​3. What is the Reaction?

The body experiences a delayed, severe hypersensitivity response to a medication that may be life-threatening. This means that you have an exaggerated immune response that shows symptoms over time, becoming more severe during your illness.

 

4. What role does Eosinophilia play?

Eosinophils are a type of white blood cell involved in allergic reactions. Having lots of these cells in your blood is a helpful marker when diagnosing DRESS Syndrome but this can also indicate other medical conditions. Even though it's in the name of this condition, it’s not necessary for a diagnosis, as some people may never show eosinophilia. Atypical lymphocytes (another type of white blood cell) are also common, and may be seen with or without eosinophilia. So, finding either eosinophilia OR atypical lymphocytes is helpful when diagnosing DRESS.

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5. How is DRESS Systemic?

DRESS affects the whole body! The syndrome may affect your skin, impact multiple internal organs, create blood abnormalities, and involve your nervous system. 

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6. What are the Symptoms? 

You may start feeling symptoms days to weeks after starting a medication (common is 2 to 6 weeks after taking the drug). And you may continue to experience them after you stop taking the medicine that's causing your reaction. You may first experience a fever and rash, swollen lymph nodes and swelling, particularly of the face. Then, you may have blood abnormalities, and organ injury. DRESS most commonly affects the liver, but it can also hurt your kidneys, lungs, and heart. Your skin may eventually become flakey and peel. 

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Checklist of Common  Early DRESS Symptoms:

• Fever (38º to 40ºC)/(100º to 104ºF)

• Skin eruption/rash

• Facial swelling

• Swollen lymph nodes

• Low energy or general sense of feeling unwell

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Have Symptoms? Act Now.

If you are taking medication and develop any of the above symptoms:

1. Blame the drug.

2. Seek medical attention immediately.

3. Work with your doctors to stop taking the medication you suspect is causing your reaction.

4. Insist that doctors seriously consider and test for DRESS Syndrome. 

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Images of DRESS Syndrome

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7. How are viruses associated?

Human herpesvirus 6 (HHV-6) - among other herpes viruses like Human Herpesvirus 7 (HHV-7), Epstein Barr (EBV) and Cytomegalovirus (CMV), has been shown to play an important role in DRESS. This virus, which lays dormant in almost everyone, has been demonstrated to reactivate in over 50% of DRESS patients. Those with viral reactivation may have a more severe course of illness.

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8. How do you diagnose and treat DRESS Syndrome?

It is most important to identify and discontinue the offending drug.  However, DRESS is tricky in that it can continue to get worse even after stopping the drug.  It is imperative to diagnose DRESS quickly and follow up with proper testing.  Due to the progressive nature of the illness, certain conditions like eosinophilia and HHV-6 reactivation may not show up initially in blood test. Retesting for these at the right time could make a significant difference in diagnosis and treatment. 

 

Well-defined criteria (RegiSCAR) has been developed to aid in a scoring system for diagnosis.

Treatment varies by person and by condition, since people can have different reactions.

 

Common treatment includes steroids, IVIG, cyclosporine, and antivirals.  

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Diagnostic Tests

You'll need a variety of tests to identify if you're experiencing DRESS. Common abnormal results include:

• Atypical lymphocytes

• Elevated liver enzymes (ALT/AST)

• Eosinophilia

• Herpes virus reactivation (most common, HHV-6) 

• Leukocytosis

• Kidney function (blood urea nitrogen (BUN), creatinine)

• Viral test: (HHV-6, HHV-7, EBV, CMV)

• Skin Biopsy

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10. What complications can result from DRESS?

Some people may receive prompt diagnosis and treatment, and avoid some of the most severe symptoms. However, a crucial characteristic about DRESS Syndrome is that seemingly successful, initial treatment doesn’t always mean lasting recovery.

• Serious flares during steroid taper

• Myocarditis

• Organ Injury: Liver, Kidney and Lung

• Autoimmune disorders: Type 1 diabetes and thyroiditis

• Recurrent viral reactivations

• A flare in symptoms caused by another medication (can be related or unrelated to original offending medication).

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Continue Watching Your Symptoms After Treatment

Be sure to watch your symptoms for months after starting your initial treatment. If you notice symptoms coming back or getting worse, go to your doctor immediately. Document every reaction that you’re having. 

 

Be diligent — every moment counts and could save your life!