D72.12 Drug rash with eosinophilia and systemic symptoms syndrome

Name of the Condition

• Common Name: Drug rash with eosinophilia and systemic symptoms syndrome
• Medical Terms: Drug-induced hypersensitivity syndrome, DRESS syndrome

Summary

Drug rash with eosinophilia and systemic symptoms syndrome (DRESS) is a severe, drug-induced hypersensitivity reaction characterized by a widespread rash, elevated eosinophils, and systemic involvement of organs such as the liver, kidneys, or lungs. It typically develops 2–6 weeks after starting a medication and requires prompt recognition and discontinuation of the causative drug.

Causes

DRESS is triggered by an adverse immune response to certain medications, most commonly anticonvulsants, allopurinol, sulfonamides, or minocycline. The reaction may involve reactivation of latent viruses (e.g., herpesviruses) and dysregulated immune activation, though the exact mechanism is not fully understood.

Risk Factors

• Prior exposure to the causative drug
• Genetic predisposition (e.g., HLA associations)
• Pre-existing viral infections (e.g., EBV, CMV)
• History of drug allergies or hypersensitivity reactions

Symptoms

• Diffuse maculopapular rash, often progressing to exfoliative dermatitis
• Fever and malaise
• Lymphadenopathy (swollen lymph nodes)
• Organ involvement (e.g., hepatitis, nephritis, pneumonitis)
• Eosinophilia (elevated eosinophil count in blood)

Diagnosis

Diagnosis is clinical, supported by laboratory findings (eosinophilia, elevated liver enzymes) and temporal association with a suspected drug. Other causes of eosinophilia or rash must be excluded. Skin biopsy may show eosinophilic infiltration, and viral reactivation tests may be considered.

Treatment Options

• Immediate discontinuation of the suspected drug
• Supportive care (hydration, antipyretics)
• Corticosteroids (e.g., prednisone) for severe cases
• Antiviral therapy if viral reactivation is present
• Monitoring of organ function (liver, kidney) during recovery

Prognosis and Follow-Up

Most patients recover fully with drug withdrawal and treatment, but recovery may take weeks to months. Long-term follow-up is recommended to monitor for organ damage or recurrence. Mortality is rare but can occur with severe organ involvement.

Complications

• Severe hepatitis or liver failure
• Acute kidney injury
• Interstitial pneumonitis or respiratory failure
• Chronic organ dysfunction (e.g., thyroiditis)
• Rarely, progression to Stevens-Johnson syndrome or toxic epidermal necrolysis

Lifestyle & Prevention

• Avoid re-exposure to the causative drug and related compounds
• Inform healthcare providers of drug allergies
• Use alternative medications when possible
• Monitor for early signs of rash or systemic symptoms after starting new drugs

When to Seek Professional Help

Seek immediate medical attention if you develop a rash with fever, swollen lymph nodes, or organ-related symptoms (e.g., jaundice, shortness of breath) after starting a new medication.

Tips for Medical Coders

Document the causative drug, timing of symptom onset, and evidence of systemic involvement (e.g., lab results, organ dysfunction) to support the DRESS diagnosis. Ensure the code aligns with clinical documentation of drug exposure and hypersensitivity features.