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

Treatment of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)

The cornerstone of DRESS syndrome management is immediate discontinuation of the culprit drug and prompt initiation of systemic corticosteroids, with methylprednisolone 0.5-1 mg/kg/day for moderate cases and 1-2 mg/kg/day for severe cases. 1

Diagnosis and Clinical Features

DRESS syndrome is characterized by:

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

Treatment Algorithm

Step 1: Immediate Management

• Discontinue the suspected culprit drug immediately
• Assess severity based on extent of organ involvement and clinical status
• Admit to intensive care unit if:
  • Extensive skin involvement (≥10% BSA with epidermal detachment)
  • Mucosal involvement
  • Severe organ dysfunction
  • Hemodynamic instability

Step 2: Systemic Corticosteroid Therapy

• Moderate cases: Methylprednisolone 0.5-1 mg/kg/day IV
• Severe cases: Methylprednisolone 1-2 mg/kg/day IV 1
• Continue until clinical and laboratory improvement
• Taper slowly over 6-8 weeks to prevent relapse

Step 3: Supportive Care

• Continuous hemodynamic monitoring
• Strict hydroelectrolytic balance
• Skin care similar to burn patients
• Topical emollients and high-potency topical corticosteroids
• Oral antihistamines for pruritus

Step 4: Management of Steroid-Hyporesponsive Cases

• For patients not responding to systemic steroids within 7 days:
  • Add intravenous immunoglobulin (IVIG) at 1-2 g/kg total dose 2
  • IVIG has been shown to resolve fever within a median of 1 day and improve liver enzymes within a median of 13 days

Step 5: Alternative Therapies for Refractory Cases

• Cyclosporine may be considered as second-line therapy 1, 3
• Plasmapheresis for severe cases with multi-organ involvement 3

Monitoring and Follow-up

• Daily assessment of:

  • Vital signs
  • Skin manifestations
  • Complete blood count with differential
  • Liver and kidney function tests
  • Other organ-specific tests based on involvement

• Long-term follow-up:

  • Monitor for potential sequelae including autoimmune diseases
  • Patch testing and delayed intradermal testing (≥6 months after complete healing)
  • Patient education regarding permanent avoidance of culprit drug and structurally related compounds

Prognosis

• Most patients recover completely with appropriate therapy
• Mortality rate approximately 7-10%, primarily from visceral organ compromise 1, 4
• Risk factors for mortality include:
  • Older age (≥57 years)
  • Kidney involvement
  • ICU admission 4
• Relapse may occur in approximately 8.5% of cases 4

Common Pitfalls and Caveats

1. Delayed recognition: DRESS syndrome often presents 2-6 weeks after drug initiation, making it challenging to identify the culprit drug.

2. Premature steroid tapering: Too rapid tapering of corticosteroids can lead to relapse of symptoms.

3. Failure to distinguish from other severe cutaneous adverse reactions: DRESS syndrome must be differentiated from Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN).

4. Underestimating severity: DRESS syndrome can progress rapidly to multi-organ failure if not managed aggressively.

5. Inadequate monitoring: Patients require close monitoring for development of new organ involvement even after initial improvement.