printed by INCOMPLETE KAWASAKI DISEASE: a case study Reese Graves, MD and Sally P. Weaver, PhD, MD McLennan County Medical Education and Research Foundation, Family Medicine Residency Program, Waco, Texas M.H. is a 5 year old African American female who presented with 4 days of fever to 102 F, dry lips and a pruritic, pustular rash covering her entire body starting the day prior to presentation. No prior medical problems or hospitalizations. Taking Rondec and Tylenol prior to the rash. Benadryl added for itching once rash appeared. INCOMPLETE KAWASAKI DISEASE - -Suspect IKD in children with fever for 5 days and at least two to three of the associated clinical findings. - - KD is extremely unlikely in patients with normal ESR/CRP levels and normal platelet counts by the seventh day of fever. - - Complication rates are similar between IKD and KD, necessitating a high incidence of suspicion. KAWASAKI DISEASE (KD) -An uncommon, self-limited vasculitis resulting in coronary artery aneurysms in 20-25% of untreated children. -Diagnostic criteria: - Fever for 5 days PLUS four of the following: 1) bilateral conjunctival injection 2) changes in the mucous membranes of the oropharynx 3) a polymorphous rash 4) cervical lymphadenopathy 5) changes of the extremities: (peripheral edema, peripheral erythema, periungual desquamation) -Associated laboratory findings: -Leukocytosis-Elevated ESR/CRP -Thrombocytosis-Hypoalbuminemia -Sterile pyuria-Elevated transaminases INCOMPLETE KAWASAKI DISEASE - Incomplete Kawasaki disease (IKD) is diagnosed when evidence of Kawasaki disease (KD) is present, though fewer than 4 classical clinical criteria are present. - IKD represents approximately 15% of KD cases and has a similar rate of coronary artery abnormalities. - The least common clinical signs in IKD are rash, conjunctivitis and lymphadenopathy (two of which our patient manifested). - Evidence suggests that IKD responds as well as KD to standard therapy and does not represent a separate disease but rather incomplete penetrance of KD. - -Fimbres, Angela M., Shulman, Stanford T. Kawasaki Disease. Pediatrics in Review. 2008;29: Hsieh YC, Wu MH, Wang JK, et al. Clinical features of atypical Kawasaki disease. J Microbiol Immunol Infect. 2002;35(1): Newburger, J., Takahashi, M., Gerber, M., et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association Pediatrics. 2004;114: Sonobe T, Kiyosawa N, Tsuchiya K, et al. Prevalence of coronary artery abnormality in incomplete Kawasaki disease. Pediatr Int. 2007;49(4): EXAM: T 102 F, erythematous “strawberry tongue”, cervical lymphadenopathy, micropustular rash with desquamation involving genitalia, scalp, and the extensor surface of both arms and legs, and tachycardia with an otherwise normal cardiac exam. LAB: WBC 19K, Plt 320K, Albumin 3.0, ESR 32 Urinalysis showed moderate leukocyte esterase but culture was negative. CXR normal, influenza negative, and blood cultures were all negative. HISTORY EXAM AND LABORATORY EVALUATION DIAGNOSIS AND DISCUSSION BIBLIOGRAPHY M.H. remained febrile on the day after admission. Normal echocardiogram on hospital day 1. IVIG was given hospital day 2 due to persistent fever. Over next 2 days, fever abated and rash improved. M.H. was discharged with two weeks of high dose aspirin followed by six weeks of low dose aspirin. A repeat echocardiogram was recommended in one week and six weeks following discharge. HOSPITAL COURSE PROPOSED ALGORITHM FOR DIAGNOSING INCOMPLETE KAWASAKI DISEASE (from Newburger, et al., 2004) MICROPUSTULAR RASH AT PRESENTATION RASH AT DISCHARGE SUMMARY This poster printing was donated by Daniel Blueprint, Waco, TX.