What is Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)?

From the Guidelines

DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is a severe, potentially life-threatening drug hypersensitivity reaction characterized by skin eruptions, fever, internal organ involvement, lymphadenopathy, and blood abnormalities including eosinophilia. It typically develops 2-8 weeks after starting a new medication, with common culprits including anticonvulsants (carbamazepine, phenytoin), antibiotics (minocycline, vancomycin), allopurinol, and sulfonamides. Management involves immediate discontinuation of the suspected drug, supportive care, and often systemic corticosteroids (typically prednisone 1-2 mg/kg/day, tapered slowly over 2-3 months) 1. Hospitalization is frequently required for severe cases. DRESS has a mortality rate of approximately 10%, primarily due to liver failure, and patients may experience long-term autoimmune complications even after recovery. The pathophysiology involves delayed T-cell-mediated hypersensitivity reactions, often with genetic predispositions and possible viral reactivation (particularly HHV-6) 1. Some key points to consider in the management of DRESS include:

• Immediate discontinuation of the suspected drug
• Supportive care, including fluid and electrolyte balance, minimizing insensible water losses, and preventing infection
• Systemic corticosteroids, such as prednisone 1-2 mg/kg/day, tapered slowly over 2-3 months
• Hospitalization for severe cases
• Avoidance of the causative medication and structurally similar drugs
• Consideration of the risk of DRESS relapse, which is 12% in some studies, and the potential for human herpesvirus reactivation 1. The most recent and highest quality study recommends that patients with DRESS should permanently avoid the causative medication and structurally similar drugs 1.

From the Research

Definition of DRESS

• DRESS stands for Drug Reaction with Eosinophilia and Systemic Symptoms, also known as Drug-Induced Hypersensitivity Syndrome (DiHS) 2.
• It is a severe, systemic, T cell mediated drug reaction with combinations of cutaneous, hematologic, and internal organ involvement 2.

Clinical Features of DRESS

• The clinical features of DRESS are delayed, stepwise, and heterogeneous, making this syndrome challenging to recognize and diagnose 2.
• Clinical manifestations vary, with a monomorphic maculopapular eruption being the most common, whereas facial edema and mucosal involvement are less frequently observed 3.
• Multiple organs are commonly affected, with liver and kidney involvement being prominent, and cardiac involvement is associated with the severity of eosinophilia 3.

Diagnosis of DRESS

• 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.
• The RegiSCAR score, as well as age, may play a role in predicting disease severity 3.

Treatment of DRESS

• The mainstay treatment of DRESS is prompt withdrawal of the culprit drug, supportive treatment, and immunosuppression depending on the severity of disease 2.
• Systemic corticosteroids are the primary treatment option, and other immunosuppressants have also been employed in treatment and show promise in future therapy 4, 3.
• Novel treatment approaches with targeted therapy, cyclosporine, intravenous immunoglobulin, and plasmapheresis have been gaining interest as alternative mono- and adjuvant therapies 5.