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

Treatment of DRESS Syndrome

The first-line treatment for DRESS syndrome is immediate discontinuation of the culprit drug and administration of systemic corticosteroids, with methylprednisolone IV 1-2 mg/kg/day recommended for severe cases. 1

Diagnosis and Initial Assessment

• DRESS syndrome is characterized by:

  • Morbilliform rash with facial edema
  • Eosinophilia (>700 cells/μL or >10% of total WBC)
  • Systemic symptoms (fever, lymphadenopathy)
  • Internal organ involvement (hepatitis, nephritis, pneumonitis, myocarditis)
  • Typically occurs 2-6 weeks after drug initiation 1

• Differentiate from other severe cutaneous reactions like Stevens-Johnson syndrome (SJS):

  • DRESS: facial edema, limited mucosal involvement, eosinophilia
  • SJS: blistering/skin sloughing, prominent mucosal involvement 1

Treatment Algorithm

Step 1: Immediate Management

• Discontinue the suspected causative drug immediately 1, 2
• Assess severity based on extent of organ involvement and skin manifestations
• Consider ICU admission for patients with:
  • Extensive skin involvement (≥10% body surface area)
  • Significant mucosal involvement
  • Severe organ dysfunction
  • Hemodynamic instability 1

Step 2: Pharmacological Treatment

• For moderate to severe cases:

  • Methylprednisolone IV 0.5-1 mg/kg/day (moderate) or 1-2 mg/kg/day (severe) 1
  • Continue systemic corticosteroids with gradual tapering over 6-8 weeks to prevent relapse 2, 3

• For mild cases:

  • Topical high-potency corticosteroids
  • Oral antihistamines for pruritus
  • Topical emollients 1

Step 3: Supportive Care

• Continuous hemodynamic monitoring
• Strict hydroelectrolytic balance
• Prevention of secondary infections
• Skin care similar to that for burn patients 1

Step 4: Management of Steroid-Resistant Cases

• Consider add-on therapy with intravenous immunoglobulin (IVIG) at 1-2 g/kg total dosage 4
  • IVIG has shown efficacy in steroid-hyporesponsive cases, resolving fever within a median time of 1 day and improving liver enzymes within 13 days 4
• Cyclosporine may be considered as an alternative second-line therapy 1

Monitoring and Follow-up

• Monitor organ function regularly (liver enzymes, renal function, complete blood count)
• Consult appropriate specialists based on affected organ systems 1, 2
• Educate patients about:
  • Permanent avoidance of the causative medication
  • Creating a complete medical alert for drug allergy
  • Monitoring for potential sequelae, including autoimmune diseases 1

Prognosis

• Most patients recover completely with appropriate therapy
• Approximately 10% mortality rate, primarily from visceral organ compromise 2
• Some patients may develop chronic complications 2

Important Considerations

• Early recognition is crucial as DRESS syndrome can be life-threatening
• The European Society for Medical Oncology (ESMO) guidelines emphasize immediate interruption of therapy for DRESS syndrome 5
• Patch testing or lymphocyte transformation tests may help identify culprit drugs, but should be delayed until ≥6 months after complete healing 1, 2
• Avoid structurally related compounds to prevent cross-reactivity 1