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

Treatment of DRESS Syndrome

The treatment of DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome requires immediate discontinuation of the offending drug, systemic corticosteroids, and supportive care in a specialized setting such as a burn unit or ICU. 1

Initial Management

• Immediate discontinuation of the suspected causative drug is the first and most crucial step in managing DRESS syndrome 1
• Prompt dermatology consultation should be obtained for all suspected cases of DRESS syndrome 1
• Complete workup including:
  • Total body skin examination with attention to all mucous membranes 1
  • Complete blood count with differential (to assess eosinophilia) 1
  • Liver and kidney function tests 1
  • Urinalysis (to assess for associated nephritis) 1
  • Blood cultures if fever is present 1
  • Skin biopsy if diagnosis is uncertain 1

Treatment Protocol

For Severe DRESS (Grade 3-4):

• Admit patient immediately to a burn unit or ICU with dermatology and wound care services consultation 1
• Initiate IV methylprednisolone at 1-2 mg/kg/day, tapering when toxicity resolves to normal 1
• Provide supportive care including:
  • Fluid and electrolyte balance management 1
  • Minimizing insensible water losses 1
  • Infection prevention 1
  • Topical treatment with emollients and high-strength topical corticosteroids 1
  • Oral antihistamines for symptomatic relief 1

For Steroid-Unresponsive Cases:

• Consider intravenous immunoglobulin (IVIG) at a total dosage of 1-2 g/kg in patients not responding to systemic steroids 1, 2
• Cyclosporine may also be considered in severe or steroid-unresponsive cases 1

Specialized Care

• Consult appropriate specialists based on organ involvement:

  • Ophthalmology, otolaryngology, urology, or gynecology for mucous membrane involvement to prevent sequelae from scarring 1
  • Pain or palliative care consultation for symptom management 1

• Monitor closely using serial clinical photography to track skin manifestations 1

Duration of Treatment

• Systemic corticosteroids should be weaned over at least 4 weeks 1
• Prolonged immunosuppression may be necessary in DRESS syndrome due to its T-cell immune-directed toxicity 1

Special Considerations

• Unlike in Stevens-Johnson syndrome, corticosteroids are not contraindicated in DRESS syndrome as the underlying mechanism is T-cell immune-directed toxicity 1
• Adequate immunosuppression is necessary with corticosteroids or other agents and may need to be prolonged 1
• Follow patients closely as DRESS syndrome can have a protracted course with potential flares during steroid tapering 3

Pitfalls and Caveats

• Do not delay treatment while attempting to identify the causative agent; immediate discontinuation of all suspected drugs is critical 4
• Avoid prophylactic use of corticosteroids when initiating medications known to cause DRESS, as this has not proven effective and may increase the risk of skin rash 1
• Be aware that DRESS typically occurs after 6 weeks of treatment with the offending drug, which distinguishes it from other drug reactions 1
• Monitor for chronic complications as approximately 10% of patients die from visceral organ compromise despite appropriate treatment 3

By following this treatment protocol and addressing all aspects of DRESS syndrome management, mortality and morbidity can be significantly reduced.