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

Hama, Natsumi; Abe, Riichiro; Gibson, Andrew; Phillips, Elizabeth

doi:10.1016/j.jaip.2022.02.004

Cited by 116 publications

(125 citation statements)

References 168 publications

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“…To diagnose DRESS cases, the Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) consortium established its criteria in 2007 (Table 1). [7] Besides, there is also a scoring system of RegiSCAR (Table 2). [8] The presented patient was awarded 5 points according to RegiSCAR, indicating the following: eosinophilia of peripheral blood = 13% (1 point), target erythema which was widespread on trunk and 4 limbs and facial edema (2 points), involvement of internal organs (hepatomegaly and impaired liver function) (1 point), and timing (occurrence of the first symptoms 23 days after the treatment of antiepileptic), as well as the exclusion of another, alternative diagnosis (1 point).…”

Section: Discussionmentioning

confidence: 99%

Antiepileptic drug-induced hypersensitivity syndrome with liver function abnormality and fever as the first manifestation: A case report

Liu

Huang

et al. 2023

Medicine

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Rationale: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe cutaneous drug reaction characterized by fever, lymphadenopathy, hematologic abnormalities, single or multiple organ involvement, and viral reactivation. [1] The most frequently reported offending drugs are aromatic antiepileptic agents, antibiotics, and allopurinol. [2] Though a relatively rare syndrome, DRESS can lead to severe multi-organ system dysfunction, and in some cases even death. DRESS is one of the severe drug eruptions in dermatological diseases, but it is difficult to diagnose for internist. In this paper, a typical drug hypersensitivity syndrome with abnormal liver function and fever as the first manifestations was reported. The objective of this study was to improve the understanding of rare drug hypersensitivity syndrome in digestion and other fields, and to avoid missed diagnosis and misdiagnosis.Patient Concerns: A 33-year-old Chinese female was initially diagnosed with acute hepatic insufficiency. Combined with the suspicious drug history, she developed DRESS with fever, target erythema, left lymph node enlargement, hematological abnormalities, and abnormal liver function.Diagnoses: Combined with the above characteristics, liver toxicity is the main manifestation, accompanied by fever, mainly moderate to high fever (above 38 °C) , sporadic rash, other organs (kidney, immune system) damage, and a marked increase in eosinophil granulocytic. Therefore the patient was diagnosed with definite case of DRESS syndrome based on clinical and laboratory findings.Interventions: Hormones (methylprednisolone 60 mg/day for 12 days and 80 mg/day for 12 days) and immunoglobulins (intravenous immunoglobulin 10 g/day for 5 days and 20 g/day for 7 days) were given.Outcomes: The patient was discharged from the hospital after recovery. One month after discharge, she was re-admitted to the hospital because of elevated blood sugar and was diagnosed as diabetes.Lessons: DRESS syndrome is a rare but life-threatening hypersensitivity reaction. The mortality will be very high if it's not diagnosed and treated timely. This paper presents a successful case of methylprednisolone plus intravenous immunoglobulin therapy, which provides a stronger evidence for the future diagnosis and treatment of the disease.

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Section: Discussionmentioning

confidence: 99%

Antiepileptic drug-induced hypersensitivity syndrome with liver function abnormality and fever as the first manifestation: A case report

Liu

Huang

et al. 2023

Medicine

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“…On more than one occasion, attempts have been made to integrate theories that explain the nature of the syndrome and its relationship with various agents, where they suggest both the participation of an immune disruption (affecting lymphocytes and macrophages), anomalies in the processing of toxification with subsequent grouping of reactive metabolites, genetic variations, and even the presence of infectious agents (such as the Herpes 6 virus); all yielding results not conclusive. 1,2,5,6 In most studies it is emphasized that this syndrome usually appears after the administration of a drug, with aromatic anticonvulsants being most frequently observed, followed by sulfonamides (drug involved in this case), glycopeptide-type antibiotics and allopurinol, among many others. 1,2,[7][8][9][10][11][12][13] The clinical picture usually begins two to six weeks after the start of consumption of the associated drug.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 Currently, the pathological cascade for the genesis and development of this syndrome is not known with certainty, so it has postulated in addition to the pharmacological interaction, phagocytic activation and the release of cytokines mediated by lymphocytes, microbiological invasion (viruses and bacteria) and genetic anomalies, all without reaching a reproducible conclusion. 1,2,5 It is a disease with a variable prognosis: if an early identification and suspension of the associated drug is made, once the recommended treatment is indicated there is a total remission of all manifestations or on the contrary, lead to multi-organ failure and therefore death. Mortality is close to 10%, with secondary liver failure due to eosinophilic invasion being the culprit almost entirely.…”

Section: Introductionmentioning

confidence: 99%

DRESS syndrome by sulfonamides, about a case

Contreras¹,

Zepeda

Gutiérrez³

et al. 2022

Int J Basic Clin Pharmacol

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Among the most feared toxico-dermas is drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome, a rare drug dermatitis that occurs after acute exposure to drugs whose clinical impact is based on multiple organs (skin, liver, kidneys, lungs, heart) and cell lines (eosinophils and lymphocytes). It is an entity with high mortality if it is not identified early, its treatment consists of the immediate suspension of the responsible drug and the administration of steroids, these being the therapeutic protagonists. A 64-year-old male patient with clinical, biochemical and histopathological criteria compatible with DRESS syndrome. It is essential to suspect the clinical course of DRESS syndrome before the appearance of dermatosis with multisystem involvement associated with the use of drugs, emphasis is placed on its early identification and the establishment of timely treatment to modify its prognosis.

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“…Marie-Charlotte Brüggen 1,2,3,4 Stephan Traidl 1,2,5 , Yasutaka Mitamura 1, 6 , Sarah Walsh 4,7 , Lars French 4,8,9 , Emanual Maverakis 10 , Saskia Ingen-Housz-Oro 4,11,12 Manuscript Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as Drug-induced hypersensitivity syndrome (DIHS), is a rare but severe delayed-type drug hypersensitivity reaction [1]. Its reported incidence ranges between 2 and 5 cases per million per year and the mortality between 5 and 10% [2].…”

Section: Authorsmentioning

confidence: 99%

“…GCS are the cornerstone of the management of severe DRESS cases. In therapy-refractory patients, systemic immunosuppressants and more recently JAK inhibitors and anti-IL5R or anti-IL5 antibodies can been used as a second-line treatment [1,9]. The benefit of antivirals (e.g.…”

Section: Authorsmentioning

confidence: 99%

Medical algorithm: Diagnosis and treatment of Drug reaction with eosinophilia and systemic symptoms

Brüggen

Traidl

Mitamura

et al. 2022

Preprint

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Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as Drug-induced hypersensitivity syndrome (DIHS), is a rare but severe delayed-type drug hypersensitivity reaction [[](#ref-0001)1]. Its reported incidence ranges between 2 and 5 cases per million per year and the mortality between 5 and 10% [[](#ref-0002)2]. DRESS is characterized by the occurrence of an extensive rash with face edema, lymphadenopathy and fever and organ damage, all of which seems to result from massive drug-directed T cell response and associated eosinophilia. DRESS is a complex condition, its clinical presentation varies depending on the cutaneous manifestation(s), affected target organ(s) and reaction severity. The diagnosis of DRESS is further challenged by the clinical overlay with autoimmune, infectious and lymphoproliferative conditions, which have to be considered in the differential diagnosis (Table 1). Eosinophilia is detected in only 80 % of DRESS patients and can be masked by e.g. the administration of systemic glucocorticoids (GCS). Furthermore, there are various differences in the DRESS diagnostic criteria (Table 1) developed by the Japanese SCAR (JSPS) [[](#ref-0003)3] and RegiSCAR [[](#ref-0004)4] groups, the most notable being the inclusion of herpes viremia in the criteria developed by the JSPS. All these clinical challenges underline the importance of a systematic and comprehensive approach when encountering a patient with suspected DRESS. Based on the most recent literature and our clinical expertise, we therefore suggest the medical algorithm depicted in Figure 1. DRESS should be evoked as a differential diagnosis in patients with a rash suspected to be drug-related and associated with head-and-neck edema [[](#ref-0005)5]. Clinical history-taking is a critical element to consolidate or discard a drug-related etiology: most importantly, this should explore the dynamics of both possible DRESS clinical symptoms and drug exposure(s) (date of onset, way and length of administration, previous exposures / reactions). A long drug exposure prior to disease onset, i.e. 2-8 weeks, is indicative for DRESS rather than other drug hypersensitivities – but the duration may vary depending on the causative drug. A thorough clinical examination, basic laboratory work-up, electrocardiogram, and - if a rash is present - a skin biopsy should also be performed. If the clinical presentation and drug exposure history substantiate the DRESS diagnosis, additional investigations should be performed depending on the suspected target organ damage (cf. case “complementary, patient-specific work-up”). Once the diagnosis is established, a severity assessment is warranted, since DRESS can range from mild forms with very limited organ damage to fulminant ones, e.g. characterized by (multi-)organ failure. There are no consensual severity scoring. In this algorithm, we suggest the scoring system used in France (RCT DRESSCODE, https://clinicaltrial.gov NCT01987076).

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

Cited by 116 publications

References 168 publications

Antiepileptic drug-induced hypersensitivity syndrome with liver function abnormality and fever as the first manifestation: A case report

Antiepileptic drug-induced hypersensitivity syndrome with liver function abnormality and fever as the first manifestation: A case report

DRESS syndrome by sulfonamides, about a case

Medical algorithm: Diagnosis and treatment of Drug reaction with eosinophilia and systemic symptoms

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