Makindo Medical Notes

Related Subjects: Asthma |Acute Severe Asthma |Eosinophilic granulomatosis (Churg Strauss) |Loffler's syndrome (Pulmonary Eosinophilia) |Pulmonary Eosinophilia and CXR changes |Drug Reaction Eosinophilia Systemic Symptoms

The life-threatening potential of DRESS syndrome is high and the mortality is estimated to be around 10% in multiple studies. Causes skin rash, fever, lymphadenopathy, and involvement of internal organs such as the liver, kidneys, and lungs.

About

• Severe idiosyncratic drug reaction with a long latency period.
• Also known as anticonvulsant hypersensitivity syndrome
• It is a severe potentially life-threatening due to reaction to certain medications.

Aetiology

• Aromatic anticonvulsive drugs (carbamazepine, lamotrigine, phenobarbital)
• Drugs: minocycline, Allopurinol, abacavir, or nevirapine.
• Usually 2- to 6-week latency period for carbamazepine. Allopurinol.

Drugs: may take several weeks

• Carbamazepine
• Phenytoin
• Lamotrigine
• Valproic Acid
• Sulfonamides (e.g., Sulfamethoxazole)
• Allopurinol
• Minocycline
• Abacavir
• Nevirapine
• Vancomycin
• Clozapine
• Dapsone
• Doxycycline
• Rituximab
• Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

Clinical: Potentially fatal

• Clinical symptoms, anywhere from 2 to 8 weeks after initiating the offending drug
• Fever, rash, urticarial, maculopapular eruption
• Vesicles, bullae, pustules, purpura, target lesions
• Facial oedema, cheilitis, and erythroderma
• SJS/TEN, erythema multiforme
• Hepatitis, pneumonitis, myocarditis, pericarditis, nephritis, and colitis

Investigations

• Eosinophilia, leukocytosis, and abnormal liver function tests
• Exclusion of other potential causes such as viral infections or autoimmune diseases.
• RegiSCAR (Registry of Severe Cutaneous Adverse Reactions) criteria are often used to aid in diagnosis.

Management

• Drug withdrawal, the earlier the better the prognosis.
• Supportive and symptomatic
• Corticosteroids such as Prednisolone often used, but the evidence is scant
• Ciclosporin may also be required as immunosuppressant

Prevention

• Genetic screening for high-risk HLA alleles, especially before initiating medications known to cause DRESS syndrome.
• Awareness and education of healthcare providers and patients about the potential severe reactions associated with certain drugs.
• Careful monitoring of patients starting on high-risk medications, particularly in the first few weeks of therapy.
• Avoidance of re-exposure to the offending drug and related compounds in the future.

Prognosis

• The prognosis of DRESS syndrome varies; with prompt recognition and appropriate treatment, most patients recover fully.
• However, delayed diagnosis and treatment can lead to severe complications and increased mortality rates.
• Long-term sequelae may include chronic kidney disease, thyroid dysfunction, and other persistent organ impairments.

Conclusion

• DRESS syndrome is a serious adverse drug reaction that requires immediate medical attention.
• Early identification and cessation of the causative agent are essential for favorable outcomes.
• Prevention strategies, including genetic screening and vigilant monitoring, play a vital role in reducing the incidence of DRESS syndrome.

References

• Drug reaction with eosinophilia and systemic symptoms (DRESS)