• | 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:
• | 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 (Beau’s 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
|