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

Treatment of DRESS Syndrome

The first and most crucial step in managing DRESS syndrome is immediate discontinuation of the suspected causative drug, followed by prompt dermatology consultation and initiation of systemic corticosteroids. 1, 2

Initial Management

• Immediately discontinue the suspected causative drug as this is the cornerstone of DRESS syndrome management 1, 2
• Obtain prompt dermatology consultation for all suspected cases 1
• Perform a complete workup including:
  • Total body skin examination
  • Complete blood count with differential (to assess for eosinophilia >700/μL or >10%)
  • Liver and kidney function tests
  • Urinalysis
  • Blood cultures
  • Skin biopsy if diagnosis is uncertain 1, 3

Treatment Protocol

Systemic Therapy

• Initiate systemic corticosteroids as first-line therapy:
  • IV methylprednisolone 1-2 mg/kg/day for severe cases 1, 2
  • Oral prednisone for less severe cases 4
• Wean corticosteroids slowly over at least 4 weeks to prevent relapse 1, 2
• Consider intravenous immunoglobulin (IVIG) at a total dosage of 1-2 g/kg in patients not responding to systemic steroids 1
• Cyclosporine may be considered in severe or steroid-unresponsive cases 1

Supportive Care

• For severe DRESS syndrome, admit to a burn unit or ICU with dermatology and wound care services consultation 1, 5
• Manage fluid and electrolyte balance 1, 2
• Minimize insensible water losses 1
• Implement infection prevention measures 1, 5
• Provide topical treatment and oral antihistamines for symptomatic relief 1, 2
• Use serial clinical photography to track skin manifestations 1

Specialized Care Based on Organ Involvement

• Consult appropriate specialists based on organ involvement:
  • Hepatology for liver involvement (elevated ALT, AST)
  • Nephrology for kidney involvement (elevated creatinine)
  • Cardiology for cardiac involvement (myocarditis, pericarditis)
  • Pulmonology for lung involvement (pneumonitis)
  • Ophthalmology, otolaryngology, urology, or gynecology for mucous membrane involvement 1, 2, 3
• Consider pain or palliative care consultation for symptom management 1

Special Considerations

• Unlike Stevens-Johnson syndrome, corticosteroids are not contraindicated in DRESS syndrome 1
• Adequate immunosuppression is necessary with corticosteroids or other agents due to T-cell immune-directed toxicity 1, 2
• Avoid prophylactic use of corticosteroids when initiating medications known to cause DRESS, as this has not proven effective 1
• Be aware that relapse can occur in approximately 12% of cases, particularly with DRESS associated with viral reactivation 2
• Monitor patients closely as DRESS syndrome has a mortality rate of approximately 10%, primarily from visceral organ compromise 5

Duration of Treatment

• Systemic corticosteroids should be weaned over at least 4 weeks 1
• Prolonged immunosuppression may be necessary due to the T-cell immune-directed toxicity of DRESS syndrome 1, 2
• Follow patients for at least 3 months to ensure complete resolution and monitor for potential chronic complications 4

Prognosis

• Most patients recover completely after drug withdrawal and appropriate therapy 5
• Some patients may suffer from chronic complications 5
• Mortality rate is approximately 10%, primarily due to visceral organ compromise 5