From the Guidelines
DRESS syndrome is a severe drug-induced hypersensitivity reaction that typically appears 2-8 weeks after starting a medication, characterized by fever, skin rash, facial swelling, enlarged lymph nodes, and internal organ involvement, and requires immediate discontinuation of the suspected medication and supportive care, including systemic corticosteroids, to prevent relapse and reduce mortality. The syndrome is often caused by common culprit drugs such as anticonvulsants (carbamazepine, phenytoin), allopurinol, sulfonamides, and certain antibiotics 1. The presentation of DRESS syndrome includes:
- Fever
- Skin rash (often starting on the face and upper body)
- Facial swelling
- Enlarged lymph nodes
- Internal organ involvement (liver, kidneys, lungs, or heart) Blood tests typically show elevated eosinophils and liver enzymes. Management of DRESS syndrome requires:
- Immediate discontinuation of the suspected medication
- Supportive care
- Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) for 6-8 weeks with a slow taper to prevent relapse Severe cases may require hospitalization and specialty consultation. The mortality rate is approximately 10%, primarily due to liver failure or other organ damage 1. Patients who develop DRESS syndrome should avoid the causative drug and potentially cross-reactive medications for life, and should inform all healthcare providers about this reaction. According to the most recent study 1, the sensitivity of patch testing for DRESS is >50% for many drugs, but testing should be avoided until >6 months have elapsed from the acute reaction and/or the patient has been off systemic corticosteroid treatment for >1 month.
From the Research
Definition and Characteristics of DRESS Syndrome
- DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) syndrome is a rare, potentially life-threatening, delayed, drug-induced hypersensitivity reaction 2, 3, 4, 5, 6.
- It is characterized by a broad spectrum of presentation, including rash, fever, lymphadenopathy, atypical lymphocytes, and hepatic involvement 2, 3, 4, 5.
Clinical Findings and Treatment
- The most frequent offending drugs include dapsone, vancomycin, carbamazepine, and aromatic antiepileptic agents 2, 3.
- Immediate withdrawal of the culprit drug and administration of systemic corticosteroids is the most widely accepted treatment 2, 3, 5.
- Additional treatment options for severe or steroid-resistant cases include intravenous immunoglobulins (IVIGs) 2, 3, cyclophosphamide, cyclosporine, and N-acetylcysteine 5, 6.
Management and Prognosis
- Prompt recognition and management of DRESS syndrome are crucial to prevent severe morbidity and mortality 5.
- The addition of IVIG or other immunosuppressants may be helpful in hastening recovery in cases refractory to systemic steroid treatment 2, 3, 6.
- Comparative studies using a placebo group are needed to further evaluate the efficacy of these treatments 2.