KAWASAKI DISEASE - acute febrile mucocutaneous syndrome

                          Dr Tomisaku Kawasaki published a case series of 50 children in 1967, who were febrile and all had a rash, non-exudative conjunctivitis, erythema of the palms and soles of the feet, and cervical lymphadenopathy. This constellation of signs Dr Kawasaki termed “acute febrile mucocutaneous syndrome”; however the eponym Kawasaki disease has been accepted worldwide. 

Symptoms

Kawasaki disease often begins with a high and persistent fever greater than 102 °F, often as high as 104 °F. A persistent fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually go away with normal doses of acetaminophen (Tylenol) or ibuprofen.
Other symptoms often include:

• Extremely bloodshot or red eyes (without pus or drainage)
• Bright red, chapped, or cracked lips
• Red mucous membranes in the mouth
• Strawberry tongue, white coating on the tongue, or prominent red bumps on the back of the tongue
• Red palms of the hands and the soles of the feet
• Swollen hands and feet
• Skin rashes on the middle of the body, NOT blister-like
• Peeling skin in the genital area, hands, and feet (especially around the nails, palms, and soles)
• Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
• Joint pain and swelling, frequently on both sides of the body

Additional symptoms may include:

• Irritability
• Diarrhea, vomiting, and abdominal pain
• Cough and runny nose

DIAGNOSIS: 

Kawasaki disease is a systemic vasculitis predominantly affecting children under the age of 5 years. It has a number of classic clinical features required for diagnosis. 

     

In 1990 the American Heart Association committee on rheumatic fever, endocarditis, and Kawasaki disease gave the case definition that has been generally accepted—ie, a febrile illness of at least five days with at least four of the five following signs and no other reasonable cause for the findings:

• Bilateral conjunctival injection – (there is no corneal ulceration but there may be a concomitant anterior uveitis on slit lamp examination)
• Oral changes (erythema of lips or oropharynx, strawberry tongue due to prominent papillae, or fissuring of the lips) 
  
  
  
• Peripheral extremity changes (oedema, erythema, or generalised or periungal desquamation); erythema is seen in the first week whereas desquamation begins about 14–21 days after the onset of the illness 
  
  
  
  
  
• Rash – this starts in the first few days; it is often diffuse and polymorphic and lasts a week before fading. Vesicles are rarely seen; however, the rash can appear macular, maculopapular, urticarial, scarlettina or even morbilliform
• Cervical lymphadenopathy is found in about 50% of cases; most often there is a painful solitary enlarged lymph gland, > 1.5 cm in diameter 
  
  
                         Fever is an essential feature; it is most often sudden in onset and swinging, going above 40°C. It must last at least for five days but can persist for up to a month. If coronary arterial aneurysms (CAA) are present, one of the most important complications of Kawasaki disease, then only three of the clinical features are required to clinch the diagnosis. 
  
  
  
  
  
  

  DIFFERENTIAL DIAGNOSIS

  The differential diagnoses of Kawasaki disease include:
  
  
• Streptococcal infection (including scarlet fever, toxic shock-like syndrome)
• Staphylococcal infection (such as toxic shock syndrome or scalded skin syndrome)
• Measles, rubella, roseola infantum, Epstein Barr virus, influenza A and B, adenovirus
• Mycoplasma pneumoniae
• Stevens-Johnson syndrome
• Systemic idiopathic juvenile arthritis

One of the difficulties of securing the diagnosis is that the clinical features of Kawasaki disease may appear sequentially rather than at the same time, and the feature most commonly identified is desquamation, which occurs late in the disease when cardiac complications may have occurred. 

Results of laboratory investigations

There may be abnormal laboratory findings such as raised white cell count with neutrophilia (> 20 × 10⁶/ml) changing to a lymphocytosis at the end of the first week.

By day 14 there may be a hypochromic anaemia and thrombocytosis (> 1000 × 10⁹/ml).

Erythrocyte sedimentation rate is usually raised and C reactive protein and sterile pyuria and proteinuria can be detected. None of these tests is pathognomic of Kawasaki disease. 

Complications of Kawasaki disease

• Irritability and aseptic meningitis
• Gallbladder hydrops
• Diarrhoea
• Hepatitis
• Otitis media
• Pancreatitis
• Myositis
• Pericarditis and myocarditis
• Aneurysm formation can lead to peripheral gangrene, cerebral infarction and cardiac arterial aneurysm (this may lead to thrombosis, myocardial infarction and dysrhythmia) 
  
  
  
  
  
                       Morbilliform eruption in a 2 year old boy with Kawasaki Disease.

• Treatment

  Children with Kawasaki disease are admitted to the hospital. Treatment must be started as soon as the diagnosis is made to prevent damage to the coronary arteries and heart.
  Intravenous gamma globulin is the standard treatment. It is given in high doses. The child's condition usually greatly improves within 24 hours of treatment with IV gamma globulin.
  High-dose aspirin is often given along with IV gamma globulin.
  Even when they're treated with aspirin and IV gamma globulin, up to 25% of children may still develop problems in their coronary arteries. Some research has suggested that adding steroids to the usual treatment routine may improve a child's outcome, but more research is needed.