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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.

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OBJECTIVE: To determine prevalence, causes and risk factors of ADE in hospitalized patients. METHODS: Analytical, observational, case-control study of patients with ADE. For statistical analysis, the following were calculated: percentages, frequencies, averages; odds ratio, χ2 test and multiple binary logistic regression. Data analysis was carried out with the Statistical Package, for the Social Sciences 23 program. RESULTS: A 132 patients were registered: 66 cases (26 EM and 40 RAM) and 66 controls; with average age of 35 years (SD 17.41). The prevalence of adverse drug events was 3.6%. The most frequently reported medications: antibiotics and anti-inflammatories. The frequency of adverse events by gender was: 39.3% men and 60.7% women. The services with the greatest patient care: emergencies, surgery; the most frequent route of administration: intravenous (32.3%). The main symptoms: skin. (32.3%) frequent symptoms: cutaneous. Associated symptoms RAM: type A pruritus (OR: 8.5; p = 0.001; IC95%: 0.035-0.393), type B pruritus (OR: 11; p = 0.001; CI95%: 0.021-0.368) urticaria (OR: 19; p = 0.005; IC95%: 0.007-0.412). Risk factors Associated EAM: female (OR: 2.6; p = 0.05; CI95%: 1.33-5.43), history of allergy (OR: 3.4; p = 0.033; CI95%: 1.04-8.40), prolonged hospital stays (OR: 5.4; p = 0.023; IC95%: 3.82-6.74). CONCLUSIONS: Patient safety is a priority when prescribing any drug, which represents a key point in prevention.

OBJETIVO: Determinar la prevalencia, causas y factores de riesgo asociados con eventos adversos a medicamentos en pacientes hospitalizados. MÉTODOS: Estudio de casos y controles, observacional, analítico, llevado a cabo en pacientes con eventos adversos a medicamentos. Para el análisis estadístico se calcularon: porcentajes, frecuencias, promedios; razón de momios, prueba de χ2 y regresión logística binaria múltiple. El análisis de los datos se efectuó con el programa Statistical Package, for the Social Sciencies 23. RESULTADOS: Se registraron 132 pacientes: 66 casos (26 EM y 40 RAM) y 66 controles, con edad promedio de 35 años (DS 17.41). La prevalencia de eventos adversos a medicamentos fue del 3.6%. Los medicamentos reportados con mayor frecuencia: antibióticos y antiinflamatorios. La frecuencia de eventos adversos por género fue: 39.3% hombres y 60.7% mujeres. Los servicios con mayor atención de pacientes: urgencias y cirugía; vía de administración más frecuente: intravenosa (32.3%). Los principales síntomas fueron los cutáneos. Los síntomas asociados con reacción adversa a medicamentos: prurito tipo A (RM: 8.5; p = 0.001; IC95%: 0.035-0.393), prurito tipo B (RM: 11; p = 0.001; IC95%: 0.021-0.368) urticaria (RM: 19; p = 0.005; IC95%: 0.007-0.412). Los factores de riesgo asociados con eventos adversos a medicamentos: mujer (RM: 2.6; p = 0.05; IC95%: 1.33-5.43), antecedente de alergia (RM: 3.4 p = 0.033; (IC95%: 1.04-8.40) y estancia hospitalaria prolongada (RM: 5.4; p = 0.023; IC95%: 3.82-6.74). CONCLUSIONES: La seguridad de los pacientes es una prioridad al momento de prescribir cualquier fármaco, lo que representa un punto clave en la prevención.

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O alopurinol, de uso contínuo oral, é o tratamento de escolha para os distúrbios hereditários do glicogênio. Apesar de não ser comum, a reação de hipersensibilidade ao alopurinol se torna um problema quando esta é a única medicação disponível para o controle da doença de base. Nestes casos, a dessensibilização é uma alternativa viável. No presente relato, descrevemos o caso de um paciente com diagnóstico de doença de depósito de glicogênio tipo I, com exantema pruriginoso generalizado ao alopurinol, tratado com um protocolo de dessensibilização oral acelerado. Este tratamento permitiu o uso contínuo deste medicamento sem novas reações em longo prazo.

Continuous oral allopurinol use is the first-line treatment for hereditary glycogen disorders. While hypersensitivity reactions to allopurinol are uncommon, they can pose challenges when this medication is the only available option for the long-term treatment of the underlying disorder. In such cases, desensitization emerges as a viable alternative. We report the case of a patient with glycogen storage disease type I who developed a generalized pruritic rash due to allopurinol. Drug intolerance was successfully managed using a rapid oral desensitization protocol, which allowed an uneventful long-term use of allopurinol.

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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.

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INTRODUÇÃO: A anafilaxia é uma reação alérgica multissistêmica grave, de início agudo e potencialmente fatal. Poucos são os dados sobre sua epidemiologia no Brasil. O Registro Brasileiro de Anafilaxia da Associação Brasileira de Alergia e Imunologia (RBAASBAI) teve como objetivo ampliar o conhecimento sobre anafilaxia em indivíduos brasileiros. MÉTODOS: Estudo observacional transversal com questionário online sobre dados demográficos, desencadeantes suspeitos, manifestações clínicas, atendimento durante a reação, investigação diagnóstica e aconselhamento após a reação de pacientes que experimentaram uma reação anafilática. RESULTADOS: Entre junho/2021 e abril/2023, foram incluídos 237 pacientes (131 femininos): 99 crianças/adolescentes; 127 adultos e 11 idosos. Houve predomínio de meninos entre crianças/adolescentes (55,5%), e de mulheres entre os adultos (64,5%), e mediana de idade de 22 anos (< 1 a 77 anos). As manifestações cutâneas (92,8%) foram as mais frequentes, seguidas pelas respiratórias (70,1%), gastrointestinais (52,3%), neurológicas (36,3%) e cardiovasculares (35,3%). Os principais desencadeantes foram: alimentos (43,0%), medicamentos (26,2%), himenópteros (21,6%) e látex (2,5%); os alimentos entre crianças (leite, ovo, amendoim/castanhas), e os fármacos (anti-inflamatórios e antibióticos) entre os adultos. Quanto ao tratamento, 61,1% recebeu adrenalina (52,7% por profissional e 8,4% via autoinjetor de adrenalina -AIA). Uma adolescente (12 anos) faleceu após picada de abelha. A maioria recebeu plano escrito de emergência (78,1%) e foi ensinada a usar o AIA (70%). CONCLUSÃO: Os alimentos foram os desencadeantes mais comuns entre crianças/adolescentes, e os fármacos entre adultos brasileiros. A adrenalina continua sendo subutilizada, reforçando a necessidade de maior disseminação do tratamento adequado da anafilaxia.

INTRODUCTION: Anaphylaxis is a life-threatening, acute, severe multisystem allergic reaction.There is little data on its epidemiology in Brazil. The Brazilian Anaphylaxis Registry of the Brazilian Association of Allergy and Immunology (RBA-ASBAI) was devised to expand knowledge about anaphylaxis in Brazilian individuals. METHODS: Cross-sectional observational study using an online questionnaire to collect data on demographics, suspected triggers, clinical manifestations, treatment during the reaction, diagnostic workup, and post-reaction counseling in patients who have experienced an anaphylactic reaction. RESULTS: Between June 2021 and April 2023, 237 patients were included (131 female): 99 children/adolescents (<18yo), 127 adults (18-64yo), and 11 older adults (65-77yo). There was a male predominance in the pediatric group (55.5%), while females were predominant among adults (64.5%). The median age was 22 years (range, <1 to 77). The most frequent clinical manifestations were cutaneous (92.8%), followed by respiratory (70.1%), gastrointestinal (52.3%), neurological (36.3%), and cardiovascular (35.3%). The most common triggers were foods (43.0%), drugs (26.2%), venoms (21.6%), and latex (2.5%). Foods (milk, egg, peanuts/tree nuts) predominated among children, versus drugs (mostly nonsteroidal anti-inflammatory drugs and antibiotics) among adults. Regarding treatment, 61.1% received epinephrine (52.7% by a healthcare professional and 8.4% via epinephrine auto-injector [EAI]). One teenager (12yo) died due to a bee sting. Most patients received a written emergency plan (78.1%) and were taught how to use the EAI (70%). CONCLUSION: Foods were the most common triggers of anaphylaxis among Brazilian children and adolescents, while drugs predominated among adults. Epinephrine continues to be underused, highlighting the need for greater awareness of proper treatment of anaphylaxis.

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Drug hypersensitivity reactions (DHR) in children have a significant impact on clinical practice and public health. Both under-diagnosis (due to under-reporting) and over-diagnosis (due to the overuse of the term "allergy") are potential issues. The aim of this narrative review is to describe the most recent findings of DHR in children/adolescents and gaps regarding epidemiology, antibiotic allergy, antiepileptic hypersensitivity, vaccine allergy, and severe cutaneous adverse reactions (SCAR) in this age group.

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ABSTRACT Objective: To characterize drug hypersensitivity associated with dental treatments. Material and Methods: Data from 5,302 dental patients extracted from the Faculty of Dental Medicine were used to investigate drug use history, drug hypersensitivity, and associations with oral health outcomes. The chi-square test was used, and values of p ≤ 0.05 were considered statistically significant. Results: The frequency of patients' self-reported drug hypersensitivity was 26.42% (n = 1,401). The highest frequencies were for opioid/narcotic analgesics (20.84%, n = 292), antibiotics (18.13%, n = 961), and non-steroidal anti-inflammatory drugs (10.46%, n = 141). Most of the patients (68.65%, n = 3,640) reported using medications, mostly for cardiovascular disease (43.1%, n = 1,569), for psychiatric/neurological disorders (39.75%, n = 1,447), drugs that affect the endocrine system (32.55%, n= 1,185), and drugs for pain (24.92%, n = 907). Higher drug hypersensitivity frequencies were associated with older White female subjects (p<0.0001). Associations were also identified between drug hypersensitivity and history of the following dental procedures: tooth extractions (p=0.003), root canal treatment (p=0.0004), prosthodontic treatments (p<0.0001), and orthodontic treatments (p=0.007). Conclusion: A high frequency of self-reported drug hypersensitivity in dental patients was found, with a higher occurrence in older White women and those with a history of more extensive and invasive dental care.

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ABSTRACT A 69-year-old female was referred with sudden unilateral painless decreased vision that began 2 days after uncomplicated cataract surgery in the left eye. Visual acuity was hand motion and biomicroscopy showed a mild anterior chamber reaction, no hypopyon, and an intraocular lens that had been placed within the capsular bag. A dilated fundus examination revealed optic disk edema, widespread deep and superficial intraretinal hemorrhages, retinal ischemia, and macular edema. A cardiological evaluation was normal and thrombophilia tests were negative. After surgery, prophylactic vancomycin (1mg/0.1ml) had been injected intracamerally. The patient was diagnosed with hemorrhagic occlusive retinal vasculitis likely secondary to vancomycin hypersensitivity. Recognition of this entity is important to ensure early treatment and the use of intracameral vancomycin in the fellow eye should be avoided after cataract surgery.

RESUMO Esse caso se refere a uma paciente de 69 anos, sexo feminino, com relato de baixa acuidade visual súbita e indolor no olho esquerdo, de início 2 dias após cirurgia de catarata sem complicações. A acuidade visual era de movimento de mãos e a biomicroscopia mostrou reação de câmara anterior moderada, sem hipópio, e lente intraocular posicionada dentro do saco capsular. A fundoscopia evidenciou edema de disco óptico, hemorragias difusas intrarretinianas superficiais e profundas, isquemia retiniana e edema macular. A avaliação cardiológica foi normal e os testes para trombofilia foram negativos. Ao final da cirurgia foi injetado antibioticoprofilaxia com vancomicina (1mg/0,1ml) na câmara anterior. A paciente foi diagnosticada com vasculite hemorrágica oclusiva da retina secundária à hipersensibilidade a vancomicina. O reconhecimento dessa entidade é importante para o tratamento precoce e para evitar o uso de vancomicina intracameral em caso de cirurgia de catarata no olho contralateral.

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Objective: To assess the reproducibility of symptoms in drug challenge tests. Methods: The study included patients with positive cutaneous or challenge test throughout 2019. For each patient, clinical suspicion according to Karch-Lasagna algorithm was registered. Primary outcome was the reproducibility of symptoms in the provocation tests using a paired analysis of data with McNemar test. Results: Eighty-nine patients were included, 16 of them presented more than one positive test. Thirty were skin tests positive and 75 reacted to provocation tests. Eighty nine percent of patients who reacted in challenge test were probably or possibly reactors according to Karch-Lasagna scale. Symptoms of initial reaction did not differ from those triggered in challenge tests. Conclusions: Karch-Lasagna scale is useful in predicting the response to drug provocation tests. In most of the positive studies, results were suggested by clinical history and no differences were found between symptoms triggered in challenge test and that referred to in the previous reaction.

Objetivo: Evaluar la reproducibilidad de los síntomas en pruebas de exposición con fármacos. Métodos: Estudio retrospectivo, efectuado en pacientes con prueba cutánea o exposición positiva, atendidos en 2019. De cada paciente se registró la sospecha clínica según el algoritmo de Karch-Lasagna. El resultado principal fue la reproducibilidad de síntomas en las pruebas de exposición, mediante el análisis de datos emparejado con prueba de McNemar. Resultados: Se incluyeron 89 pacientes, y de estos 16 reportaron varias pruebas positivas. Se obtuvieron 30 pruebas cutáneas y 75 de exposición positivas. En el 89% de las pruebas de exposición positivas, las reacciones iniciales se clasificaron en probables o posibles, según la escala de Karch-Lasagna. Los síntomas reportados en la reacción inicial no difirieron de los de las pruebas de exposición. Conclusiones: La escala de Karch-Lasagna es un método útil para predecir la respuesta en las pruebas de exposición con fármacos. En la mayor parte de las pruebas positivas, los resultados fueron sugeridos por la historia clínica, sin diferencias entre la manifestación de síntomas en la prueba de exposición versus los referidos en la reacción inicial.

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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.

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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.

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Drug reaction with eosinophilia and systemic symptoms, known by its acronym in English as DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms), clinically manifests with fever, facial edema, lymphadenopathy, a morbilliform rash, and organ involvement. Laboratory results reveal leukocytosis, atypical lymphocytes, eosinophilia, and alterations of liver and kidney function tests. The actual incidence of DRESS is unknown, because it may vary depending on the type of medication and the immune status of each patient; also, because many cases remain undiagnosed or untreated. The drugs most associated with DRESS include antiepileptics, antibiotics, antituberculosis, and non-steroidal anti-inflammatory agents (NSAIDs). Its diagnosis is sometimes made late and can become a challenge. The diagnostic criteria proposed by the international Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) help to establish the diagnosis through a score system based on clinical and laboratory findings. The first step to identify the culprit is a thorough clinical history that includes all suspects, emphasizing those most known to cause DRESS syndrome according to the context and the literature. A skin biopsy may also be helpful in the diagnostic process. Patch testing is the test of choice to search for the culprit in cases of DRESS. Regarding prognosis, the estimated mortality due to DRESS is 3.8%. The main causes of mortality include fulminant hepatitis and liver necrosis. Several indicators of poor prognosis have been identified and these include an eosinophil count above 6000 × 103/µL, thrombocytopenia, pancytopenia, leukocytosis and coagulopathy. This article aims to review the evidence available regarding the epidemiology, pathophysiology, clinical and laboratory findings, diagnosis, and treatment of DRESS.

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RESUMEN Introducción: La desensibilización farmacológica induce un estado temporal de hiposensibilidad a un medicamento que se realiza a pacientes con reacciones mediadas por Inmunoglobulina E. Reporte de caso: Presentamos el caso de un paciente con exacerbación respiratoria de fibrosis quística que presentó hipersensibilidad inmediata a colistina, un antibiótico esencial para su tratamiento, tanto por la sensibilidad del microorganismo, como la falta de disponibilidad de otros medicamentos. Se realizó el procedimiento en Unidad de Cuidados Intensivos de forma exitosa y el paciente completó el esquema del tratamiento. Conclusión: Actualmente el paciente se encuentra en tratamiento específico para su enfermedad de fondo y no ha presentado nuevos episodios de exacerbación ni neumonías.

ABSTRACT Introduction: Pharmacological desensitization induces a temporary hyposensitivity to the causative drug in patients with Immunoglobulin E-mediated drug reactions. Case of report: We present the case of a patient with respiratory exacerbation of cystic fibrosis who presented immediate hypersensitivity to colistin, an essential antibiotic for its treatment-both due to the sensitivity of the microorganism and the lack of availability of other drugs. The procedure was successfully performed in the Intensive Care Unit, and the patient completed the treatment schedule. Conclusion: Currently, the patient is undergoing specific treatment for his underlying disease and has not presented any new episodes of exacerbation or pneumonia.

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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.

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Introdução: A hipersensibilidade a fármacos é uma condição clínica debilitante, acompanhada de experiência emocional intensa e pode afetar a qualidade de vida relacionada à saúde (QVRS). A repercussão das reações de hipersensibilidade a drogas (RHD) na qualidade vida (QV) pode ser verificada pela utilização de questionário específico, o Drug Hypersensitivity Quality of Life Questionnaire (DrHy-Q), desenvolvido originalmente na língua italiana. O objetivo foi traduzir, adaptar transculturalmente e validar a versão do DrHy-Q para a língua portuguesa (cultura brasileira, DrHy-Qb), verificando a consistência interna, validação de constructo e reprodutibilidade do DrHy-Qb, como instrumento específico de avaliação da QV nos pacientes brasileiros com hipersensibilidade a fármacos. Métodos: A adaptação do questionário consistiu na tradução e retrotradução realizadas de forma independente por três tradutores bilíngues, seguidas por pré-teste. A versão final, DrHy-Qb juntamente com o questionário de qualidade de vida resumido (SF-36), foi respondido por 84 pacientes (69% feminino, 40,3±15,2 anos) acompanhados em ambulatório especializado. Na análise fatorial, a validação de constructo foi realizada pelo cálculo do coeficiente de correlação de Pearson, de consistência interna pelo coeficiente alfa de Cronbach, e da reprodutibilidade pelo coeficiente de correlação intraclasse. Resultados: A análise estatística evidenciou consistência interna (α = 0,936) e reprodutibilidade (r: 0,984; IC95% = 0,963-0,993; p < 0,001) excelentes. A correlação entre o DrHy-Qb e o SF-36 total foi negativa e moderada (r = -0,394; p < 0,01). Conclusões: O DrHy-Qb foi adequadamente traduzido, adaptado e validado para a cultura brasileira, podendo ser útil na avaliação da qualidade de vida dos pacientes com hipersensibilidade a fármacos.

Introduction: Drug hypersensitivity is a clinical condition that can impair health-related quality of life. Originally developed in Italian, the Drug Hypersensitivity Quality of Life Questionnaire (DrHy-Q) measures the impact of drug hypersensitivity on quality of life. The objective of this study was to translate, crossculturally adapt, and validate the DrHy-Q to Brazilian Portuguese (DrHy-Qb). To be successful, the DrHy-Qb must be internally consistent, maintain construct validity, and be reproducible as an assessment tool for quality of life in Brazilian patients with drug hypersensitivity. Methods: Translation and back-translation were performed by 3 bilingual translators, followed by a pretest and a final version. The final version, the DrHy-Qb, and the 36-Item Medical Outcomes Study Short-Form General Health Survey (SF-36) were administered to 84 patients (69% female, 40.3±15.2 years) from a specialized outpatient clinic. Factorial analysis included Pearson's correlation coefficient to validate the construct, Cronbach's alpha to assess the internal consistency, and intraclass correlation coefficient to determine reproducibility. Results: Statistical analysis showed excellent internal consistency (α = 0.936) and reproducibility (r: 0.984; 95% CI: 0.963-0.993; p < 0.001). The correlation between the DrHy-Qb and the SF-36 was moderate and negative (r: -0.394; p < 0.01). Conclusions: This study showed that the DrHy-Qb was successfully translated, adapted, and validated into Brazilian Portuguese, and can be used to assess quality of life in patients with drug hypersensitivity.

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Introduction: Stevens-Johnson syndrome (SJS) is caused by a delayed immune response triggered by the association of genetic and environmental factors. This reaction can be mediated mainly by some drugs. Objective: The purpose of this article is to report the case of a patient with SJS associated with carbamazepine. Case report: A man was hospitalized complaining of odynophagia, whitish plaques in the mouth and swelling of the lips. Clinical examination revealed ulcerated and erosive lesions involving the buccal mucosa, lips, tongue and hard palate. The lips were swollen and with hemorrhagic crusts. Papular and purplish-colored lesions were observed on the skin. The patient reported that the lesions started 10 days ago, coinciding with the start of carbamazepine use. The diagnosis of SJS was established based on clinical information and the patient treated with support therapy. Conclusion: SJS is a clinical condition that affects the oral mucosa and can be triggered by the use of carbamazepine. (AU)

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Os anti-inflamatórios não esteroidais (AINE) são os fármacos mais frequentemente associados a reações de hipersensibilidade (RH) na prática clínica. Na parte 2 dessa atualização sobre as RH aos AINE, discutiremos os aspectos clínicos dessas reações, com foco nos sinais e sintomas, como diferenciar os fenótipos clínicos, fazer a orientação desses pacientes e quando indicar procedimentos complementares, como testes cutâneos, de provocação e dessensibilização.

Nonsteroidal anti-inflammatory drugs are a major cause of drug hypersensitivity reactions in clinical practice. In this "Update Part 2", we discuss the clinical picture, including the main signs and symptoms, how to distinguish clinical phenotypes, how to manage affected patients, and when to indicate additional procedures, such as skin testing, challenge, and desensitization.

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ABSTRACT BACKGROUND AND OBJECTIVES: Peripheral neuropathy is a rare condition with many etiologies. Common symptoms are numbness, paresthesia, weakness and neuropathic pain. Treatment consists in frst-line agents such as anticonvulsants and some antidepressants. Te aim of this study was to report a case of chronic pain refractory to several therapies in a patient with absolute contraindication to the use of all anticonvulsants and antidepressants drugs. CASE REPORT: Female patient, a 40-year-old treated for trigeminal neuralgia with decompression that developed chronic occipital pain refractory to radiofrequency and onset of transient and bilateral T4 sensory and motor polyneuropathy after viral meningitis. In addition, she showed a severe pharmacodermy (Drug Rash with Eosinophilia and Systemic Symptoms- DRESS Syndrome) after using carbamazepine and other anticonvulsants, as well as allergy to all analgesics and opioids except morphine. Epidural puncture with insertion of a catheter was performed aiming at a 5-day test through intermittent epidural morphine bolus to assess the possibility of morphine pump implantation. CONCLUSION: The test was successful and the patient referred to the neurosurgery team. At the 6-month follow-up after the insertion of the morphine intrathecal pump, the strategy has proven to be efective in controlling pain secondary to polyneuropathy.

RESUMO JUSTIFICATIVA E OBJETIVOS: Neuropatia periférica é uma condição rara, de etiologia multifatorial. Dormência, parestesia, redução de força muscular e dor neuropática são sintomas comuns. O tratamento consiste em uso de anticonvulsivantes e antidepressivos. O objetivo deste estudo foi relatar o caso de dor crônica refratária a diversas terapias de uma paciente com contraindicação absoluta para uso de todos os fármacos anticonvulsivantes e antidepressivos. RELATO DO CASO: Paciente do sexo feminino, 40 anos, com história de neuralgia do trigêmeo abordada previamente com cirurgia, com cefaleia occipital crônica refratária à radiofrequência e polineuropatia bilateral T4 sensorial e motora após meningite viral. No curso do tratamento, apresentou grave farmacodermia (Drug Rash with Eosinophilia and Systemic Symptoms - Síndrome DRESS) após o uso de carbamazepina e outros anticonvulsivantes, além de reação alérgica a todos analgésicos e opioides, exceto morfina. Optou-se por analgesia teste por via peridural, durante 5 dias, com bolus intermitentes e diários de morfina para avaliação de possibilidade de implante de bomba de morfina. CONCLUSÃO: O teste foi considerado bem-sucedido e a paciente encaminhada para neurocirurgia. No seguimento de 6 meses após implante de bomba por via subaracnoidea, esta estratégia se mostrou eficaz no controle da dor secundária à polineuropatia.

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Introduction: About 80-95% of patients infected with the human immunodeficiency virus (HIV) develop skin manifestations, which are markers of the patients' immune status. Objective: To describe the dermatologic manifestations and the clinical and sociodemographic factors of hospitalized patients diagnosed with HIV and their correlation with CD4 T-lymphocyte count. Materials and methods: We conducted an observational, cross-sectional, and retrospective study of the medical records of 227 adult patients with HIV diagnosis evaluated by dermatology in a hospital in Medellín, Colombia. Results: We included 227 patient records with 433 dermatologic manifestations, 64.4% of them infectious. The most frequent manifestations were oral candidiasis, condylomata acuminata, and drug reactions. Moreover, a statistically significant relationship was found between disseminated herpes zoster virus and secondary syphilis with a CD4 count between 200-499 cells/mm3 (p=0.04 and 0.028, respectively). There was also a statistically significant relationship between oral candidiasis and a CD4 count of less than 100 cells/mm3 (p=0.008). Conclusions: The relationship between disseminated herpes zoster with CD4 between 200-499 cells/mm3 suggests that, despite having high CD4 counts, severe forms of the disease may occur due to possible T-cell dysfunction and depletion of the immune system. Additionally, the relationship between oral candidiasis and CD4 less than 100 cells/mm3 indicates the potential role of oral candidiasis as an essential marker of weakened immune status in HIV patients.

Introducción. Entre el 80 y el 95 % de los pacientes infectados por el virus de inmunodeficiencia humana (HIV) desarrollan manifestaciones en la piel que sirven como marcadores de su estado inmunológico. Objetivos. Describir las manifestaciones dermatológicas y los factores clínicos y sociodemográficos de los pacientes hospitalizados con diagnóstico de HIV y su correlación con el recuento de linfocitos T CD4. Materiales y métodos. Se hizo un estudio observacional de corte transversal y retrospectivo a partir del registro de las historias clínicas de 227 pacientes mayores de edad con diagnóstico de HIV, evaluados por dermatología en un hospital de Medellín, Colombia. Resultados. Los 227 registros daban cuenta de 433 manifestaciones dermatológicas, el 64,4 % de ellas infecciosas. Las tres manifestaciones más frecuentes fueron candidiasis oral, condilomas acuminados y reacciones a medicamentos. Se encontró una relación estadísticamente significativa entre el virus del herpes zóster (HZ) diseminado y la sífilis secundaria, con un recuento de CD4 entre 200 y 499 células/mm3 (p=0,04 y 0,028, respectivamente), y entre la candidiasis oral y un recuento de CD4 menor de 100 células/mm3 (p=0,008). Conclusiones. La relación entre el herpes zóster diseminado y un recuento de CD4 entre 200 y 499 células/mm3 sugiere que, a pesar de los recuentos altos, se pueden presentar formas graves de la enfermedad debido a una posible disfunción de las células T y el agotamiento del sistema inmunológico. La relación entre la candidiasis oral y un recuento de CD4 menor de 100 células/mm3 plantea la posibilidad de considerar esta infección micótica como un marcador importante de debilitamiento inmunológico de los pacientes con HIV.

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El síndrome de hipersensibilidad a medicamentos, con eosinofilia y síntomas sistémicos (DRESS de sus siglas en inglés) es un síndrome muy infrecuente que se caracteriza por erupción dérmica, eosinofilia y compromiso sistémico. Inicialmente fue descrito asociado a medicamentos anticrisis (antiepilépticos), pero posteriormente ha sido descrito en asociación a otros medicamentos. Entre los medicamentos anticrisis, se ha descrito predominantemente su asociación a fármacos aromáticos o con estructura similar: fenitoína, carbamazepina, fenobarbital, oxcarbazepina y lamotrigina. Entre los medicamentos anticrisis no aromáticos, se ha descrito su presentación en algunos pacientes expuestos a gabapentina, zonisamida, valproato, rufinamida y raramente a levetiracetam. La severidad del síndrome se relaciona al tiempo de exposición al fármaco, por lo que su detección precoz es de vital importancia. Describimos el primer caso en nuestro país, de un paciente con DRESS inducido por levetiracetam y revisamos los infrecuentes reportes de DRESS en pacientes expuestos a levetiracetam descritos en la literatura.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a very rare syndrome characterized by skin rash, eosinophilia and systemic compromise. This was initially described in association with antiseizures drugs, but later it was described in association with other drugs. Among antiseizure drugs, it has been described association with aromatic drugs as phenytoin, carbamazepine, phenobarbital, oxcarbazepine and lamotrigine; and non-aromatic antiseizure drugs as gabapentin, zonisamide, valproate, rufinamide and rarely to levetiracetam. The severity of the syndrome is related to the time of exposure to the drug, so early detection is of vital importance. We describe the first case in our country, of a patient with DRESS induced by levetiracetam, and we review the infrequent scientific reports of DRESS in patients exposed to levetiracetam.