Retinopathy in lupus transitioned to Kikuchi-Fujimoto disease

PURPOSE: We present a patient with systemic lupus erythematosus with significant vaso-occlusive retinal findings mimicking antiphospholipid antibody syndrome, who developed Kikuchi-Fujimoto disease. OBSERVATIONS: Our patient was initially diagnosed with systemic lupus erythematosus with antiphospholipid antibody syndrome given consistent serologic markers and profound retinal vascular ischemia. However, on subsequent follow up, she presented with fever and lymphadenopathy and underwent lymph node biopsy, which declared histologic findings of Kikuchi-Fujimoto disease. Repeat markers for antiphospholipid antibody syndrome were negative and she was taken off lifelong anticoagulation. CONCLUSIONS AND IMPORTANCE: Systemic lupus erythematosus and Kikuchi-Fujimoto disease may have many similar features and even biomarkers, and given the potential overlap of presentation, clinicians must carefully distinguish between these diseases to prevent unnecessary treatment.