From the Guidelines
DRESS syndrome is a severe, potentially life-threatening condition that requires immediate discontinuation of the offending medication, supportive care, and systemic corticosteroids, with a mortality rate of approximately 10% mainly due to liver failure, as reported in the most recent study 1.
Key Characteristics of DRESS Syndrome
- Skin eruptions
- Fever
- Lymphadenopathy
- Hematologic abnormalities (especially eosinophilia)
- Internal organ involvement
Management of DRESS Syndrome
- Immediate discontinuation of the suspected drug
- Supportive care
- Systemic corticosteroids (typically prednisone 1-2 mg/kg/day, tapered slowly over 2-3 months to prevent relapse) 1
- Hospitalization for close monitoring and management of organ dysfunction in severe cases
Pathophysiology and Long-term Consequences
- Delayed hypersensitivity reaction
- Genetic predisposition
- Possible viral reactivation (particularly HHV-6)
- Long-term autoimmune sequelae following recovery
- Mortality rate of approximately 10% mainly due to liver failure 1
Recommendations for Patients with DRESS Syndrome
- Avoid the causative drug and structurally related medications permanently, as rechallenges can trigger rapid and more severe reactions
- Consider patch testing or delayed intradermal testing for diagnosis and to assess cross-reactivity, but only after at least 6 months have elapsed from the acute reaction and the patient has been off systemic corticosteroid treatment for at least 1 month 1
From the Research
Definition and Characteristics of DRESS Syndrome
- DRESS syndrome is a serious and potentially fatal adverse effect to therapeutic medications, characterized by fever, rash, and eosinophilia 2, 3, 4.
- The syndrome involves multiorgan manifestations, including hematologic, hepatic, renal, pulmonary, cardiac, neurologic, gastrointestinal, and endocrine abnormalities 3, 4.
- The incidence of DRESS syndrome varies among different ethnicities due to genetic differences 2.
Diagnosis and Diagnostic Criteria
- The European Registry of Severe Cutaneous Adverse Reactions to Drugs and Collection of Biological Samples (RegiSCAR) criteria is commonly used for diagnosis 2, 5.
- Diagnostic criteria include clinical and laboratory findings, such as fever, lymphadenopathy, hematologic abnormalities, and organ involvement 5.
- A skin biopsy and patch testing may be helpful in the diagnostic process 5.
Causes and Triggers
- Anticonvulsants, sulfonamides, and other medications are common offending agents 3, 5.
- Viral reactivation, especially of human herpes virus 6, Epstein-Barr virus, and cytomegalovirus, is a common feature of DRESS syndrome 4, 6.
- HLA allele associations and JAK/STAT activation may play a role in the disease 6.
Treatment and Management
- Early removal of the causative agent and treatment with anti-histamines, emollients, and corticosteroids are essential for management 2, 4.
- Plasmapheresis and other alternative drugs may be used in severe cases 2.
- Viral testing and antiviral therapy may be necessary in some cases 6.
- JAK inhibitors may be an effective treatment option for DRESS syndrome 6.