By Disease Name > Kawasaki Disease

Kawasaki Disease

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AKA mucocutaneous lymph node syndrome
an acute multi-system vasculitis of unknown etiology that is associated with marked activation of T-cells and monocyte/macrophages
young children 2-6 years old

 

clinically defined as fever of 5 days duration plus 4 of 5 other criteria:

1.polymorphous exanthem
most commonly urticarial and diffuse deep red, maculopapular eruption
dermatitis in the diaper area (perineum) is common, and followed by desquamation
2.optho - bilateral conjunctivitis an almost constant feature (but no discharge vs. SJS)
3.oral - strawberry tongue” and “cherry red” lips with fissuring and crusting (though no sore throat vs. Scarlet fever)
4.acral - hands and feet red and edematous (non-pitting edema and tenderness may limit walking);  peeling similar to that seen in scarlet fever (Beaus lines also appear in nails weeks later)
5.lymph node cervical LAN, often a single node

 

 

labs:

fever,  leukocytosis with a shift to the left (vs. mono with lymphocytosis)
acute phase reactants ESR, c-reactive protein, serum alpha1-antitrypsin
(left shift and acute phase makes sense because a vasculitc disease ??? i.e. neuts and immune complexes???)
thrombocytosis a distinctive sign of this disease;  peaks at 600,000 to 1.5 million platelets during the convalescent phase (2 to 3 weeks after onset of illness)

 

likewise coronary artery aneurysms usually manifest after approximately 2 weeks of illness (histopathology necrotizing vasculitis especially of medium sized arteries such as coronary arteries; these pathologic findings are usually NOT evident on skin biopsy)

 

prognosis:

although self-limited, coronary artery aneurysms occur in up to 20% of patients in convalescent phase
baseline echo and repeat in ~3 weeks and 8 weeks after disease onset
myocardial infarction is the primary cause of death in KD, occuring in ~2% of patients within the first year after the acute disease

 

 

treatment high dose ASA asa 3 to 5 mg/kg QD PO, IV gamma globulin 2g/kg single infusion

 

etiology - evidence that KD is toxin-mediated, super antigen driven

 

ddx - Scarlet fever