Histiocytic necrotizing lymphadenitis or Kikuchi disease is a rare entity; furthermore, its association with systemic lupus erythematosus (SLE) was only described in 11 patients in seven reports in the world literature. As the disease affects young women and manifests as a systemic illness associated with lymphadenopathy (usually cervical), the importance of continued follow-up to see whether it remains a self-limiting process or evolves into systemic lupus erythematosus should be emphasized. We present the clinical and histopathological data of eight patients with Kikuchi disease diagnosed in one tertiary care centre, two of whom evolved into SLE. White blood count was normal in all except the two patients who evolved into SLE; erythrocyte sedimentation rate was elevated in all patients tested. Kikuchi disease could be misdiagnosed as non-Hodgkin's lymphoma or tuberculous lymphadenitis; furthermore, awareness of its association with SLE is emphasized.