DRESS Syndrome
Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome
DRESS syndrome is a severe, potentially life-threatening drug reaction characterized by extensive rash, fever, lymphadenopathy, hematologic abnormalities, and internal organ involvement.
Key Points
- Mortality rate: 10-30%
- Onset: Usually 2-8 weeks after drug exposure
- Most common in children and young adults
- Long recovery period (weeks to months)
Underlying Mechanisms
Immunological Factors
- Type IV hypersensitivity reaction
- T-cell mediated immune response
- Genetic predisposition (HLA associations)
- Viral reactivation (particularly HHV-6)
Common Triggering Medications
- Anticonvulsants:
- Carbamazepine
- Phenytoin
- Phenobarbital
- Lamotrigine
- Antibiotics:
- Sulfonamides
- Minocycline
- Beta-lactams
- Antiviral drugs:
- Abacavir
- Nevirapine
- Other medications:
- Allopurinol
- Dapsone
- Sulfasalazine
Risk Factors
- Genetic polymorphisms
- Ethnicity (higher risk in some Asian populations)
- Immunological status
- Previous drug reactions
Clinical Manifestations
Cutaneous Manifestations
- Maculopapular rash (>50% body surface area)
- Facial edema
- Scaling
- Mucosal involvement
- Progression characteristics:
- Initial onset on face, upper trunk
- Downward spread
- Purpuric lesions in severe cases
Systemic Features
- Fever (>38.5°C)
- Lymphadenopathy
- Usually generalized
- Painful in some cases
- Organ Involvement:
- Liver (80% cases)
- Kidney (40% cases)
- Lung (30% cases)
- Heart (15% cases)
- CNS involvement possible
Hematologic Abnormalities
- Eosinophilia (>700 cells/µL)
- Atypical lymphocytes
- Thrombocytopenia
- Anemia
Diagnostic Criteria and Workup
RegiSCAR Scoring System
- Fever >38.5°C
- Enlarged lymph nodes
- Eosinophilia
- Atypical lymphocytes
- Skin involvement >50% BSA
- Organ involvement
- Resolution ≥15 days
Laboratory Investigations
- Complete blood count with differential
- Liver function tests
- Renal function tests
- Coagulation profile
- Viral studies (HHV-6, EBV, CMV)
- Skin biopsy (when indicated)
Monitoring Parameters
- Daily vital signs
- Serial organ function tests
- Fluid balance
- Skin progression
Treatment Strategy
Immediate Actions
- Discontinuation of culprit drug
- Hospital admission
- Isolation precautions
- Vital sign monitoring
Supportive Care
- Fluid and electrolyte management
- Antipyretics
- Skin care
- Emollients
- Topical steroids
- Wound care if needed
Systemic Therapy
- Systemic corticosteroids
- Starting dose: 1-2 mg/kg/day prednisolone
- Gradual tapering over 6-8 weeks
- Alternative therapies
- IVIG in severe cases
- Cyclosporine
- Plasmapheresis
Potential Complications and Sequelae
Acute Complications
- Hepatic failure
- Renal failure
- Respiratory distress
- Myocarditis
- Sepsis
- DIC
Long-term Sequelae
- Autoimmune diseases
- Thyroid dysfunction
- Type 1 diabetes
- Chronic liver disease
- Psychological impact
Preventive Measures
Primary Prevention
- Genetic screening when applicable
- Careful medication history
- Avoiding cross-reactive medications
- Patient education
Documentation and Communication
- Medical alert bracelet
- Detailed documentation in medical records
- Communication with other healthcare providers
- Family education about risks
