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 causative medication, hospitalization, and systemic corticosteroid therapy as the mainstay of treatment. 1

Initial Assessment and Diagnosis

• Evaluate for key diagnostic features:

  • Skin rash (typically diffuse, maculopapular)
  • Eosinophilia
  • Systemic symptoms (fever, lymphadenopathy)
  • Internal organ involvement (liver, kidney, lungs, heart)
  • Temporal relationship to drug exposure (typically 2-6 weeks after initiation)

• Laboratory evaluation:

  • Complete blood count with differential (looking for eosinophilia)
  • Liver function tests
  • Renal function tests
  • Consider skin biopsy if diagnosis is uncertain

Management Algorithm

Step 1: Immediate Actions

• Immediately discontinue the suspected culprit medication 1
• Hospitalize the patient - severe cases should be admitted to an ICU or burn unit 1, 2
• Consult dermatology urgently 1

Step 2: Severity Assessment

• Grade 3-4 (severe): Skin involvement >50% BSA and/or presence of:
  • Mucous membrane detachment
  • Vesicles, pustules, purpura
  • Systemic symptoms with abnormal laboratory values 1

Step 3: Systemic Treatment

• Initiate IV methylprednisolone 0.5-1 mg/kg/day for moderate cases 1
• Increase to 1-2 mg/kg/day for severe cases with significant organ involvement 1, 2
• Convert to oral corticosteroids once response is noted
• Taper steroids slowly over at least 4 weeks to prevent rebound 1, 3

Step 4: Supportive Care

• Fluid and electrolyte management
• Minimize insensible water losses
• Prevent secondary infections
• Apply topical emollients and high-potency topical corticosteroids for skin symptoms 1
• Oral antihistamines for pruritus 1

Step 5: Specialist Consultations

• Consult appropriate specialists based on organ involvement:
  • Hepatology for liver involvement
  • Nephrology for renal involvement
  • Pulmonology for lung involvement
  • Cardiology for cardiac involvement 1

Step 6: Alternative Therapies for Steroid-Resistant Cases

• Consider IVIG or cyclosporine in severe or steroid-unresponsive cases 1, 2

Monitoring and Follow-up

• Daily monitoring of:

  • Vital signs
  • Skin involvement
  • Complete blood count
  • Liver and kidney function tests
  • Other affected organ systems

• Continue treatment until:

  • Resolution of skin manifestations
  • Normalization of laboratory abnormalities
  • Resolution of organ dysfunction

Special Considerations

• Prolonged steroid tapering is often necessary due to risk of rebound eosinophilia and symptom recurrence 3
• Patients with delayed onset of certain organ involvement may require more prolonged corticosteroid treatment 3
• Consider patch testing or lymphocyte transformation tests to identify the culprit drug after recovery, especially when multiple medications are suspected 2

Prognosis

• Most patients recover completely with appropriate treatment
• Mortality rate is approximately 10%, primarily due to visceral organ compromise 2
• Some patients may develop chronic complications despite appropriate therapy

Common Pitfalls to Avoid

1. Delayed recognition - Early diagnosis is critical; be vigilant for DRESS after the 6th week of treatment with high-risk medications 1
2. Premature steroid tapering - This can lead to rebound symptoms and worsening of condition 3
3. Inadequate monitoring - DRESS can affect multiple organ systems, requiring comprehensive monitoring
4. Reintroduction of culprit medication - Permanently avoid the causative drug and structurally related compounds
5. Failure to recognize atypical presentations - DRESS may initially present with limited organ involvement before progressing 3, 4

By following this management approach, clinicians can effectively treat DRESS syndrome and minimize the risk of morbidity and mortality associated with this severe adverse drug reaction.