KAWASAKI (MUCOCUTANEOUS LYMPH NODE) SYNDROME–(KD)–1974
AN ACUTE FEBRILE MULTISYSTEM VASCULITIS!!–USUALLY IN CHILDREN LESS THAN 5 YEARS OLD!!—COMMON!
PRESUMED INFECTIOUS/TRANSMISSABLE BASED ON EPIDEMIOLOGY
ASIANS AND BLACKS MORE SUSCEPTIBLE
PRESUMED TO BE AN IMMUNOLOGIC RXN TO AN INFXOUS AGENT WHICH EVOKES AN AUTOIMMUNE RESPONSE—REFRACTORY TO ABX
SINE QUA NON = ACUTE FEBRILE PHASE LASTING > 5 D (7-10)
BILAT NONPURULENT CONJUNCTIVITIS, MUCOSAL INJECTION, STRAWBERRY TONGUE, STOMATITIS, ERYTHEMATOUS DRY FISSURED LIPS–CAN EVEN BLEED, DESQUAMATION OF THE DIGITS (PAINFUL AND SWOLLEN)
EXTREMITY SWELLING AND ERYTHEMA–REFUSAL TO WALK= MOST SPECIFIC FINDING
RASH (PERINEAL)
CERVICAL LAD (MAY LOOK LIKE ABSCESS ON CT) WITHOUT SUPPURATION—-AT LEAST 50% OF THE PTS—-USUALLY FIRM AND SOMEWHAT TENDER—NON-SUPPURATIVE/NONFLUCTUANT–INACTIVE GERMINAL CENTERS—HISTIOCYTES—PERIVASCULAR INFLAMMATION
CT–NO SIGN OF ABSCESS—-PTS HAVE MARKED NUCHAL RIDGIDITY
PROLONGED COURSE (20%)CAN LEAD TO CADz—–LEAD TO ISCHEMIC CARDIOMYOPATHY—MI —EVEN DEATH
Dx: Hx, SERIAL PE WITH CLINICAL KNOWLEDGE,  FNA REVEALS NO ORGANISMS, Bx OF THE LAD REVEALS NONSPECIFIIC VASCULITIC CHANGES——-HIGH WBC, ESR, IgE, AND PLTS(THROMBOCYTOSIS)
(90% OF THE CASES WILL BE TREATED AS A NECK ABSCESS)
Rx: ASA 80MG/KG/DAY AND IgG 400MG/KG/DAY
IgG MUST BE GIVEN WITH IN 9-10 DAYS TO AVOID THE IMMUNE MEDIATED ATTACK ON THE CORONARIES
ASA IS PRIMARILY FOR SYMPTOMATIC RELEIF—–?REYE SYNDROME (HEPATOTOXICITY IN CHILDREN < 16 DUE TO ASA—N/V, OBTUNDATION, COMA-SEIZURES–DEATH) HAS NOT BEEN MENTIONED.
STEROIDS ARE CONTRAINDICATED
MAY AT SOME TIME WANT TO GET AN ULTRASOUND OF THE CORONARIES