DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) is a severe idiosyncratic drug reaction characterized by a wide array of symptoms and a prolonged latency period. Although diagnostic criteria for the syndrome have been established, they still require validation. Additionally, management of DRESS syndrome lacks robust support from laboratory data. The syndrome typically presents with fever, rash, lymphadenopathy, eosinophilia, and various systemic manifestations ranging from mild to severe.
Symptoms usually manifest 2 to 8 weeks after the introduction of the triggering drug and commonly include fever, rash, lymphadenopathy (LAP), eosinophilia, leukocytosis, and abnormal liver function tests. DRESS syndrome carries a significant risk of mortality, with a mortality rate of approximately 10%. Among the drugs implicated in causing the syndrome, antiepileptic medications such as phenytoin and phenobarbital are considered the primary culprits, with an estimated incidence of 1 per 5,000 to 10,000 exposures.
Observations
In a 2006 study, researchers examined 216 cases of drug-induced cutaneous side effects with systemic symptoms to better understand the diverse clinical patterns of DRESS syndrome and its association with specific medications. They compared patient records with reports from the literature to identify potential DRESS syndrome-inducing drugs. They found that patients experiencing febrile skin eruptions accompanied by eosinophilia and/or systemic symptoms were often undergoing treatment with anticonvulsants, minocycline, allopurinol, abacavir, or nevirapine. This research sheds light on the variability in clinical presentations of DRESS syndrome and highlights the importance of identifying the causative medications.
Diagnosis
The diagnosis of drug rash with eosinophilia and systemic symptoms syndrome is mainly clinical. During diagnosis of the DRESS syndrome one must consider the latency period, diversity of symptoms, and exclusion of similar non-drug-induced conditions. There are several diagnostic criterias set by different groups of experts and many of them have been put to successful test. The RegiSCAR group suggested criteria for hospitalized patients with a drug rash to diagnose DRESS syndrome while a Japanese group suggested another set of diagnostic criteria, which includes HHV-6 activation.
Management
Recognition of the drug rash with eosinophilia and systemic symptoms (DRESS) syndrome as early as possible is crucial, and prompt withdrawal of the causative drug is imperative. Early discontinuation of the offending medication enhances the likelihood of a favorable prognosis. Treatment for DRESS syndrome primarily focuses on supportive and symptomatic measures, with corticosteroids commonly prescribed. However, evidence regarding the effectiveness of corticosteroids in managing DRESS syndrome remains limited.