Epstein-Barr virus-associated Kikuchi disease in two children

The authors describe two children with Kikuchi necrotizing lymphadenitis, t he main manifestations of which were cervical lymphadenopathy, fatigue, and fever. The diagnosis was based on histopathologic findings after open biop sy. Results of serologic studies, immunoperoxidase staining for Epstein-Bar r virus (EBV) latent membrane protein, in situ hybridization for Epstein-Ba rr encoded RNAs, and polymerase chain reaction amplification of EBV Epstein -Barr nuclear antigen-1 (EBNA) DNA suggested that EBV was the causative age nt in both patients. The disease was mild and subsided after complete surgi cal resection in one patient, with a follow-up of 1 year. In the other pati ent, a short course of corticosteroids led to complete clinical remission w ithin 2 months, but the child still has biologic signs of persistent EBV in fection. He experienced relapse with a large cervical mass and fever 28 mon ths after the initial onset. Histologic findings were identical to those at initial presentation. Symptoms again resolved spontaneously within 2 weeks , but the follow-up was short (12 mos) and the child's EBNA antibodies are still absent. No evidence of immunodeficiency was found in either child. Th e cause of Kikuchi disease is unknown. but a viral or postviral hyperimmune reaction has been proposed. Malignant lymphoma and systemic lupus erythema tosus are differential diagnoses. Early recognition of Kikuchi disease mini mizes potentially harmful and unnecessary investigations and treatments. Th ese findings add Kikuchi disease to the protean manifestations of chronic E BV infection.