Case report on a rare entity–Kikuchi Fujimoto Disease (KFD) in a person with underlying Systemic Lupus Erythematosus (SLE)

Kikuchi-Fujimoto disease (KFD) is a rare benign condition of necrotizing histiocytic lymphadenitis. The case of a 31-year-old gentleman who presented with fever, weight loss, and a single tender right-sided cervical lymph node is described here. Symptomatic treatment was provided initially, but the course was complicated with an episode of aseptic meningitis and admission to the medical intensive care unit (MICU). The Liver enzymes and lactate dehydrogenase (LDH) were also found to be very high during this period but returned to baseline. He was also found to have oral ulcers, pleural effusion, positive serology for lupus, and pancytopenia with lymphopenia and autoimmune hemolytic Anemia. The entire illness lasted for about six weeks. Although tissue diagnosis was delayed because of the MICU admission, KFD was finally diagnosed after cervical lymph node biopsy. Cases of KFD has been reported widely, but most of them had an uneventful course. We believe that the eventful course and the presence of underlying lupus make this case worth reporting. Take away lesson from this case is that tissue diagnosis should not be delayed in persons presenting with generalized or localized lymphadenopathy associated with systemic symptoms and along with common conditions rare entities like KFD should be considered especially if the patient does not respond to routine treatment.