Disease & disorder

drug rash with eosinophilia and systemic symptoms

MONDO:0015340

DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is a hypersensitivity reaction characterized by a generalized skin rash, fever, eosinophilia, lymphocytosis and visceral involvement (hepatitis, nephritis, pneumonitis, pericarditis and myocarditis) and, in some patients, reactivation of human herpes virus 6.

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Current registry observations. Phase counts may overlap. Study start dates are not registry registration dates. Investigational interventions are not approved-treatment recommendations.

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Background context

Drug rash with eosinophilia and systemic symptoms

Drug rash with eosinophilia and systemic symptoms or drug reaction with eosinophilia and systemic symptoms (DRESS), also termed drug-induced hypersensitivity syndrome (DIHS), is a rare reaction to certain medications. It involves primarily a widespread skin rash, fever, swollen lymph nodes, and characteristic blood abnormalities such as an abnormally high level of eosinophils, low number of platelets, and increased number of atypical white blood cells (lymphocytes). DRESS usually involves damage to the internal organs via inflammation and the syndrome has about a 1.2-7% mortality rate. Treatment consists of stopping the offending medication and providing supportive care. Systemic corticosteroids are commonly used as well but no controlled clinical trials have assessed the efficacy of this treatment. DRESS is classified as one form of severe cutaneous adverse reactions (SCARs). In addition to DRESS, SCARs includes four other drug-induced skin reactions: the Stevens–Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), Stevens–Johnson/toxic epidermal necrolysis overlap syndrome (SJS/TEN) and acute generalized exanthematous pustulosis (AGEP). The SCARs disorders have similar disease mechanisms. New strategies are in use or development to screen individuals at risk for DRESS to aid them in avoiding medications that increase the risk of DRESS. Alternative medications are used in all individuals testing positive for these predispositions. Prior to 1996, there were numerous reports on individuals presenting with a medication-induced disorder now recognized as the DRESS synd...

Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.

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Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.