What Is DRESS Syndrome? Know Everything About the Hypersensitivity Reaction

Drug Rash with Eosinophilia and Systemic Symptoms Syndrome (DRESS) Syndrome is a rare but serious reaction to medication that can affect several organs in the body. It is characterised by a widespread skin rash, fever, and other systemic symptoms.

The National Centre for Biotechnology Information mentioned: “Drug rash with eosinophilia and systemic symptoms syndrome is a severe idiosyncratic drug reaction with a long latency period. It has been described using many terms; however, drug rash with eosinophilia and systemic symptoms syndrome appears to be the most appropriate.” 

The Government of India's Indian Pharmacopoeia Commission (IPC) has issued a public safety alert regarding the potential risk of developing DRESS Syndrome associated with the use of the painkiller Meftal. 

The recent safety alert concerning Meftal underscores the importance of reporting any adverse reactions you may experience while taking this medication. By actively contributing to the Pharmacovigilance Programme of India (PvPI), you play a vital role in ensuring the safety and well-being of yourself and others.

What are the Symptoms of the DRESS Syndrome? 

DRESS Syndrome  is characterised by a combination of symptoms that typically develop 2-8 weeks after starting the medication. Here are the key symptoms of DRESS syndrome:

Core Symptoms:

• Skin rash: This is the most common symptom, often starting on the face and upper body and spreading to other areas. The rash can be red, itchy, and scaly. It can also be morbilliform (resembling measles), urticarial (hives), or maculopapular (flat red spots).
• Fever: This is typically a high fever, often above 38 degrees Celsius (100.4 degrees Fahrenheit).
• Enlarged lymph nodes: This occurs in two or more areas of the body, such as the neck, armpits, or groin.
• Eosinophilia: This is an increase in the number of eosinophils, a type of white blood cell.

Additional Symptoms:

• Facial swelling
• Fatigue and malaise
• Atypical lymphocytosis (increased number of lymphocytes with an abnormal appearance)
• Inflammation of internal organs, such as the liver, kidneys, lungs, and heart
• Haematological abnormalities, such as anaemia or thrombocytopenia
• Sore throat and mouth ulcers
• Conjunctivitis (inflammation of the eye)
• Jaundice (yellowing of the skin and whites of the eyes)
• Myalgia (muscle pain)
• Arthralgia (joint pain)

It's important to note that not everyone will experience all of these symptoms. Some people may only have a few, while others may have many. 

If you are experiencing any of these symptoms after starting a new medication, it's important to see a doctor right away. Early diagnosis and treatment are essential to prevent severe complications.

What are the Risk factors for DRESS syndrome:

These are the different causes behind the DRESS Syndrome according to New Zealand Medicines and Medical Devices Safety Authority:

Medications:

Anticonvulsants (e.g., carbamazepine, phenytoin)

Antibiotics (particularly beta-lactams, e.g., amoxicillin, penicillin)

Allopurinol

Others:

Non-steroidal anti-inflammatory drugs (NSAIDs)

Captopril

Mood stabilisers

Antiretrovirals

Specific risks for certain medications:

Anticonvulsants: 1 in 1,000 to 1 in 10,000 exposed individuals

Allopurinol: Increased risk with renal impairment and concomitant use of thiazide diuretics. This is likely due to increased concentrations of the allopurinol metabolite oxypurinol.

Genetic factors:

Individuals with a first-degree relative who has experienced DRESS syndrome have a significantly increased risk, as high as 25%.

What Is the Treatment of DRESS Syndrome? 

Early identification and removal of the offending medication are critical for a positive outcome in DRESS syndrome. Studies suggest that the sooner the medication is stopped, the better the prognosis. This underscores the importance of quick recognition and decisive action.

Treatment primarily focuses on managing symptoms and supporting the body's natural healing process. Corticosteroids are commonly used for this purpose, but the evidence supporting their effectiveness remains limited. In more severe cases, additional immunosuppressants like cyclosporin may be necessary.

In summary, prompt diagnosis and withdrawal of the causative drug are paramount for managing DRESS syndrome effectively. While corticosteroids are often used for symptom management, further research is needed to solidify their role in treatment. For more severe cases, additional immunosuppressant medications may be required.