What are the symptoms and treatment of DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome?

DRESS Syndrome Symptoms and Treatment

DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a severe, potentially life-threatening drug hypersensitivity reaction characterized by skin rash, fever, lymphadenopathy, hematologic abnormalities, and multi-organ involvement that requires immediate discontinuation of the suspected causative drug, prompt dermatology consultation, and initiation of systemic corticosteroids. 1

Clinical Presentation

Cutaneous Manifestations

• Morbilliform (maculopapular) confluent rash is the most common presentation, typically involving more than 30% of body surface area 1
• Rash may develop 2-6 weeks after exposure to the offending drug, distinguishing it from other drug reactions 1, 2
• Facial edema is commonly present 3

Systemic Manifestations

• Fever (typically moderate to high fever above 38°C) is a hallmark symptom 4
• Lymphadenopathy, particularly cervical and axillary 1, 5
• Constitutional symptoms including rigors, myalgias, and arthralgias 6

Hematologic Abnormalities

• Eosinophilia is characteristic (hence the name) 6, 4
• Atypical lymphocytosis may be present 1

Multi-organ Involvement

• Hepatitis with elevated liver enzymes (most common organ involvement) 3
• Nephritis with abnormal kidney function tests 6
• Pneumonitis presenting as respiratory distress 7
• Myocarditis and pericarditis (less common but potentially fatal) 1

Diagnostic Approach

Laboratory Evaluation

• Complete blood count with differential to identify eosinophilia 2
• Liver and kidney function tests to assess organ involvement 2
• Urinalysis to evaluate for nephritis 6
• Blood cultures if fever is present to rule out infection 2

Imaging and Additional Testing

• Skin biopsy if diagnosis is uncertain 2
• Serial clinical photography to track skin manifestations 2
• Consider viral studies as herpes virus reactivation may be associated with DRESS 1

Treatment Protocol

Immediate Management

• Discontinue the suspected causative drug immediately - this is the most crucial first step 1, 2
• Obtain prompt dermatology consultation for all suspected cases 2
• Admit patients with severe symptoms or organ involvement to hospital, consider ICU or burn unit for severe cases 6, 2

Pharmacological Management

• Initiate systemic corticosteroids: IV methylprednisolone 1-2 mg/kg/day for severe cases 1, 2
• Taper corticosteroids slowly over at least 4 weeks to prevent relapse 2
• Consider intravenous immunoglobulin (IVIG) at 1-2 g/kg in patients not responding to systemic steroids 2, 4
• Cyclosporine may be considered in severe or steroid-unresponsive cases 2

Supportive Care

• Manage fluid and electrolyte balance 2
• Minimize insensible water losses 2
• Implement infection prevention measures 2
• Provide topical treatment and oral antihistamines for symptomatic relief 2

Special Considerations

Monitoring and Follow-up

• Monitor for relapse, which can occur in approximately 12% of cases 1
• Be vigilant for development of long-term sequelae, including autoimmune conditions like diabetes 4
• Consult appropriate specialists based on organ involvement (e.g., hepatology for liver involvement) 2

Prevention

• Genetic testing may be useful before prescribing certain medications known to cause DRESS (e.g., HLA-B*5701 for abacavir) 1
• Avoid prophylactic use of corticosteroids when initiating medications known to cause DRESS 2
• Patch testing should be avoided until at least 6 months after the acute reaction 1

Common Pitfalls

• Delayed recognition of DRESS syndrome can lead to increased mortality (reported between 5-20%) 3
• DRESS must be distinguished from other severe cutaneous adverse reactions like Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 1
• Unlike in Stevens-Johnson syndrome, corticosteroids are not contraindicated in DRESS syndrome and are actually recommended 2