Enigmatic Kikuchi-Fujimoto Disease: A Comprehensive Review

Xavier Bosch, MD; Antonio Guilabert, MD; Rosa Miquel, MD; Elias Campo, MD


Am J Clin Pathol. 2004;122(1) 

In This Article

Literature Review and Epidemiology

Literature Review

We reviewed relevant articles published in English-language medical journals from 1972 (when KFD was first reported) to the present day through a MEDLINE search using the keywords "kikuchi" and "histiocytic necrotizing lymphadenitis." In addition to original and review articles and editorials, we included outstanding case reports and letters.


KFD is known to have a worldwide distribution with a higher prevalence among Japanese and other Asiatic people.[1,2,3,4,5] It is scarcely known in the Western hemisphere. In fact, the first description of the disorder outside Asia was made by Pileri and colleagues6 in 1982 (with Kikuchi as a coauthor). Cases reported by Pileri et al[6] included 23 from the former West Germany and individual cases from Iran, Italy, South Korea, and Spain. It since has been reported worldwide.[7,8,9,10,11,12,13,14,15,16,17,18] Affected patients most often are adults younger than 40 years (range, 19 months to 75 years).[16,18,19,20,21] A recent series from Taiwan of 61 patients with KFD revealed a mean age of 21 years.[22] In general, a female preponderance has been reported (female/male ratio, 4:1).[17,19,20] Yet recent reports from Eastern countries seem to indicate that the female preponderance was overemphasized in the past and that the actual ratio is closer to 1:1.[17,22]


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