Incomplete, atypical kawasaki disease or evolving systemic juvenile idiopathic arthritis: a case report

Kawasaki disease is an acute febrile condition seen in children. However, it is also well recognized that some patients do not fulfill the classic diagnostic criteria for the diagnosis of kawasaki disease. The incomplete form of kawasaki disease is termed as 'Incomplete KD' or 'Atypical KD'. We present a case of a 6 year old child with a history of prolonged fever, periorbital, oral and lip changes, changes in the extremities and an erythamatous, maculopapular rash. Based on the physical exam and her echocardiogram that showed right coronary artery dilatation, Intravenous immune globulin was administered in this patient. This patient was refractory to two doses of intravenous immune globulin and therefore was started on methylprednisolone, to which she responded dramatically. The diagnostic dilemma primarily arose when this child presented with joint pain a day after her discharge from the hospital and a positive laboratory workup. So, was this a case of incomplete Kawasaki refractory to intravenous immuno globulin therapy or systemic juvenile idiopathic arthritis? We suggest that physicians should be cognizant of the fact that they must individualize every patient's management to the best of their knowledge and judgment, rather than merely going by the guidelines.