CLINICAL PRESENTATION (How a Patient Usually Looks and Feels)

 

Early stages of reaction

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• Fever (38 to 40C/ 100 to 104F)

• Malaise (weakness and fatigue)

• Rash**: the skin eruption often starts as morbilliform/maculopapular (flat and red and wide-spread) and progresses quickly to be more confluent, appearing to merge together. Typically the upper trunk and face are initially affected.

• Enlarged lymph nodes

 

*It’s important to note that all symptoms are not always present.
**While morbilliform rash is the most common rash type in DRESS patients, some may present with erythema multiform-like (pink or red blotches) or a pustular type of rash.

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Progressive stages of reaction

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• Continued and worsening fever

• Progressive rash: may eventually progress to lower extremities and will eventually cover more than 50% of body surface area. Later, exfoliative-dermatitis (peeling of skin) occurs.

• Facial edema (swelling): occurs in at least half of patients. It begins as mild and can progress to be severe, rendering the patient almost unrecognizable.

• Mucus membranes: Inflammation may occur in half of cases causing pain in the mouth or larynx, making it difficult to speak and eat or drink.

• Enlarged lymph nodes

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SYSTEMIC SYMPTOMS

 

Unlike the symptoms listed above,what’s happening internally with DRESS is not evident until the patient seeks medical help and diagnostic tests are run. DRESS usually involves one or more organ and hematologic (blood) abnormalities. Because this syndrome exists on a continuum, some, but not all, of these symptoms may appear through the course of illness. Symptoms may continue to deteriorate and flare-ups may occur for weeks and even months after stopping the offending medication.

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Organs

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Liver: The most common organ affected in DRESS. Most fatalities are due to liver failure. Patient may appear jaundice (yellowing of the skin and white of the eyes).

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Kidney: Acute interstitial nephritis (kidney inflammation). May involve decreased urine output, blood in urine.

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Lung: May present with mild cough or dyspnea (difficult/labored breathing).

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Heart: DRESS-related myocarditis is an often under-recognized but potentially fatal. It may occur later in the course of illness and present with: dyspnea (difficulty/labored breathing), tachycardia (abnormal, increased heart rate), hypotension (abnormally low blood pressure) and chest pain.

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It’s important to note that not all patients will have chest pain, and that myocarditis can occur immediately AND months after the first appearance of fever and rash. Caution needs to be taken as myocarditis may occur when corticosteroid treatment is too rapidly reduced. Of the two forms; “hypersensitivity myocarditis” is often self limiting and responsive to therapy, and “acute necrotizing eosinophilic myocarditis (ANEM),” can be highly fatal (up to 50%).

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Other Organs: A number of other organs may be affected by DRESS as well...

• Gastrointestinal Tract: vomiting, diarrhea, bleeding

• Pancreas: pancreatitis

• Gallbladder

• Spleen

• Thyroid: autoimmune thyroiditis (usually later in the course of DRESS)

• Brain: encephalitis, meningitis

• Peripheral Nerves

• Eyes

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LAB ABNORMALITIES

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Eosinophilia

• May be detected in blood and organs. It is transient during the course of illness and may be undetectable up to 50% of the time.

 

Leukocytosis

 

Atypical lymphocytes

 

Other labs associated with Hemophacogytic Syndrome  

• Elevated triglycerides 

• Elevated ferritin

• Elevated CD25 (sIL-2 receptor)

 

Elevated liver enzymes

• Alanine Aminotransferase (ALT) 

• Aspartate Aminotransferase (AST)

 

Viral Reactivation

• Human herpes virus 6 (HHV-6)

• Human herpes virus 7 (HHV-7)

• Cytomegalovirus (CMV) and

• Epstein-Barr virus (EBV)

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DIAGNOSING DRESS >

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