section name header

General Reference ⬇

Peds 2004;114:1706; Lancet 2004;364:533

Pathophys and Cause ⬆ ⬇

Cause:Unknown

Pathophys:A vasculitis of medium-sized vessels and a mucocutaneous lymph node disease

Epidemiology ⬆ ⬇

Incidence = 5-8/100 000/yr

Signs and Symptoms ⬆ ⬇


Sx: Prolonged high fever, variety of rashes, red eyes, nodes

Si:Fever in children age 1-8 yr, nonpitting edema, cervical lymphadenopathy, and desquamation of skin esp palms, perineal area, trunk, lips, as well as nonexudative conjunctivitis, and “strawberry” tongue (pictures—Nejm 1995;333:1391)

Complications ⬆ ⬇

25% get coronary artery aneurysms later; risk factors = male, age <1 yr, fever after IVIG

r/o measles, scarlet fever, RMSF, leptospirosis, EBV, JRA

Lab and Xray ⬆ ⬇

Lab:

Hem:ESR, platelets, and wbc elevated

Treatment ⬆

Rx: IgG iv w/i 1st 10 d of fever to prevent complications, 2 gm/kg iv over 10 h × 1 (Nejm 2007;356:663; 1991;324:1633)