How do you initially manage a patient suspected of DRESS syndrome?

Initial Management of Suspected DRESS Syndrome

Immediately discontinue all suspected causative medications and initiate systemic corticosteroids (IV methylprednisolone 1-2 mg/kg/day) while arranging urgent dermatology consultation. 1, 2, 3

Immediate Actions (First 24 Hours)

Drug Discontinuation

• Stop the offending drug immediately—this is the single most critical intervention that can be life-saving 1, 2, 3, 4
• If multiple drugs are potential culprits and the specific offender is unclear, discontinue all non-essential medications started within the past 2-6 weeks 1, 2
• Common causative agents include anticonvulsants (phenytoin, carbamazepine, phenobarbital), antibiotics (vancomycin, sulfonamides, beta-lactams), allopurinol, and antiretrovirals 1, 5, 6

Urgent Consultation and Admission

• Obtain immediate dermatology consultation for all suspected cases 1, 2, 3
• Admit patients with severe symptoms or organ involvement to hospital; consider ICU or burn unit admission for cases with >50% body surface area involvement, hemodynamic instability, or significant multi-organ dysfunction 1, 3

Initial Diagnostic Workup

• Complete blood count with differential to assess for eosinophilia (>700/μL or >10% of WBCs) and atypical lymphocytes 1, 2, 3
• Comprehensive metabolic panel including liver function tests (ALT, AST, alkaline phosphatase, bilirubin) and kidney function (BUN, creatinine) 1, 2, 3
• Urinalysis to evaluate for nephritis 1, 3
• Blood cultures if febrile to rule out infectious mimics 1
• Skin biopsy if diagnosis is uncertain (will show perivascular lymphocytic infiltration with eosinophils and dermal edema) 1, 3

Pharmacologic Management

First-Line: Systemic Corticosteroids

• Initiate IV methylprednisolone 1-2 mg/kg/day for all suspected DRESS cases 1, 2, 3
• Unlike Stevens-Johnson syndrome, corticosteroids are NOT contraindicated in DRESS and are essential therapy 1, 3
• Plan for a minimum 4-week taper to prevent relapse, as DRESS involves T-cell immune-directed toxicity with long-lasting memory responses 1, 2, 3
• Start proton pump inhibitor for gastrointestinal prophylaxis during corticosteroid therapy 1

Second-Line Therapies (Steroid-Unresponsive Cases)

• Consider intravenous immunoglobulin (IVIG) at 1-2 g/kg total dosage for patients not responding to systemic steroids 1, 3
• Cyclosporine may be considered in severe or steroid-unresponsive cases 1, 3

Supportive Care Measures

• Fluid and electrolyte balance management with attention to minimizing insensible water losses 1, 2, 3
• Infection prevention measures given immunosuppression and skin barrier compromise 1, 2, 3
• Topical corticosteroids for symptomatic relief of skin lesions 1, 2, 4
• Oral antihistamines for pruritus management 1, 2
• Serial clinical photography to track progression of skin manifestations 3

Specialist Consultations Based on Organ Involvement

• Ophthalmology, otolaryngology, urology, or gynecology for mucous membrane involvement 3
• Cardiology if myocarditis or pericarditis suspected (occurs in 4-21% of cases) 1
• Nephrology for significant kidney involvement (creatinine >1.5 times baseline) 1
• Pain or palliative care consultation for symptom management 3

Critical Pitfalls to Avoid

• Do not delay steroid initiation while awaiting definitive diagnosis—clinical suspicion is sufficient to begin treatment 1, 2, 3
• Do not taper steroids prematurely—minimum 4 weeks required, as relapse occurs in 12% of cases with inadequate immunosuppression 1, 2
• Do not perform drug challenge with the suspected culprit except in extreme circumstances, as this can trigger severe reactions 1
• Do not perform patch testing or delayed intradermal testing until at least 6 months after complete resolution and at least 4 weeks after discontinuing systemic steroids 1
• Do not use prophylactic corticosteroids when initiating medications known to cause DRESS, as this has not proven effective 3

Distinguishing DRESS from Other Severe Cutaneous Reactions

• DRESS has a longer latency period (2-6 weeks) compared to Stevens-Johnson syndrome/toxic epidermal necrolysis 1, 2
• DRESS features prominent eosinophilia and multi-organ involvement, particularly hepatitis, which distinguishes it from SJS/TEN 1, 3
• DRESS typically occurs after 6 weeks of treatment, whereas eczematous dermatitis from drugs like telaprevir occurs earlier and SJS/DRESS are suspected when symptoms appear after week 6 7, 3