What is Drug Rash with Eosinophilia and Systemic symptoms (DRES)?

From the Guidelines

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is a severe cutaneous adverse reaction characterized by a drug-induced hypersensitivity syndrome.

Key Characteristics of DRESS

• It is a type of delayed hypersensitivity reaction, typically occurring more than 6 hours after drug administration, often after days to weeks of treatment 1.
• DRESS is characterized by a skin rash, eosinophilia, and systemic symptoms such as fever, lymphadenopathy, and involvement of internal organs like the liver, kidneys, and lungs.
• The sensitivity of patch testing (PT) for DRESS is greater than 50% for many drugs, but due to the risk of relapse, testing should be avoided until more than 6 months have elapsed from the acute reaction and the patient has been off systemic corticosteroids for more than 1 month 1.

Diagnosis and Testing

• Delayed intradermal testing (dIDT) and PT are used for diagnosis, with dIDT being more sensitive for certain delayed reactions like DRESS 1.
• The performance of these tests should be done at least 6 weeks to 6 months after complete healing of the reaction and 4 weeks after discontinuation of systemic steroids or other immunosuppressants 1.
• The use of PT and/or dIDT can help determine causality and cross-reactivity patterns, especially in cases where multiple drugs are involved 1.

Management and Prevention

• Management involves withdrawal of the offending drug and supportive care, with corticosteroids sometimes used to manage symptoms 1.
• Prevention includes avoiding the culprit drug and potentially other drugs within the same class, depending on cross-reactivity patterns 1.
• For NSAIDs, which are common causes of DRESS, consideration should be given to the chemical structure of the culprit NSAID, and an alternative class might be tolerated 1.

From the Research

Definition and Characteristics of DRESS

• Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is a severe, systemic, T cell mediated drug reaction with combinations of cutaneous, hematologic, and internal organ involvement 2.
• It is also known as Drug-Induced Hypersensitivity Syndrome (DiHS) or Drug Reaction with Eosinophilia and Systemic Symptoms (DReSS) 2, 3.
• DRESS is characterized by widespread skin rash, multi-system involvement, and often eosinophilia 4.

Clinical Features and Diagnosis

• Clinical features of DRESS are delayed, stepwise, and heterogeneous, making this syndrome challenging to recognize and diagnose 2.
• Two sets of validated diagnostic criteria exist that can be employed to diagnose DReSS/DiHS 2.
• In vitro and in vivo tests can be employed to confirm the diagnosis and help identify culprit drugs 2.
• A score by the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) allows to classify suspect patients into no, possible, probable, or definite DRESS cases 5.

Treatment and Management

• The mainstay treatment of DRESS is prompt withdrawal of the culprit drug, supportive treatment, and immunosuppression depending on the severity of disease 2.
• Systemic corticosteroid therapy is the widely used first line of management 4, 6.
• New studies have revealed other potential treatment armamentariums, but the administration of systemic corticosteroids remains the primary treatment option 6.

Triggers and Risk Factors

• Anticonvulsants, sulphonamides, or antibiotics are common triggers of DRESS, with antibiotics being responsible for up to 30% of pediatric cases 5.
• Non-steroidal anti-inflammatory drugs (NSAIDs) such as celecoxib can be triggers in rare cases 4.
• Genetic predisposition through specific human leukocyte antigen (HLA) alleles and metabolism defects, viral reactivation, and immune dysregulation are also involved in the pathogenesis of DRESS 2.