Dress Syndrome: What You need to know?

dress syndrome

DRESS stands for “Drug Rash with Eosinophilia and Systemic Symptoms”. It is a rare but serious condition that can affect anyone taking certain medications. It is a type of allergic reaction that causes skin rashes, fever, blood abnormalities, and swelling of various organs. If not treated promptly and properly it can be life-threatening.

What is DRESS syndrome?

DRESS syndrome is a delayed hypersensitivity reaction to some medications that occurs within 2 to 8 weeks of starting the medication but it can also happen later.

It is thought to be triggered by an abnormal immune response to the drug, causing widespread inflammation and tissue damage.

Symptoms of Dress Syndrome

The most common symptoms of DRESS syndrome are:

  • If you have a high temperature of more than 100.4℉ (38℃) that lasts for several days or weeks.
  • If you have widespread redness of the skin that may be itchy, swollen, blistered, or peeling.
  • If you have Enlarged lymph nodes in at least two different areas of the body.
  • If you have Inflammation or damage to one or more organs such as the liver, kidneys, lungs, heart, or thyroid.
  • If you have an increase in white blood cells called eosinophils or atypical lymphocytes.

As the condition progresses, other symptoms may appear depending on the organs affected. These may include:

  • Jaundice (yellowing of the skin and eyes) due to liver damage.
  • Dark urine and reduced urine output due to kidney damage.
  • Shortness of breath, coughing, wheezing, or chest pain due to lung damage.
  • Palpitations, irregular heartbeat, or low blood pressure due to heart damage.
  • Abdominal pain, nausea, vomiting, or diarrhea due to pancreas damage.

DRESS syndrome is a rare condition that affects about one in 10,000 to one in 100,000 people who take certain drugs.

The mortality rate of DRESS syndrome is estimated to be between 5% and 10%. The most common causes of death are liver failure, infection, or multi-organ failure.

symptoms of dress syndrome

Which drugs cause DRESS Syndrome?

Some medications can trigger DRESS syndrome. It alters the metabolism of certain enzymes in the body, leading to the accumulation of toxic metabolites that cause inflammation and tissue damage. Other medications may directly or indirectly activate the immune system, causing it to attack the body’s cells and organs.

Many medications have been reported as the cause but some are more common than others. According to the DRESS Syndrome Foundation, the most frequently reported medications are:

  1. Antiepileptics (medicines used to treat seizures), such as carbamazepine, lamotrigine, phenytoin, and phenobarbital.
  2. Allopurinol (a medicine used to treat gout and high uric acid levels).
  3. Antibiotics (medicines used to treat infections), such as sulfonamides (eg, sulfasalazine, trimethoprim/sulfamethoxazole), vancomycin, minocycline, dapsone, and penicillin.
  4. Antiretrovirals (medicines used to treat HIV infection), such as abacavir, nevirapine, and efavirenz.

Other drugs that have been associated with it include:

  • Anti-inflammatory drugs (e.g., ibuprofen, naproxen),
  • Antipsychotics (e.g., clozapine, olanzapine),
  • Antidepressants (e.g., fluoxetine, sertraline),
  • Antithyroid drugs (e.g., methimazole, propylthiouracil) and,
  • Antidiabetic drugs (eg, sulfonylureas).

The exact mechanism of how these medications cause is not completely understood but may include:

  1. Formation of reactive metabolites that bind to proteins and generate immune responses.
  2. Activation of T-cells that recognize the drug or its metabolites as foreign antigens and produce inflammatory cytokines.
  3. Induction of autoimmunity by exposing or mimicking latent self-antigens.

However, not everyone who takes these drugs will develop DRESS syndrome. It depends on individual factors such as genetics, age, gender, ethnicity, and medical history.

Some Risk Factors

It can affect anyone who takes certain medications, but certain factors may increase the risk, such as:

1. Age: DRESS syndrome is more common in children and older adults.

2. Genetics: Some people may have a genetic predisposition to developing DRESS syndrome.

3. Viral infections: Some viral infections, such as human herpesvirus 6 (HHV-6) can reactivate or worsen DRESS syndrome.

DRESS Syndrome Diagnoses

DRESS syndrome can be difficult to diagnose because it has variable clinical features and no specific diagnostic test.

Some tests may be done to support the diagnosis and assess the organ involvement. These include:

1. Blood tests.

To check the levels of eosinophils, atypical lymphocytes, liver enzymes, kidney function, and thyroid hormones.

2. Skin biopsy.

To examine a sample of skin under a microscope for signs of inflammation and drug reaction.

3. Viral tests.

To detect possible viral infections such as herpes simplex virus 6 (HHV-6), Epstein-Barr virus (EBV), or cytomegalovirus (CMV).

4. Imaging tests.

To look for abnormalities in the organs such as ultrasound, CT scan, or MRI.

Some diagnostic criteria have been proposed to help identify it, such as:

1. RegisCAR criteria.

These criteria state that at least three of the following should be present to consider a DRESS syndrome diagnosis. These include:

  • If you have drug exposure within 3 months before the onset of symptoms.
    A fever greater than 100.4℉ (38℃).
  • Rash affecting more than 50% of the body surface area.
  • Eosinophilia above 1.5 x 10^9/L.
  • Lymphadenopathy in two or more locations and,
  • Resolution of symptoms after stopping the drug.

2. Japanese Consensus Group Criteria.

These include:

  • If you have exposure to medication within 8 weeks before the onset of symptoms.
  • If you have a fever above 38°C.
  • Rash affecting more than 10% of the body surface area.
  • Eosinophilia above 1.5 x 10^9/L.
  • Atypical lymphocytosis above 5%.
  • Involvement of at least two internal organs; and
  • Resolution of symptoms after stopping the drug.

How long does it take to recover?

The good news is that most people with dress syndrome recover within 6-9 weeks after diagnosis and treatment. The recovery time may vary depending on the severity of the reaction, the type of drug involved, and the individual’s response to treatment.

Some people may need a longer course of steroids or other immunosuppressants to control inflammation and prevent organ damage. Some people may also experience relapses or complications, such as infection, liver failure, or autoimmune disease.

Treatment for Dress Syndrome

Step 1:

The first and most important step in treating DRESS syndrome is to discontinue the medication that caused it. This may be enough to resolve the situation in some cases.

This can be challenging because sometimes it is not clear which drug is responsible. Your doctor will review your medication list and try to identify the most likely culprit. They may also do a skin test or blood test to check for specific antibodies against the drug.

Step 2:

The second step in treating DRESS syndrome is to control the inflammation and prevent further damage to your organs. This usually involves taking corticosteroids (such as prednisone) for several weeks or months. Corticosteroids are powerful anti-inflammatory medications that can reduce the symptoms and complications of DRESS syndrome.

Step 3:

The third step in treating DRESS syndrome is to monitor your condition closely and treat any complications that arise. You may need to stay in the hospital for some time until your condition stabilizes. You may also need to have regular blood tests and imaging tests (such as ultrasound or CT scans) to check your organ function and look for signs of infection or other problems.

However, when stopping the medication does not resolve the condition, you may need additional treatment for your symptoms.

Some helpful treatments aimed at reducing the symptoms of DRESS syndrome include:

  • Topical steroids: To reduce skin inflammation and itching.
  • Emollients: To moisturize and protect the skin.
  • Antihistamine: To relieve itching and allergic reactions.
  • Antibiotics: To prevent or treat secondary bacterial infections of the skin or organs.

If you suspect that you or someone you know has DRESS syndrome, seek medical help immediately.

If you suspect that you have dress syndrome or have any questions about it, please consult your doctor immediately. Your doctor can help you diagnose the condition, identify the culprit drug, and prescribe appropriate treatment. Your doctor can also monitor your recovery and contact you regularly.

1 thought on “Dress Syndrome: What You need to know?”

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