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Kikuchi-Fujimoto disease, or necrotizing lymphadenitis, is a rare benign, self-limiting syndrome that was first described independently by Kikuchi and Fujimoto in 1972. It is prevalent mainly in Asian countries. However, cases have recently been reported from most parts of the world.
The exact pathogenesis of KFD is unknown. The disease has distinctive pathologic features that separate it from toxoplasmosis.1 Although it has been suggested that KFD can be caused by infectious agents such as Yersinia enterocolitica, herpesvirus, cytomegalovirus, and varicella-zoster, parainfluenza, and Epstein-Barr viruses, recent studies have failed to confirm this theory.2 The presence of cytoplasmic tubuloreticular structures, like those seen in systemic lupus erythematosus, suggests that abnormal cell-mediated immune response is an etiologic factor.3 Lin et al4 theorized that the absence of monoclonal T-cell receptor rearrangements excludes the possibility of T-cell lymphoma and that the presence of oligoclonal pattern is evidence of a benign immune reaction.
Pathology Quiz Case—Diagnosis. Arch Otolaryngol Head Neck Surg. 2004;130(10):1234. doi:10.1001/archotol.130.10.1234
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