A case of drug-induced hypersensitivity syndrome complicated with fulminant type 1 diabetes and type 2 myocardial infarction.

Drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is a type of drug eruption that causes multiorgan disorders after the administration of certain drugs such as anticonvulsants. Herein, we report the case of a 66-year-old man with DiHS/DRESS complicated by fulminant type 1 diabetes (FT1D), shock, and cardiac involvement who was treated conservatively without systemic corticosteroid administration. He had taken carbamazepine for trigeminal neuralgia for 7 weeks until he noticed eruptions on his trunk. Two days after admission, he developed diabetic ketoacidosis, resulting in hypovolemic shock. The patient was diagnosed with FT1D, and insulin was administered. Additionally, the patient had a fever over 38°C, elevated white blood cells (>20 000/µL), liver dysfunction, atypical lymphocytes, and lymphadenopathy, but no evidence of viral reactivation. The lymphocyte transformation test for carbamazepine was positive, and human leukocyte antigen typing revealed the A31:01 haplotype, a risk factor for carbamazepine-induced cutaneous adverse drug reactions. Collectively, a diagnosis of atypical DiHS and a definitive case of DRESS was made. Moreover, myocardial dysfunction wall motion was observed. A close examination revealed mild coronary artery stenosis, leading to a diagnosis of type 2 myocardial infarction due to relative ischemia. The patient was carefully monitored without systemic corticosteroid administration because both clinical findings and laboratory data peaked on the same day. The patient's eruption and general condition improved, and he was discharged 4 weeks later. While most cases of DiHS/DRESS with cardiac involvement present with myocarditis, the possibility of ischemic heart disease should be considered in patients with cardiac involvement under shock.

DRESS syndrome: quetiapine associated case report and literature review.

Drug rash with eosinophilia and systemic symptoms (DRESS syndrome) is a rare systemic adverse drug reaction with a high mortality rate. Cases of DRESS syndrome have been reported with almost all classes of psychiatric medications, but data remains limited. We describe the case of a 33-year-old woman who presented with acute respiratory distress syndrome secondary to severe pulmonary blastomycosis. Her hospital course was complicated by severe agitation for which the psychiatry consult team was involved and several medications were trialed including quetiapine. She developed a diffuse erythematous rash during her hospital stay and later eosinophilia and transaminitis consistent with DRESS syndrome due to either quetiapine or lansoprazole based on the timeline. Both medications were discontinued, and she was started on a prednisone taper leading to resolution of the rash, eosinophilia, and transaminitis. Her HHV-6 IgG titer later returned elevated at 1:1280. DRESS syndrome along with many other cutaneous drug reactions can be associated with psychiatric medications and familiarity and recognition are imperative. There are limited reports of quetiapine-associated DRESS syndrome in the literature; however, rash and eosinophilia should alert psychiatrists to the potential for quetiapine to be a precipitant for DRESS syndrome.

DRESS/DiHS syndrome induced by Propylthiouracil: a case report.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as Drug-induced hypersensitivity syndrome (DiHS), is a severe adverse drug reaction. Propylthiouracil, a member of thiouracils group, is widely used in medical treatment of hyperthyroidism. Propylthiouracil is associated with multiple adverse effects such as rash, agranulocytosis hepatitis and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, but rarely triggers DRESS/DiHS syndrome. Here, we describe a severe case of propylthiouracil-induced DRESS/DiHS syndrome. CASE PRESENTATION: A 38-year-old female was treated with methimazole for hyperthyroidism at first. 4 weeks later, the patient developed elevated liver transaminase so methimazole was stopped. After liver function improved in 2 weeks, medication was switched to propylthiouracil therapy. The patient subsequently developed nausea and rash followed by a high fever, acute toxic hepatitis and multiple organ dysfunction (liver, lung and heart), which lasted for 1 month after propylthiouracil was started. According to the diagnostic criteria, the patient was diagnosed of DRESS/DiHS syndrome which was induced by propylthiouracil. As a result, propylthiouracil was immediately withdrawn. And patient was then treated with adalimumab, systematic corticosteroids and plasmapheresis in sequence. Symptoms were finally resolved 4 weeks later. CONCLUSIONS: Propylthiouracil is a rare cause of the DRESS/DiHS syndrome, which typically consists of severe dermatitis and various degrees of internal organ involvement. We want to emphasize through this severe case that DRESS/DiHS syndrome should be promptly recognized to hasten recovery.

DRUG-INDUCED HYPERSENSITIVITY SYNDROME CAUSED BY LAMOTRIGINE, A CASE REPORT.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, also known as drug-induced hypersensitivity syndrome (DIHS), is an under-recognized and potentially life-threatening hypersensitivity reaction associated with a variety of medications, many of them anti-epileptics. Patients with DRESS syndrome typically present with rash, swelling, fever, and systemic manifestations. We report a case of a patient admitted to out hospital after the administration of an anticonvulsive drug lamotrigine. She was presented with high fever, rash, face oedema and elevated liver enzymes. At admission, all previous medications were discontinued, systemic corticosteroid therapy was administered, and the patient was monitored for signs of clinical recovery. This case report suggests that in patients presenting with skin rash and systemic abnormalities after a recent change in medications, physicians should consider DRESS syndrome as a possible diagnosis and switch to a more aggressive therapy if removal of the offending agent does not result in clinical improvement. Early diagnosis can reduce the risk of complications and the mortality rate.

Case report: Drug rash with eosinophilia and systemic symptoms syndrome in a patient with anti-interferon-γ autoantibody-associated immunodeficiency.

A 56-year-old Chinese woman with previous disseminated mycobacterium avium complex infection and recurrent cervical abscesses from Burkholderia cepacia complex visited our hospital. She was diagnosed with adult-onset immunodeficiency (AOID) and tested positive for interferon-γ-neutralizing autoantibody. Ceftazidime was administered as the initial antimicrobial treatment, which was later combined with sulfamethoxazole-trimethoprim (SMZ-TMP). She developed drug rash with eosinophilia and systemic symptoms (DRESS) syndrome after SMZ-TMP administration and improved after withdrawal of the culprit antibiotic and systemic glucocorticoids treatment. Her cervical infection was eventually cured after combined therapy of long-term antibiotics and anti-IFN-γ autoantibodies (AIGA) titer-lowering treatments including glucocorticoids, rituximab, and plasmapheresis. This is the first case of DRESS syndrome in the setting of AIGA-induced AOID and is worthy of notice.

Severe graft-versus-host disease-like enterocolitis accompanied with cytomegalovirus-reactivation in drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms.

Drug-induced hypersensitivity syndrome (DIHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug adverse reaction with skin eruption and visceral organ involvement. The characteristic clinical features of DIHS/DRESS are reactivation of human herpesviruses (HHV) and the development of autoimmune diseases, but their pathogenesis and associations are not yet understood. Here, we report a 66-year-old man who presented with fever, generalized erythema, diffuse lymphadenopathy, and diarrhea after 3 weeks of treatment with zonisamide. Reactivation of HHV-6 and cytomegalovirus (CMV) was detected during the clinical course. The patient was diagnosed with DIHS/DRESS and treated with systemic prednisolone, i.v. immunoglobulin therapy, and ganciclovir. However, severe enterocolitis persisted for 6 months. A series of examinations revealed features of both CMV enterocolitis, as indicated by identification of a few CMV-positive cells on immunohistochemical analysis, and graft-versus-host disease (GVHD)-like enterocolitis indicated by orange-peel appearance on endoscopic examination and histopathological loss of goblet cells. Intractable enterocolitis continued and the patient finally died of pneumonia. An autoimmune predisposition in DIHS/DRESS patients in combination with CMV reactivation was considered to trigger the severe enterocolitis of this case that showed GVHD-like features of the gastrointestinal tract. GVHD-like organ damage is a pathological condition rarely observed in DIHS/DRESS but should be recognized as one of the most severe complications of the disease.

Three cases of drug-induced hypersensitivity syndrome associated with mRNA-based coronavirus disease 2019 vaccines.

Drug-induced hypersensitivity syndrome (DiHS) is a severe drug eruption that can induce reactivation of herpesviruses such as human herpesvirus 6, resulting in symptom flare-up and organ damage. DiHS is known as drug reaction with eosinophilia and systemic symptoms (DRESS) in Europe. We report three cases of DiHS that could have been triggered by mRNA-based coronavirus disease 2019 (COVID-19) vaccines. In these three patients, symptoms of DiHS developed 2-6 days after the first dose of an mRNA-based COVID-19 vaccine. Although there have been no reports of DiHS/DRESS induced by mRNA-based COVID-19 vaccines in domestic and international journals despite the progress in vaccination worldwide, we speculate that mRNA-based COVID-19 vaccines might have triggered the development of DiHS/DRESS in our patients. In the current coronavirus epidemic, it might be important to assess mRNA-based COVID-19 vaccination status and date of vaccination when evaluating a patient with DiHS/DRESS.

Atypical, Levetiracetam-induced Hypersensitivity Syndrome Complicated by Fulminant Liver Failure in a Patient Undergoing Hemodialysis.

A 59-year-old man undergoing hemodialysis was administered levetiracetam, after which he developed a systemic rash, high fever, severe liver dysfunction, and leukocytopenia with reactivation of human herpes virus 6. Atypical drug-induced hypersensitivity (DIHS) was diagnosed, and prednisolone was administered at 60 mg/day. However, liver failure rapidly progressed, and the patient died 12 days following treatment. Despite the rarity of DIHS with concomitant fulminant liver failure from levetiracetam and sufficient clearance thereof by hemodialysis, our case suggests that this syndrome may still ensue, resulting in mortality, even in hemodialysis patients. Although no treatment has yet been established, strict monitoring and aggressive treatment may be required.

Drug-Induced Hypersensitivity Syndrome (DIHS)/Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS): Clinical Features and Pathogenesis.

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) is one example of a severe delayed T-cell-mediated adverse drug reaction. DIHS/DRESS presents with fever, widespread rash and facial edema, organ involvement, and hematological abnormalities, including eosinophilia and atypical lymphocytosis. DIHS/DRESS is associated with relapse 2 to 4 weeks after acute symptoms, often coinciding with reactivation of prevalent chronic persistent human herpesviruses such as human herpesvirus 6, EBV, and cytomegalovirus. The mortality of DIHS/DRESS is up to 10% and often related to unrecognized myocarditis and cytomegalovirus complications, with longer-term consequences that contribute to morbidity including autoimmune diseases such as thyroiditis. It is essential that all potential drug causes, including all new drugs introduced within the 8 weeks preceding onset of DIHS/DRESS symptoms, are identified. All potential drug culprits, as well as drugs that are closely related structurally to the culprit drug, should be avoided in the future. Systemic corticosteroids have remained the mainstay for the treatment of DIHS/DRESS with internal organ involvement. Steroid-sparing agents, such as cyclosporine, mycophenolate mofetil, and monthly intravenous immune globulin, have been successfully used for treatment, and careful follow-up for cytomegalovirus reactivation is recommended. Strong associations between HLA class I alleles and DIHS/DRESS predisposition include HLA-B∗13:01 and dapsone, HLA-B∗58:01 and allopurinol, and HLA-B∗32:01 and vancomycin. These have opened a pathway for prevention, risk stratification, and earlier diagnosis. Single-cell sequencing and other studies of immunopathogenesis promise to identify targeted treatment approaches.

Morphologic Spectrum of Lymphadenopathy in Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome.

CONTEXT.­: Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DRESS) is a drug-induced, adverse T-cell-mediated hypersensitivity reaction that most often involves skin. The pathologic findings of DRESS-related lymphadenopathy have been described infrequently in the literature. OBJECTIVE.­: To present a case series of DRESS-related lymphadenopathy with an emphasis on the morphologic spectrum. DESIGN.­: We describe detailed clinical and pathologic findings along with the literature review. We focus on the differential diagnosis between DRESS lymphadenopathy and angioimmunoblastic T-cell lymphoma (AITL). RESULTS.­: There were 4 men and 1 woman with a mean age of 41 years (range, 23-59 years). One patient (20%) died. Three lymph node biopsy specimens showed a pattern reminiscent of AITL (AITL-like pattern) and 2 cases showed necrotizing lymphadenitis (Kikuchi-like pattern), associated with vasculitis in 1 case. The AITL-like morphology of DRESS-related lymphadenopathy may be difficult to distinguish from genuine AITL. The clinical information is important for differential diagnosis, including history of drug exposure, age, and the rarity or absence of AITL-associated manifestations such as hemolytic anemia and hypergammaglobulinemia. Molecular analysis of the T-cell receptor genes is helpful, typically revealing a polyclonal pattern in DRESS-related lymphadenopathy. CONCLUSIONS.­: In the literature, 4 histologic patterns of DRESS lymphadenopathy have been described: reactive lymphoid hyperplasia, necrotizing lymphadenitis, Hodgkin lymphoma-like, and AITL-like. These patterns, particularly those that resemble lymphoma, highlight the importance of correct diagnosis to avoid unnecessary therapies.

An investigation into the avoidability of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rising morbidity amongst hospitalized patients. Whilst clinical protocols for the management of individual DRESS cases have been well established, determination of potential prevention of these cases by utilizing novel "avoidability" tools has remained unexplored. This retrospective study reviewed records of patients who presented to the emergency department of Weill Cornell Medicine-affiliated Hamad General Hospital, Doha Qatar with suspected DRESS syndrome. These cases were independently adjudicated (utilizing the RegiSCAR, and JSCAR tools) as DRESS-drug pairs by a team of two clinical pharmacists and two General Physicians. They were then rated for potential avoidability with the Liverpool adverse drug reactions avoidability tool (LAAT) by the same team of raters. A total of 16 patients satisfied RegiSCAR criteria for DRESS syndrome. The mean age of the study population was 41.5 years (SD ± 13.3). The study population was predominantly male (n = 12; [75%]). The median latent period from drug ingestion to clinical presentation was 14 days (interquartile range [IQR] 6.5, 29). The median RegiSCAR and J-SCAR scores were 6 (IQR 5, 6.8), 5 (IQR 4, 5.8) respectively. Utilizing the LAAT, about 60% of the DRESS syndrome-drug pairs were rated as "avoidable" ("probable" or "definite"). The overall Krippendorf's alpha with the LAAT was 0.81 (SE 0.10, CI 0.59-1.00); with an intraclass correlation coefficient (ICC) of 0.90 (CI 0.77, 0.96.). In a randomly selected cohort of DRESS syndrome-drug pairs, a significant proportion was potentially avoidable ("possibly" and "definitely") utilizing the LAAT. This will need validation by larger sample-sized prospective studies utilizing the updated LAAT proposed by this study.

Síndrome de DRESS en una paciente con fibrosis quística: reporte de un caso pediátrico / DRESS syndrome in a patient with cystic fibrosis: a pediatric case report

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

Síndrome de reacción a drogas con eosinofilia y síntomas sistémicos asociado a herpesvirus humano 6. Caso pediátrico tratado con ciclosporina y corticoides / Drug reaction with eosinophilia and systemic symptoms syndrome associated with human herpesvirus 6. A pediatric case treated with cyclosporine and corticosteroids

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.

When fever is more than infection: two cases of vancomycin-induced drug reaction with eosinophilia and systemic symptoms (DRESS).

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, but serious systemic hypersensitivity reaction associated with a range of medications. We present two cases of vancomycin-induced DRESS, which occurred simultaneously in the orthopaedic ward in an outer metropolitan hospital. These cases demonstrate the complexity in the diagnosis and management of this inflammatory syndrome on the background of known infection as well as evidence for linezolid as an alternative to vancomycin. The first case was managed conservatively, but developed progressive renal and liver injury along with demonstrated cytomegalovirus reactivation and recurrent colitis, and was eventually palliated. The second was commenced on intravenous glucocorticoids and achieved remission, although had ongoing renal dysfunction at the time of discharge from outpatient follow-up.