Dress por litio: reporte de un caso pediátrico / Lithium dress: a pediatric case report / Dress por Lítio: relato de caso pediátrico

La reacción a medicamentos con eosinofilia y síntomas sistémicos denominada DRESS(por sus siglas en inglés, Drug Reaction with Eosinophilia and Systemic Symptoms) hace parte de un amplio espectro denominado toxicodermias. La incidencia exacta no es conocida en niños; sin embargo, en la literatura se ha estimado una tasa de mortalidad que puede llegar a ser tan alta como el 10 %. Presentamos el caso de una paciente adolescente con antecedente personal de trastorno afectivo bipolar (TAB), quien recibía de forma ambulatoria sertralina, quetiapina y trazodona. Por presencia de alucinaciones se adicionó litio al manejo. Diez días después acude al servicio de urgencias por aparición de erupción cutánea y síntomas sistémicos, por lo que se sospechó un cuadro clínico secundario a hipersensibilidad a los medicamentos.

The reaction to drugs with eosinophilia and systemic symptoms called DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is part of a broad spectrum called toxicodermias. The exact incidence is not known in children; However, a mortality rate that can be as high as 10% has been estimated in the literature. We present the case of a teenage patient with a personal history of bipolar affective disorder (BD), who received sertraline, quetiapine and trazodone on an outpatient basis. Due to the presence of hallucinations, lithium was added to the management. Ten days later she went to the emergency department due to the appearance of a skin rash and systemic symptoms, for which a clinical condition secondary to hypersensitivity to medications was suspected.

A reação a medicamentos com eosinofilia e sintomas sistêmicos denominada DRESS (Por suas siglas em inglês Drug Reaction with Eosinophilia and Systemic Symptoms) faz parte de um amplo espectro denominado toxicodermias. A incidência exata não é conhecida em crianças; No entanto, uma taxa de mortalidade que pode chegar a 10% foi estimada na literatura. Apresentamos o caso de um paciente adolescente com história pessoal de transtorno afetivo bipolar (TAB), que recebeu sertralina, quetiapina e trazodona em regime ambulatorial. Devido à presença de alucinações, foi adicionado lítio ao manejo. Dez dias depois, recorreu ao pronto-socorro devido ao aparecimento de erupção cutânea e sintomas sistêmicos, suspeitando-se de quadro clínico secundário a hipersensibilidade a medicamentos.

Síndrome de DRESS asociado al consumo de alopurinol: reporte de caso

El síndrome de DRESS (por sus siglas en inglés Drug Reaction with Eosinophilia and Systemic Symptoms) representa una farmacodermia grave con diferentes manifestaciones clínicas y paraclínicas secundarias a una reacción de hipersensibilidad farmacológica. Su incidencia exacta es desconocida pero se estima entre 1 a 1000 y 1 a 10000 casos de exposición a fármacos asociados. Se caracteriza por dermatosis generalizada extensa en conjunto con afección orgánica, linfadenopatia, eosinofilia y linfocitosis atípica. Entre los fármacos comúnmente asociados se encuentran anticomiciales aromáticos, carbamazepina, sulfonamidas y el alopurinol. Mediante el uso de la puntuación RegiSCAR es posible confirmar o descartar una sospecha de diagnóstico. El tratamiento depende de la severidad de presentación incluyendo esteroides tópicos hasta esteroide sistémico de duración variable dependiendo respuesta clínica y bioquímica. Se reporta tasas de mortalidad del 10 al 20% siendo la insuficiencia hepática la principal causa de muerte en estos pacientes. Se presenta el caso de un paciente femenino de 71 años de edad que, posterior a tratamiento con alopurinol, debuta con eritrodermia secundaria a Síndrome de DRESS.

DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) represents severe pharmacodermia with different clinical and paraclinical manifestations secondary to a drug hypersensitivity reaction. The exact incidence is unknown, but it is estimated to be between 1 in 1,000 and 1 in 10,000 cases of exposure to associated drugs. It is characterized by extensive generalized dermatosis, in conjunction with organic involvement, lymphadenopathy, eosinophilia, and atypical lymphocytosis. Commonly associated drugs include aromatic anticonvulsants, carbamazepine, sulfonamides, and allopurinol. By using the RegiSCAR score, it is possible to confirm or rule out a suspected diagnosis. Treatment depends on the severity of presentation, including topical steroids to systemic steroids of variable duration, depending on clinical and biochemical responses. Mortality rates of 10 - 20% have been reported, with liver failure being the main cause of death in these patients. We present the case of a 71-year-old female patient who, after treatment with allopurinol, developed erythroderma secondary to DRESS Syndrome.

An Unusual Case of DRESS Syndrome Presenting with Marked Leucocytosis.

Drug reaction with eosinophilia and systemic symptom (DRESS) syndrome is a rare severe drug-induced idiosyncratic hypersensitivity characterized by maculopapular and/or erythrodermic eruption, fever, peripheral lymphadenopathy, eosinophilia or atypical lymphocytosis, and visceral organ involvement. The estimated incidence of this syndrome ranges from 1/1000 to 1/10,000 drug exposures. In this report, we describe a case of DRESS syndrome in a young female with a unique presentation. The DRESS syndrome can be difficult to diagnose as its clinical findings can mimic those of other systemic diseases. This case emphasizes the importance of incorporation of the patient’s clinical and medication history in the interpretation of hematological investigations.

Drug rash with eosinophilia and systemic symptoms syndrome in an elderly man on carbamazepine � A jeopardising clinical entity

Drug rash with eosinophilia and systemic symptoms syndrome (DRESS) is a rare but serious hypersensitivity drug reaction most frequently associated with antiepileptics. We report a case of carbamazepine-induced DRESS syndrome in a 61-year-old man who was recently initiated on carbamazepine (6 weeks back) and presented with a history of acute febrile illness of 10 days duration. General examination showed multiple erythematous coalescent papules and rash over the body with relative sparing of the face with lymphadenopathy. Laboratory results revealed eosinophilia, atypical lymphocytosis, transaminitis, and negative serology for hepatitis. Registry of severe cutaneous adverse reactions (RegiSCAR) scoring system case is categorized as a definite case with a score of 7. Carbamazepine was discontinued and with the initiation of intravenous steroids; the transaminitis improved, fever and rashes resolved.

Síndrome de reacción a medicamento con eosinofilia y síntomas sistémicos (DRESS) asociado a levetiracetam: Reporte de caso

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.

A case report of Olanzapine induced DRESS Syndrome

DRESS syndrome (Drug reaction with eosinophilia and systemic symptoms) is defined as a drug-induced complex of symptoms consisting of fever, rash, lymphadenopathy, eosinophilia and a wide range of mild to severe systemic presentations. Here we report a case of a 24-year-old female who developed a severe generalized Anasarca, skin erythema, facial puffiness, reddish discoloration over the body, fever, eosinophilia, leukocytosis and hepatitis 30 days after ingestion of olanzapine. Considering the occurrence of fever, eosinophilia, enlarged lymph nodes, typical skin rash and internal organ involvement, a ‘’ Probable diagnosis “of DRESS syndrome was made using the RegiSCAR Criteria. DRESS induced by psychotropic medications have been scarcely reported. Extensive reporting and educating clinicians as well as patients regarding DRESS will lead to decreased morbidity as well as mortality. Further research is warranted in elucidating its pathogenesis with the aim of designing personalized treatment plans

Does sensitization by SARS-CoV-2 immune complexes trigger DRESS syndrome?

Abstract The diagnosis of coronavirus disease (COVID-19) has been a great challenge since the infection affects not only the respiratory system, but also different organs, given the intense inflammatory and autoimmune reaction triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Herein we present a case of a 36-year-old male patient, with some comorbidities and previous use of carbamazepine, who developed a severe condition triggered by COVID-19, including extensive exfoliative erythroderma and severe impairment of liver function, which lasted approximately 80 days.

Losartan-induced DRESS syndrome

Abstract Introduction: drug reactions with eosinophilia and systemic symptoms, known as DRESS syndrome, are a hypersensitivity reaction to medications which can lead to skin lesions and internal organ involvement. This syndrome has typically been associated with a wide variety of medications, including aromatic anticonvulsants, allopurinol and antibiotics as the main culprits. Objective: we present the case of a patient with DRESS syndrome secondary to losartan, manifesting skin symptoms and mild hepatic involvement. Up until now, there have been no reports of losartan as the cause of this condition. Prompt treatment was instated including the withdrawal of the offending medication and initiation of oral systemic steroids, with a satisfactory response. Conclusion: caregivers should be alert to the appearance of skin lesions with the use of different groups of medications, not just those typically reported, since any medication could potentially cause a hypersensitivity reaction. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.2081).

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).

Síndrome de Reacción a drogas con Eosinofilia y Síntomas Sistémicos (DRESS) asociados a Nefritis Túbulo Intersticial Aguda en un adolescente / DRESS síndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) associated with Tubulo Interstitial Nephritis in a adolescent

Resumen El síndrome de DRESS (Reacción a drogas con eosinofilia y síntomas sistémicos) es una patología poco frecuente en Pediatría, descrita por primera vez en 1996, por Bocquet. Puede presentarse en un tiempo variable luego de exposición a algunos medicamentos, se caracteriza por fiebre, compromiso cutáneo y de órganos internos. En este caso, se presenta a un paciente de 13 años, con antecedente de uso de Trimetroprim sulfa desde hace 2 meses, con cuadro de 3 días consistente en fiebre y rash cutáneo, sin compromiso de mucosas, con respuesta no favorable al manejo con esteroide, requiriendo Inmunoglobulina IV. Semanas después del inicio de los síntomas y evolución estable presenta insuficiencia renal aguda que requirió terapia de reemplazo renal. Se descartaron otras patologías subyacentes de índole autoinmune. Hubo recuperación de azoados y normalización de los demás paraclínicos el día 40 de la enfermedad. El paciente continúa asintomático, 4 meses después, con tratamiento con esteroide oral, en descenso lento y gradual. Se debe considerar la evaluación permanente de las pruebas de función renal en los pacientes que presenten Síndrome de DRESS, por su asociación con Nefritis intersticial aguda y complicaciones relacionadas.

Abstract DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a rare pathology in Pediatrics, first described in 1996 by Bocquet. It can appear in a variable period of time after exposure to some medications, it is characterized by fever, skin involvement and internal organs. A 13-year-old patient is presented, with a history of use of Trimethoprim sulfa for two months, with a disease of three days of evolution, consisting of fever and skin rash, without mucosal involvement, with an unfavorable response to steroid management, requiring Intravenous inmunoglobulin. Weeks after the onset of symptoms and stable evolution, he presented acute renal failure that required renal replacement therapy. Other underlying autoimmune pathologies were ruled out. There was recovery of renal function test and normalization of the other paraclinical on day 40 of the disease. Patient remains asymptomatic four months later, with oral steroid treatment, in slow and gradual decline. Permanent evaluation of renal function tests should be considered in patients with DRESS syndrome, due to its association with acute tubulointerstitial nephritis and related complications.

Avaliação da morfologia hepática em ratos após administração do ranelato de estrôncio / Evaluation of hepatic morphology in rats after administration of strontium ranelate

Introdução: o fármaco ranelato de estrôncio (RE) é muito utilizado na terapêutica profilática e no controle da osteoporose. Age sistemicamente diminuindo a reabsorção e aumentando a formação óssea, apresentando eventos adversos pouco esclarecidos na literatura, à exemplo a síndrome DRESS com envolvimento hepático. Objetivo: avaliar a morfologia hepática em ratos norvegicus albinus após administração do RE. Metodologia: estudo experimental com 10 ratos, divididos aleatoriamente em dois grupos, Grupo Controle (GC), sem administração do RE, e Grupo Ranelato de Estrôncio (GRE), ambos acompanhados durante 15 dias, e, em seguida, sacrificados e o fígado de cada animal colocado para fixação no solução de formaldeído a 4% durante 48 horas. Após essa etapa, foram realizados os procedimentos necessários à análise pela microscopia óptica, com lâminas coradas pela hematoxilina e eosina, e picrosirius red.Resultados: nos GC e GRE foram encontradas alterações similares, como reação ductular, dilatação sinusoidal e fibrose perissinusoidal, com intensidades distintas entre os grupos, sendo a reação ductular mais proeminente no GC, e a dilatação sinusoidal e fibrose perissinusoidal mais pronunciada no GRE. Além disso, no GC foram evidenciados achados inflamatórios, como presença de infiltrado inflamatório misto e hiperplasia de células de Kupffer, não visualizados no GRE, implicando numa possível ação anti-inflamatória do RE. Conclusão: pode-se concluir que foram visualizadas diferenças nos achados morfológicos do parênquima hepático dos ratos tratados com o RE em comparação aos não tratados, ainda que esses achados não sejam suficientes para inferir a incidência de um processo patológico característico, como cirrose ou hepatite.

Introduction: the drug strontium ranelate (SR) is widely used in prophylactic therapy and in the control of osteoporosis. It acts by reducing reabsorption and increasing bone formation systemically, presenting unclear adverse events in the literature, such as the DRESS syndrome with hepatic involvement. Objective: to evaluate hepatic morphology in norvegicus albinus rats after SR administration. Methodology: experimental group with 10 rats, divided into two groups, randomly distributed, five from the Control Group (CG), without SR administration, and the other five from the Strontium Ranelate Group (SRG), both followed for 15 days, and then sacrificed and the liver of each animal placed for fixation in 4% formalin for 48 hours. After this step, the procedures necessary for the analysis by optical microscopy were performed, with blades stained by hematoxylin e eosin, and picrosirius red. Results: in CG and SRG, similar alterations were observed, such as ductular reaction, sinusoidal dilatation and perissinusoidal fibrosis, with distinct intensities between the groups, being the ductular reaction more prominent in the CG, and sinusoidal dilation and a perissinusoidal fibrosis more pronounced in the SRG. In addition, in the CG were evidenced inflammatory findings such as the presence of mixed inflammatory infiltrate and Kupffer cell hyperplasia, not visualized in the SRG, implying a possible anti-inflammatory action of SR. Conclusion: it can be concluded that differences were observed in the morphological findings of the hepatic parenchyma of rats treated with SR compared to untreated rats, although these findings are not sufficient to infer the incidence of a characteristic pathological process, such as cirrhosis or hepatitis.

Síndrome DRESS asociado a fármacos antituberculosos / DRESS syndrome associated with antituberculous drugs

RESUMEN El síndrome DRESS es una farmacodermia grave, potencialmente fatal, que se caracteriza por eosinofilia periférica y compromiso sistémico. Los fármacos implicados con mayor frecuencia comprenden anticonvulsivantes, alopurinol, sulfasalazina y antivirales. Otros, como antibióticos, AINES y antituberculosos, también se han reportado como agentes causantes. Presentamos el caso clínico de un paciente masculino de 31 años de edad, con diagnóstico de pleuresía tuberculosa, que recibió tratamiento con isoniacida, rifampicina, pirazinamida y etambutol. A los 15 días desarrolló un exantema cutáneo febril, con compromiso hepático, pulmonar y hemodinámico, que requirió cuidados intensivos. Se suspendió el tratamiento y se administraron corticoides, con buena evolución.

ABSTRACT DRESS syndrome is a serious, potentially life-threatening adverse drug reaction, characterized by peripheral eosinophilia, and systemic compromise. The most frequently implicated drugs include anticonvulsants, allopurinol, sulfazalazine and antivirals. Others, such as antibiotics, NSAIDs and antituberculosis agents, have also been reported as causative agents. We present the clinical case of a 31-year-old male patient, diagnosed with pleural tuberculosis, who was treated with isoniazid, rifampicin, pyrazinamide and ethambutol. Fifteen days after he developed a febrile skin rash, with hepatic, pulmonary and hemodynamic involvement, which required intensive care. The treatment was suspended and corticosteroids were administered, with favorable evolution.

Reporte de caso: purpura diseminada como primera manifestación de síndrome de dress / Case report: purpuriform lesions as the first manifestation of dress syndrome

RESUMEN El síndrome de DRESS es una reacción adversa severa a fármacos presentándose más comúnmente con anticonvulsivantes y antibióticos. Se estima que su mortalidad alcanza el 10%; clínicamente se presenta con fiebre, linfadenopatías, erupciones cutáneas morbiliformes y compromiso deórganos terminal. Las reacciones adversas cutáneas medicamentosas se presentan en más de la mitad de los pacientes que están recibiendo medicamentos sistémicos tales como la carbamazepina.

SUMMARY DRESS syndrome is a severe adverse reaction to medicament which is most commonly presentedwith anticonvulsants and antibiotics. It is estimated that the mortality reaches10%;clinically the patients presentit with fever, lymphadenopathies, morbilliform cutaneous eruptions and terminal organ involvement. Adverse skin reactions to medications occur in more than half of patients who have received systemic medications, such as anticonvulsants.

A case report of drug rash with eosinophilia and systemic symptoms syndrome induced by dapsone

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a distinct, severe, idiosyncratic reaction to a drug characterized by a prolonged latency period. It is followed by a variety of clinical manifestations, usually fever, rash, lymphadenopathy, eosinophilia, and a wide range of mild-to-severe systemic presentations. Among sulfonamides, Dapsone, sulfamethoxazole-trimethoprim and sulfasalazine are the most common offending drugs. We report here a case of DRESS syndrome due to dapsone.

Altered T cell and monocyte subsets in prolonged immune reconstitution inflammatory syndrome related with DRESS (drug reaction with eosinophilia and systemic symptoms)

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe cutaneous adverse reaction involving various internal organs. Flare-ups after recovery from the initial presentation of DRESS are caused by relapse of drug-induced T-cell-mediated reactions. However, the specific underlying mechanism is unclear. Here, we report a case of a 60-year-old man with allopurinol-induced DRESS who suffered recurrent episodes of generalized rash with eosinophilia, which mimicked immune reconstitution inflammatory syndrome. Analysis of immunological profiles revealed that the percentages of T lymphocytes and regulatory T cells in the patient with DRESS were higher than those in healthy controls. In addition, there was a notable change in the subtype of monocytes in the patient with DRESS; the percentage of nonclassical monocytes increased, whereas that of classical monocytes decreased. Upon viral infection, nonclassical monocytes exhibited strong pro-inflammatory properties that skewed the immune response toward a Th2 profile, which was associated with persistent flare-ups of DRESS. Taken together, the results increase our understanding of the pathogenesis of DRESS as they suggest that expansion of nonclassical monocytes and Th2 cells drives disease pathogenesis.

Síndrome DRESS / DRESS syndrome

Resumen: El síndrome DRESS es un reacción de hipersensibilidad a fármacos severa e idiosincrásica, caracterizada por exantema, fiebre, adenopatías, alteraciones hematológicas y afectación de varios órganos. La heterogeneidad de la manifestación clínica representa un desafío diagnóstico para el médico clínico, se requiere alto índice de sospecha y descartar un amplio espectro de diagnósticos diferenciales. Las reacciones cutáneas asociadas con fármacos pueden ser cuadros potencialmente mortales, el diagnóstico oportuno puede modificar el pronóstico del paciente. Describimos el cuadro clínico y tratamiento de un paciente de 15 años con insuficiencia renal crónica que fue hospitalizado por lesiones morbiliformes generalizadas concomitantes con fiebre, linfadenopatías, esplenomegalia y eosinofilia. Descartar procesos infecciosos, autoinmunitarios y neoplásicos fue posible con estudios complementarios; el antecedente de ingestión reciente de alopurinol y los datos clínicos y de laboratorio permitieron establecer el diagnóstico definitivo de síndrome DRESS. El paciente recibió corticoesteroides tópicos y sistémicos, las manifestaciones clínicas revirtieron a partir de la segunda semana de hospitalización. Se insiste en la importancia de la identificación de factores de riesgo asociados con la aparición de este síndrome.

Abstract: Dress syndrome is a severe and idiosyncratic reaction of hypersensitivity to drugs, characterized by rash, fever, lymphadenopathy, hematological alterations and systemic compromise, the heterogeneity of the clinical presentation represents a diagnostic challenge for the clinician, a high clinical suspicion is required and the need to rule out a wide spectrum of differential diagnoses. Cutaneous reactions associated with drugs can be potentially fatal, early diagnosis can modify the patient's prognosis. We describe the clinical case and treatment of a 15-year-old male patient with chronic renal failure who was hospitalized for generalized morbilliform lesions associated with fever, lymphadenopathy, splenomegaly and eosinophilia. Complementary studies ruled out infectious, autoimmune and neoplastic processes; the antecedent of recent intake of allopurinol together with clinical and laboratory data allowed to establish a definitive diagnosis of DRESS syndrome. Patient received topical and systemic corticosteroids, clinical manifestations reverted from the second week of hospitalization. We emphasize the importance of identifying risk factors associated with the development of this syndrome.

Síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos debido a la carbamazepina: Caso pediátrico / Drug reaction with eosinophilia and systemic symptoms due to carbamazepine: Pediatric case

Entre las reacciones medicamentosas graves en la piel, se encuentran el síndrome de Stevens-Johnson, la necrólisis epidérmica tóxica y el síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos (drug reaction with eosinophilia and systemic symptoms; DRESS, por sus siglas en inglés), que son poco comunes en la población pediátrica (incidencia: 1/1000-10 000 niños), sin embargo, tienen mal pronóstico. El síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos consiste en erupciones cutáneas, alteraciones hematológicas, linfadenopatía y afectación de órganos. Se presenta el caso de un paciente masculino de 12 años que desarrolló esta patología después de iniciar el tratamiento anticonvulsivo con carbamazepina. Se considera que es importante que el personal de la salud tenga conocimiento de esta enfermedad para que sea incluida entre los diagnósticos diferenciales de pacientes con afecciones similares, ya que este síndrome es potencialmente mortal.

Severe skin reactions include Stevens-Johnson Syndrome, toxic epidermal necrolysis and Drug reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome, which are uncommon in the pediatric population (incidence 1/1000-10 000 children), but they have bad prognosis. Drug-sensitive Syndrome with eosinophilia and systemic symptoms consists in rash, hematological abnormalities, lymphadenopathy and organ involvement. We report the case of a 12-year-old male patient who developed this pathology after initiating anticonvulsant therapy with carbamazepine. We consider that it is important to be aware of this disease and to include it among the differential diagnoses in patients with similar conditions because this syndrome is life-threatening.

Trichloroethylene Hypersensitivity Syndrome: Should Be Considered When Diagnosing DRESS Syndrome

Trichloroethylene (TCE) is an organic solvent that is used for degreasing and removing impurities from metal parts. However, this solvent's characteristics and hypersensitivity can produce clinical patterns and laboratory data that mimic drug rash with eosinophilia and systemic symptoms (DRESS) syndrome. Thus, exposure confirmation is critical to making an accurate diagnosis. This is a case of TCE-induced hypersensitivity syndrome (TCE HS) in a 24-year-old Indonesian man who was working in an electro-plating business. He was admitted to a referral hospital after one month of working, and exhibited a fever with skin symptoms. He was administered immunosuppressive therapy based on an assumed diagnosis of DRESS syndrome, although he subsequently experienced cardiac arrest and did not respond to resuscitation. An investigation into his disease history confirmed that he was prescribed medications one week before he developed the skin disease, and had been periodically exposed to TCE for the previous 4 weeks. Based on these findings, it was believed that his clinical course was caused by TCE HS, rather than DRESS syndrome.

Allopurinol-induced severe cutaneous adverse reactions: A report of three cases with the HLA-B*58:01 allele who underwent lymphocyte activation test

Allopurinol-induced severe cutaneous adverse reactions (SCARs) such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome are reportedly associated with the HLA-B*58:01 genotype. Three patients who developed SCARs after allopurinol administration were subjected to HLA-B genotyping and lymphocyte activation test (LAT) to evaluate genetic risk and to detect the causative agent, respectively. All three patients given allopurinol to treat gout were diagnosed with DRESS syndrome. Symptom onset commenced 7-24 days after drug exposure; the patients took allopurinol (100–200 mg/d) for 2-30 days. HLA-B genotyping was performed using a polymerase chain reaction (PCR)-sequence-based typing (SBT) method. All patients had a single HLA-B*58:01 allele: HLA-B*13:02/*58:01 (a 63-year-old male), HLA-B*48:01/*58:01 (a 71-year-old female), and HLA-B*44:03/*58:01 (a 22-year-old male). Only the last patient yielded a positive LAT result, confirming that allopurinol was the causative agent. These findings suggest that patients with HLA-B*58:01 may develop SCARs upon allopurinol administration. Therefore, HLA-B genotyping could be helpful in preventing serious problems attributable to allopurinol treatment, although PCR-SBT HLA-B genotyping is time consuming. A simple genotyping test is required in practice. LAT may help to identify a causative agent.

Sulfasalazine Induced DRESS Syndrome: A Review of Case Reports.

Introduction: Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is designated as a lethal adverse drug effect with characteristic sign and symptoms such as skin rashes, fever, leukocytosis with eosinophilia or atypical lymphocytes, lymph node enlargement, and liver or renal dysfunction. Incidences of the DRESS range from 1/1000-1/10,000 drug exposures and are associated with a mortality rate of 10%. Pathogenesis of DRESS relates to an abnormal immune response in a genetically vulnerable individual, i.e. presence of human leukocyte antigen (HLA)*5801 and HLA-B* 5701 genotype and slow acetylation metabolic pathways. Methods: 48 cases were associated with the “Sulfasalazine-induced DRESS syndrome” reported between January 1990- March 2015 in PubMed-MEDLINE and HighWire Press. The “RegiSCAR” scoring system was used to analyze the case reports. Using this system, cases were classified into 4 categories as “no”, “possible, “probable” and “definite”. Results: The vast majority of cases were classified as “probable/definite” DRESS cases (83%). Hypereosinophilia, atypical lymphocytes and fever were significantly associated with “probable/ definite” DRESS cases. Liver involvement and skin rash was described in almost all of the cases, including “possible cases”. DRESS was found fatal in two cases. Conclusion: Awareness of DRESS is essential for diagnosis with the presence of skin rash, liver involvement, fever, hyper eosinophilia and lymphadenopathy. Early identification, followed by a prompt withdrawal of the culprit drug is the most essential measure to avoid disease evolution and to restore wellness.