What is the treatment for DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome?

From the Guidelines

The primary treatment for DRESS syndrome involves immediate discontinuation of the suspected causative medication and supportive care, with systemic corticosteroids being the mainstay of therapy, typically starting with prednisone at 1-2 mg/kg/day or equivalent, with a slow taper over 6-8 weeks to prevent relapse.

Treatment Overview

The treatment approach for DRESS syndrome is multifaceted, focusing on the removal of the offending agent, management of symptoms, and prevention of long-term complications.

• Discontinuation of the causative medication is crucial, as continued exposure can exacerbate the condition and lead to severe morbidity and mortality 1.
• Systemic corticosteroids, such as prednisone, are initiated to reduce inflammation and immune system dysregulation, with the dosage typically in the range of 1-2 mg/kg/day or equivalent.
• Supportive care is essential and includes fluid and electrolyte management, antipyretics for fever control, and topical steroids for skin manifestations.
• Antihistamines can be used to manage pruritus, improving the patient's quality of life.

Special Considerations

For severe cases involving major organ dysfunction, more aggressive interventions may be necessary:

• Methylprednisolone pulse therapy (500-1000 mg/day for 3 days) can be considered for severe presentations.
• Patients with severe organ involvement, such as liver failure, may require specialized care, including the administration of N-acetylcysteine.
• IVIG (intravenous immunoglobulin) at 2 g/kg over 5 days may be considered in steroid-resistant cases or those with life-threatening complications.

Monitoring and Tapering

Close monitoring of organ function through regular blood tests is essential throughout treatment.

• The extended steroid taper over 6-8 weeks is crucial to prevent relapse, as DRESS syndrome involves a prolonged immune dysregulation that persists even after the offending drug is eliminated.
• Premature discontinuation of steroids can lead to symptom recurrence, highlighting the importance of a gradual tapering schedule.

From the Research

Treatment Overview

The treatment for DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome involves several steps, including:

• Immediate cessation of the causative medication(s) 2, 3, 4, 5
• Use of topical corticosteroids for symptomatic relief 2
• Systemic steroid therapy, which is generally required 2
• Other immunosuppressants, which have also been employed in treatment and show promise in future therapy 2
• Plasmapheresis in severe cases 3
• Treatment with anti-histamines and emollients in mild cases 3

Management Considerations

• Patients with DRESS syndrome should be managed in an intensive care or burn unit for appropriate care and infection control 2
• Appropriate specialists should be consulted based on the affected organ systems 2
• A thorough clinical history is necessary to identify the culprit drug 5
• Patch testing is the test of choice to search for the culprit in cases of DRESS 5
• Skin biopsy may also be helpful in the diagnostic process 5

Prognosis and Complications

• The estimated mortality due to DRESS is 3.8% 5
• Main causes of mortality include fulminant hepatitis and liver necrosis 5
• Indicators of poor prognosis include an eosinophil count above 6000 × 103/μL, thrombocytopenia, pancytopenia, leukocytosis, and coagulopathy 5
• Early identification and definitive management are crucial to avoid complications 6