What is the management of DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome?

Management of DRESS Syndrome

The management of DRESS syndrome requires immediate discontinuation of the suspected causative drug, prompt dermatology consultation, and initiation of systemic corticosteroids as the first-line treatment. 1, 2

Initial Assessment and Diagnosis

• DRESS syndrome is characterized by a delayed hypersensitivity reaction that typically occurs 2-6 weeks after exposure to the offending drug, presenting with morbilliform rash, fever, lymphadenopathy, and multi-organ involvement 1
• A complete workup is essential, including:
  • Total body skin examination
  • Complete blood count with differential (looking for eosinophilia)
  • Liver and kidney function tests
  • Urinalysis
  • Blood cultures
  • Skin biopsy if diagnosis is uncertain 2

Treatment Algorithm

Step 1: Immediate Management

• Immediately discontinue the suspected causative drug(s) 1, 2, 3
• Obtain prompt dermatology consultation for all suspected cases 1, 2
• For severe cases, admit to a burn unit or ICU with dermatology and wound care services 2

Step 2: Systemic Therapy

• Initiate systemic corticosteroids:
  • IV methylprednisolone 1-2 mg/kg/day for severe cases 1, 2
  • Wean over at least 4 weeks to prevent relapse 1, 2
• For patients not responding to systemic steroids, consider:
  • Intravenous immunoglobulin (IVIG) at a total dosage of 1-2 g/kg 2, 4
  • Cyclosporine in severe or steroid-unresponsive cases 2

Step 3: Supportive Care

• Manage fluid and electrolyte balance 1, 2
• Minimize insensible water losses 1, 2
• Implement infection prevention measures 1, 2
• Provide topical treatment and oral antihistamines for symptomatic relief 1, 2

Step 4: Specialist Consultation

• Consult appropriate specialists based on organ involvement:
  • Hepatology for liver involvement
  • Nephrology for kidney involvement
  • Cardiology for myocarditis or pericarditis
  • Pulmonology for pneumonitis 1, 2

Monitoring and Follow-up

• Use serial clinical photography to track skin manifestations 2
• Monitor organ function with regular laboratory tests 1, 2
• Watch for signs of relapse, which can occur in approximately 12% of cases, particularly with DRESS associated with viral reactivation 1

Special Considerations

• Unlike in Stevens-Johnson syndrome, corticosteroids are not contraindicated in DRESS syndrome 2
• Adequate immunosuppression is necessary due to the T-cell immune-directed toxicity of DRESS 2
• Avoid prophylactic use of corticosteroids when initiating medications known to cause DRESS, as this has not proven effective and may increase the risk of skin rash 2
• Patch testing or intradermal testing should be avoided until at least 6 months after the acute reaction and at least 1 month after discontinuation of systemic corticosteroids 1

Prognosis

• Most patients recover completely after drug withdrawal and appropriate therapy 3
• Some patients suffer from chronic complications 3
• Mortality rate is approximately 10%, primarily from visceral organ compromise 3
• Early recognition and prompt management significantly improve outcomes 1, 2