Makindo Medical Notes.com

Approximately 20% to 25% of children with KD who are not treated with intravenously administered immune globulin (IVIG) develop coronary artery abnormalities. There is some thought that Kawasaki disease is due to a response to a superantigen toxin possibly of Staph. aureus or Strep. pyogenes

About

• Kawasaki disease is a rare self-limiting vasculitis of the coronary arteries
• It often goes undiagnosed especially with atypical variants
• Suggested possible relationship was with Rickettsial disease

Aetiology

• Untreated it can lead to the development of coronary artery aneurysms
• This can lead to occlusion and cardiac ischaemia
• It is potentially lethal. 80% of children affected are under 5 years old.

Clinical

• Peak age of onset is at 18 months old
• It causes a fever usually longer than the 5 days of other childhood viral illnesses.
• Lymphadenopathy is a common feature and is usually cervical and unilateral
• Peripheral oedema and skin peeling is a feature - desquamation of skin
• Strawberry tongue and a nonspecific Truncal rash

Investigations

• Raised CRP/ESR and platelets

Diagnostic : 1 Required Criteria and 4 Additional Criteria

• Required Criteria
  • Fever for at least five days generally high and spiking (often to 40°C or more), persisting for one to two weeks or longer in untreated patients.
• Additional Criteria
  • Changes in arms or legs: redness, swelling and induration of the hands and feet. One to three weeks after the onset of fever, desquamation of the fingers and toes may occur. Beau's lines (white lines across the fingernails) may appear one to two months after an acute febrile illness.
  • Polymorphic exanthem involving the trunk and extremities. Forms include urticarial exanthem, morbilliform maculopapular eruption, target lesions, and a diffuse scarlatiniform rash. Rash usually appears within five days of the onset of fever.
  • Painless, non-exudative bilateral bulbar conjunctival injection
  • Strawberry tongue, redness and cracking of the lips, and erythema of the oropharyngeal mucosa. No mouth ulcers.
  • Cervical lymphadenopathy: often unilateral, slightly tender, firm nodes.
  • Rule out other diseases with similar finding.

Management

• IV gamma globulin is effective. A dramatic response may help confirm the diagnosis
• Steroids avoided as they increase coronary aneurysms
• Aspirin is also given

References

• Dajani AS, Taubert KA, Gerber MA, Shulman ST, Ferrieri P, Freed M, et al. Diagnosis and therapy of Kawasaki disease in children. Circulation. 1993;87:1776-80.