Diagnostic Puzzle of Atypical Kawasaki Disease

Kawasaki disease (KD) is a systemic inflammatory illness characterized by medium blood vessel vasculitis and is considered to be the leading cause of acquired heart disease in children less than 5 years old in developed countries. KD can present as either a classical or atypical/incomplete presentation based on distinct laboratory parameters seen in Table 1. The exact cause of KD remains unknown; however, specific purported infectious agents have been theorized to be linked to it. It classically presents with fever and four of the following: conjunctival injection, oral mucosal changes, cervical lymphadenopathy, changes in the extremities and polymorphous rash. Rarely, KD can present with hepatobiliary syndrome such as hepatic congestion, obstructive jaundice, acalculous cholecystitis and gallbladder hydrops. We present a case of a 12-year-old, otherwise healthy male who presented with an 8-day history of upper respiratory infection symptoms and fevers up to 102 F along with significant jaundice, dilated common bile duct and transaminitis initially thought to be ascending cholangitis. His clinical course evolved to also show rash, abdominal pain, vomiting, diarrhea, cervical lymphadenopathy and non-exudative, bilateral conjunctival injection with limbic sparing. Absence of improvement to antibiotic and distinct clinical evolution prompted the diagnosis of atypical KD. He received high dose IVIG and high dose aspirin with apparent clinical improvement. His surveillance echocardiography studies and subsequent physical exam findings have all been reassuring. This case underscores how KD can initially present as cholangitis and how it can manifest as a concomitant process during a viral infection.