally, and the patient showed a remarkable improvement.

RESUMO

A síndrome DRESS é uma entidade rara e distinta, caracterizada por acometimento cutâneo e envolvimento de órgãos internos, com risco potencial de morte. O diagnóstico e o tratamento pre- coces são de vital importância. Relatos de DRESS por paraceta- mol são raros na literatura, razão pela qual apresentamos este caso. Paciente do sexo masculino, 56 anos, com surgimento de rash maculopapular, febre, linfadenopatia e hipereosinofilia 3 semanas após suspensão de paracetamol, associados ao ante- cedente familiar de reação a fármaco. Evoluiu bem após pulso- terapia com metilprednisolona.

DRESS syndrome is a rare and distinct entity characterized by cutaneous manifestations and internal organs involvement with a potential risk of death. Early diagnosis and treatment are vi- tally important. Reported cases of DRESS syndrome due to ace- taminophen are rare in the literature, and that is the reason for this case report. A 56-year-old male patient with maculopapular rash, fever, lymphadenopathy, and hypereosinophilia three we- eks after suspension of acetaminophen, associated with a family history of drug reaction. It progressed well after pulse therapy with methylprednisolone.

Assuntos

RESUMO

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.

Assuntos

RESUMO

El síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos o síndrome de DRESS según sus siglas en inglés (drug reaction with eosinophilia and systemic symptoms) se encuentra entre las reacciones medicamentosas cutáneas graves. Este consiste en una tríada clínica que incluye fiebre, exantema y compromiso sistémico, acompañado de eosinofilia y/o linfocitos atípicos.Se presenta el caso de una paciente de sexo femenino con fibrosis quística, de 18 meses de edad, quien desarrolló esta patología durante un tratamiento con trimetoprima-sulfametoxazol para erradicar Staphylococcus aureus meticilino resistente en esputo. Los pacientes con fibrosis quística reciben múltiples esquemas antibióticos según bacteriología en secreciones respiratorias para evitar el deterioro de la función pulmonar y colonización por gérmenes resistentes. Es menester conocer y sospechar este síndrome, debido al riesgo incrementado de hipersensibilidad a drogas en fibrosis quística, pronóstico ominoso y su elevada morbimortalidad

Drug reaction with eosinophilia and systemic symptoms or DRESS syndrome is among severe cutaneous drug reactions. This constitutes a clinical triad that includes fever, skin rash and systemic compromise, accompanied by eosinophilia and/or atypical lymphocytes.We present the case of an 18-month-old female patient with cystic fibrosis, who develops this pathology during a trimethoprim-sulfamethoxazole cycle as an eradicating treatment of methicillin-resistant Staphylococcus aureus in bronchial secretions. Cystic fibrosis patients receive multiple antibiotic regimens according to bacteriology in sputum, to avoid impairment in their lung function and colonization by resistant germs. Due to the increased risk of drug hypersensitivity in cystic fibrosis, an ominous prognosis and high morbidity and mortality, knowledge and a high index of suspicion of this syndrome are necessary

Assuntos

RESUMO

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado.Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.

Drug reaction with eosinophilia and systemic symptoms is a rare and potentially serious skin adverse reaction, with fever, polymorphous skin rash, facial edema, and/or lymphadenopathy. Reactivation of human herpes virus type 6 has been associated with a more severe and/or prolonged course. A 22-month-old infant under phenobarbital treatment developed erythematous-papular lesions, fever, leukocytosis, elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.

Assuntos

RESUMO

Drug reaction with eosinophilia and systemic symptoms or DRESS syndrome is among severe cutaneous drug reactions. This constitutes a clinical triad that includes fever, skin rash and systemic compromise, accompanied by eosinophilia and/ or atypical lymphocytes. We present the case of an 18-month-old female patient with cystic fibrosis, who develops this pathology during a trimethoprim-sulfamethoxazole cycle as an eradicating treatment of methicillin-resistant Staphylococcus aureus in bronchial secretions. Cystic fibrosis patients receive multiple antibiotic regimens according to bacteriology in sputum, to avoid impairment in their lung function and colonization by resistant germs. Due to the increased risk of drug hypersensitivity in cystic fibrosis, an ominous prognosis and high morbidity and mortality, knowledge and a high index of suspicion of this syndrome are necessary.

El síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos o síndrome de DRESS según sus siglas en inglés (drug reaction with eosinophilia and systemic symptoms) se encuentra entre las reacciones medicamentosas cutáneas graves. Este consiste en una tríada clínica que incluye fiebre, exantema y compromiso sistémico, acompañado de eosinofilia y/o linfocitos atípicos. Se presenta el caso de una paciente de sexo femenino con fibrosis quística, de 18 meses de edad, quien desarrolló esta patología durante un tratamiento con trimetoprima-sulfametoxazol para erradicar Staphylococcus aureus meticilino resistente en esputo. Los pacientes con fibrosis quística reciben múltiples esquemas antibióticos según bacteriología en secreciones respiratorias para evitar el deterioro de la función pulmonar y colonización por gérmenes resistentes. Es menester conocer y sospechar este síndrome, debido al riesgo incrementado de hipersensibilidad a drogas en fibrosis quística, pronóstico ominoso y su elevada morbimortalidad.

Assuntos

RESUMO

Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids elevated C-reactive protein, and abnormal liver tests. The skin biopsy was compatible with an adverse drug reaction. Treatment with systemic corticosteroids and intravenous immunoglobulin had no response. Polymerase chain reaction for human herpesvirus type 6 was positive, and cyclosporine plus prednisone was started with a good response. There is little evidence for the use of cyclosporine in adults when systemic corticosteroids are ineffective. This is the first report of pediatric drug reaction with eosinophilia and systemic symptoms treated with cyclosporine, which could be an effective alternative or an adjunct to systemic corticosteroid therapy unresponsive to conventional treatments.

La reacción a drogas con eosinofilia y síntomas sistémicos es una reacción adversa cutánea rara, potencialmente grave. Puede presentar fiebre, erupción cutánea polimorfa, edema facial y/o linfoadenopatías. La reactivación del virus herpes humano tipo 6 se asocia a un curso más grave y/o prolongado. Un lactante de 22 meses en tratamiento con fenobarbital presentó lesiones eritematopapulares, fiebre, leucocitosis, proteína C reactiva elevada y alteración de pruebas hepáticas. Se realizó biopsia de piel compatible con reacción adversa a drogas. Se trató con corticoides sistémicos e inmunoglobulina intravenosa sin respuesta. La reacción en cadena de la polimerasa para virus herpes humano tipo 6 resultó positiva. Se inició ciclosporina más prednisona, con buena respuesta. Existe poca evidencia del uso de ciclosporina en adultos, cuando los corticoides sistémicos son inefectivos. Este es el primer reporte pediátrico Podría ser una alternativa efectiva o un complemento de los corticosteroides sistémicos cuando no responde a tratamientos convencionales.

Assuntos

RESUMO

El síndrome DRESS es una reacción adversa dermatológica que puede presentarse debido a diversos medicamentos, y constituye uno de los diagnósticos más importantes por encima del síndrome de Stevens-Johnson. Se trata de un caso relacionado con una reacción adversa de muy baja frecuencia, que está documentada en la literatura científica, a varios medicamentos, entre ellos la fenitoína. Por lo mencionado, la publicación de estos casos resulta escasa y limitada. Las principales preocupaciones del paciente relacionadas con su cuadro clínico radicaban en el gran compromiso cutáneo que lo llevó a hospitalización, dolor e incomodidad, por el cual recurrió al manejo tópico generalizado con vaselina. Los hallazgos clínicos relevantes fueron: eosinofilia severa, ulceraciones cutáneas, hepatitis química y fiebre. Con los hallazgos del cuadro clínico y la evaluación de la escala RegiSCAR se hace el diagnóstico de síndrome DRESS inducido por fenitoína. Se suspende la fenitoína, se inicia levetiracetam y se administran corticosteroides y acetaminofén con evolución favorable. (AU)

DRESS syndrome is a dermatological adverse reaction can occur due to various medications, being one of the most important diagnoses above Steven-Johnson syndrome. This is a case related to a very low frequency adverse reaction that is documented in the scientific literature to several medicines among those, the phenytoin. Therefore, the publication of these cases is scarce and limited. The main concerns of the patients related to their clinical picture were due to the great cutaneous compromise that lead to hospitalization, pain and discomfort for which they resorted to generalized topical management with vaseline (petrolatum). Relevant clinical findings were severe eosinophilia, skin ulcerations, chemical hepatitis and fever. With clinical picture findings and evaluation of the RegiSCAR scale, the diagnosis of Phenytoin-induced DRESS syndrome is made. Phenytoin is discontinued, levetiracetam is started and corticosteroids and acetaminophen are administrated with favorable evolution. (AU)

Assuntos

RESUMO

Introdução: A Síndrome Stevens-Johnson (SSJ) é uma doença causada por hipersensibilidade a imunocomplexos e pode ser desencadeada por distintos fármacos, dentre eles a fenitoína. Devido sua complexidade e raridade, ainda nãohá consenso de tratamento padrão ouro, porém sabese da necessidade da atuação multidisciplinar. Para os cuidados com as feridas, pode-se citar os curativo se a fotobiomodulação (FBM). Objetivo: Relatar o uso da FBM como terapia complementar em um caso de SSJ no Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Métodos: Paciente sexo feminino, 26 anos, deu entrada na unidade de terapia intensiva (UTI) com diagnóstico de SSJ secundária ao uso de fenitonína, escore de SCORTEN 1, com área sem epitélio íntegro 10- 30% e área acometida por lesões de 94,5%, poupando apenas o couro cabeludo. Foi abordada e tratada por uma equipe multidisciplinar e solicitado vaga em centro de especializado em queimados. No sétimo dia de UTI foi iniciado tratamento com FBM, 2 J por ponto, distância entre pontos de 2cm, comprimento onda vermelho (660nm), nas feridas que não apresentavam secreção, foram cinco sessões com intervalo de três dias entre a terceira e a quarta. Resultados: A paciente apresentou melhora visível das lesões cutâneas e recebeu alta hospitalar 5 dias após cessação da FBM. Conclusão: O uso da FBM pode ser efetiva no tratamento complementar da fase aguda SSJ desencadeada por fenitoína.

Introduction: Stevens-Johnson Syndrome (SJS) is a disease caused by hypersensitivity to immune complexes and can be triggered by different drugs, including phenytoin. Due to its complexity and rarity, there is still no consensus on gold standard treatment, but the need for multidisciplinary action is known. For wound care, dressings and photobiomodulation (PBM) can be mentioned. Objective: This study is to report the use of PBM as complementary therapy in a case of SJS at Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Methods: A 26-year-old female patient was admitted to the intensive care unit (ICU) diagnosed with SJS secondary to the use of phenytoin, SCORTEN score 1, with an area without intact epithelium 10-30% and an area affected by injuries of 94.5 %, saving only the scalp. She was approached and treated by a multidisciplinary team which requested a place in a specialized burn center. On the seventh day of ICU, treatment with PBM, 2J per point was started, distance between points of 2cm, red wave length (660nm), in wounds that did not present secretion, with a total of five sessions with an interval of three days between the third and fourth. Results: The patient showed a visible improvement of skin lesions and was discharged from hospital 5 days after cessation of PBM. Conclusion: Use of PBM can be effective in complementary treatment of acute SJS phase triggered by phenytoin.

Assuntos

RESUMO

DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a severe, rare and potentially lethal idiosyncratic condition associated with the use of some drugs. Given its broad spectrum of presentation, clinical suspicion is essential for management, since it requires the immediate withdrawal of the culprit drug, support measures and the use of corticosteroids as the first line of treatment. We report a 24-year-old woman with a diagnosis of ulcerative colitis with joint involvement despite the use of infliximab, who presented symptoms, signs and laboratory compatible with DRESS syndrome on the third week after indicating sulfasalazine for her baseline disease.

Assuntos

RESUMO

RESUMEN El síndrome de hipersensibilidad por fármacos, también conocido como síndrome de DRESS, es una farmacodermia grave que se caracteriza por una erupción polimorfa diseminada, fiebre y compromiso multiorgánico. Este padecimiento tiene una incidencia que oscila entre el 0,1 % y el 0,01 % de las exposiciones farmacológicas, con una probabilidad de fallecimiento de un 20 % al 30 %. Fue descrito por primera vez en el año 1936, como una reacción adversa a la fenitoína. En la actualidad se reconoce que puede estar asociado a otros fármacos como: abacavir, metronidazol, doxiciclina, isoniazida, carbamacepina, fenobarbital, beta-bloqueadores, dapsona, ranitidina, antiinflamatorios no esteroideos y el alopurinol. Se presenta un paciente de 69 años de edad que desarrolló un síndrome de DRESS secundario a alopurinol. El paciente mostró signos poco frecuentes de esta rara enfermedad: linfocitos atípicos, hepatomegalia y afección renal; falleció poco después debido a un choque séptico por estafilococo áureo.

ABSTRACT Drug hypersensitivity syndrome, also known as DRESS syndrome, is a severe pharmacodermia characterized by a polymorphous disseminated rash, fever, and multi-organ involvement. Its incidence ranges between 0.1 to 0.01% from the pharmacological exposures, with a probability of death ranging from 20 to 30%. It was first described in 1936 as an adverse reaction to phenytoin. Nowadays, it is known that it can also be associated with other drugs such as abacavir, metronidazole, doxycycline, isoniazid, carbamazepine, phenobarbital, beta-blockers, dapsone, ranitidine, nonsteroidal anti-inflammatory drugs and allopurinol. We present a 69-year-old male patient who developed a DRESS syndrome secondary to alupurinol. The patient showed unusual signs of this rare disease such as atypical lymphocytes, hepatomegaly and kidney disease; he dies shortly after from a septic shock due to Staphylococcus aureus.

Assuntos