The labs drawn showed WBC of 14,200 (neutrophil dominant), ALT: 126 U/L, AST: 51 U/L, ALP: 207 U/L Albumin: 3.4 gm/dl, Total bilirubin: 0.6 mg/dl, ESR: 61 mm/hr, CRP: 6.9 mg/dl. Urinalysis showed trace WBC with no nitrites. Therefore, he was asked to follow up at the clinic as soon as possible. On revaluation at the clinic three days later, all symptoms still persisted. He additionally also had redness of mouth and fissuring of lips. Examination revealed erythematous tongue and oral mucosa. There was mild periungual and facial desquamation. He was sent to the ER with a strong suspicion of Kawasaki disease.
At the hospital, repeat labs revealed lower WBC of 13,900 cell bil/L (neutrophil predominant). Liver markers had also reduced: ALT: 106 U/L, AST: 66 U/L, ALP: 174 U/L, Albumin: 3 gm/dl, Lactic acid: 1.5 mmol/L, ESR was increased at 76 mm/hr, Urine routine: No WBC’s. EKG showed normal sinus rhythm and no change from previous, 2D transthoracic echocardiogram showed an ejection fraction of 65% with mild tricuspid regurgitation, right ventricular systolic pressure was 51 mmHg, right upper quadrant ultrasound was unremarkable. He was given acetaminophen 650 mg PO Q6H, diphenhydramine 25 mg PO Q6H, Intravenous immunoglobulin (IVIG), Aspirin 975 mg PO QD. Rapid strep test was negative, however ASO and DNase- B antibody was mildly positive at 480 IU/ml and 282 U/mL respectively.
Lab test later revealed a neutrophilic leukocytosis, WBC count was 14,200 cells/ cumm. There was also a mild hepatitis and markers of inflammation (ESR and CRP) were elevated. Leukocytosis and hepatitis were a possibility in IMN, TSS and Kawasaki, however neutrophilic predominance favored Kawasaki and TSS. Patient was re-evaluated at the hospital three days later. By now, he complained of multiple spikes of fever on a daily basis. There was diffuse erythema of tongue and oral mucosa. There was also mild desquamation of rash over face and periungual desquamation which is fairly specific for Kawasaki.
Our adult 18-year-old patient had persistent headache that subsided only after the administration of IVIG and ASA. Given the patient’s age group, history of throat pain and generalized rash worsened with intake of amoxicillin, Infectious Mononucleosis (IMN) was high on our differential. Other differentials considered were; generalized allergic or contact dermatitis reaction and complications of group A streptococcus (GAS) pharyngitis such as Toxic Shock Syndrome, Acute rheumatic fever and Scarlet fever. Appropriate labs done ruled these other conditions out.
While at the hospital, labs were repeated which showed persistence of neutrophil leukocytosis. Ejection Fraction was 65%, there was mild left ventricular hypertrophy (LVH) and tricuspid regurgitation, however, there were no coronary artery aneurysms. Patient was immediately started on an IVIG infusion, high dose aspirin, diphenhydramine and acetaminophen. Patient started to feel much better after the first infusion. Periungual desquamation soon progressed to a generalized desquamation of both hands and feet. CMV, EBV and Parvovirus B19 IgG antibody was positive, however IgM was negative. In addition, EBV early Ag and nuclear Ag were also positive. This ruled out Infectious mononucleosis and Parvovirus as causes. ASO and Anti-DNase B were elevated which proved prior strep infection and was likely the cause for this presentation. Blood cultures showed a preliminary result and later reviewed Gram-positive rods; Corynebacterium manumission, Corynebacterium amycolatum and Diphtheriods which was considered to be a contaminant from the IV line.
Despite 50 years of research in this condition, there is only a small body of literature describing new onset Kawasaki disease in adults. Coronary artery aneurysm occurs in 15-25% of untreated children up to 5 % of treated ones. [6] Short term sequelae of coronary aneurysms; death due to coronary vasculitis have been identified and long-term consequences of endothelial dysfunction, coronary artery disease and myocardial infarction have been described in survivors [7][8]. There is also a drastic reduction in complications if identified and treated within 10 days of onset of symptoms [9] [10].
After discharge, desquamation progressed to involve both nails in hands and feet as well. Onychomadesis has been described in Kawasaki only twice before [11]. Ten days post discharge; WBC had normalized. However, LFT’ normalized only within 1 month of discharge. EBV IgG was positive at >750 and EBV early antigen at 52.1 U/mL, however EBV IgM was <10 U/ml. Parvovirus B19 IgG and CMV IgG were positive, however CMV and Parvovirus B19 IgM were negative. Patient continued to have spikes of fevers on the first day despite the IVIG infusion. By the 5th day after IVIG, patients’ conjunctival injection, myalgia and polymorphous rash had improved. Periungual desquamation had progressed to a severe desquamation of both hands and feet. A retrospective diagnosis of Kawasaki was then made, and the patient was discharged on Keflex, Cetirizine and continued on low dose aspirin for 6 weeks.