Kikuchi Fujimoto disease with macrophage activation syndrome, seizures, and pleuritis in a young child

Abstract

Kikuchi Fujimoto disease (KFD) is rare in children and generally a benign, self-limiting disease that rarely runs a complicated course. We report a rare case of KFD in a young girl that had a complicated course with macrophage activation syndrome (MAS), posterior reversible encephalopathy syndrome (PRES) and pleuritis needing ventilatory support and prolonged immunosuppression with good recovery. This illustrates KFD can rarely also have unusual presentation needing advanced immunosuppression and a careful follow up.