{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T00:00:12Z","timestamp":1780531212718,"version":"3.54.1"},"reference-count":15,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T00:00:00Z","timestamp":1597017600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T00:00:00Z","timestamp":1597017600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Pediatr Rheumatol"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970\u2019s. Despite its rarity in the pediatric population, it is an important differential in persistent lymphadenopathy. Familial cases of KFD in the literature are rare. Here we describe the first reported case of KFD in non-identical twin sisters.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Case presentation<\/jats:title>\n<jats:p>Twin 1 presented with a 3-week history of worsening right-sided cervical lymphadenopathy, daily fevers, significant lethargy, weight loss and arthralgia of her knees and ankles at the age of 12\u2009years in 2015. She had had an unremarkable medical history. A biopsy of her lymph nodes showed histiocytic necrosis consistent with KFD. Twin 2 presented with a three-week history of lethargy, fatigue, weight loss and left-sided posterior cervical chain lymphadenopathy at 16\u2009years of age in 2018. She had a history of frequently relapsing nephrotic syndrome and celiac disease. A biopsy of her lymph nodes was undertaken and showed histiocytic necrosis consistent with KFD.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>KFD is a rare but self-limiting pathological process of necrotizing histiocytic lymphadenitis. Although further research is needed, there is an increasing amount of evidence which suggests a multifactorial pathological basis of disease. The two cases we document here are the first reported cases of familial KFD in dizygotic HLA-identical twins which reinforces the likely HLA-linkage in the etiology of KFD.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s12969-020-00457-2","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T18:03:56Z","timestamp":1597082636000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A familial case of Kikuchi-Fujimoto disease in dizygotic twins"],"prefix":"10.1186","volume":"18","author":[{"given":"Ashfaque","family":"Quadir","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ken","family":"Peacock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davinder","family":"Singh-Grewal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Alexander","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,10]]},"reference":[{"key":"457_CR1","first-page":"379","volume":"35","author":"M Kikuchi","year":"1972","unstructured":"Kikuchi M. Lymphadenitis showing focal hyperplasia with nuclear debris and phagocytes: a clinicopathological study. Acta Haematol Jpn. 1972;35:379.","journal-title":"Acta Haematol Jpn"},{"key":"457_CR2","first-page":"920","volume":"20","author":"Y Fujimoto","year":"1972","unstructured":"Fujimoto Y. Cervical subacute necrotizing lymphadenitis. A new clinicopathological entity. Intern Med. 1972;20:920\u20137.","journal-title":"Intern Med"},{"issue":"7","key":"457_CR3","doi-asserted-by":"crossref","first-page":"1701","DOI":"10.1681\/ASN.2014030247","volume":"26","author":"RA Gbadegesin","year":"2015","unstructured":"Gbadegesin RA, Adeyemo A, Webb NJ, Greenbaum LA, Abeyagunawardena A, Thalgahagoda S, et al. HLA-DQA1 and PLCG2 are candidate risk loci for childhood-onset steroid-sensitive nephrotic syndrome. 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CMAJ. 2011;183(1):E58\u201360.","journal-title":"CMAJ."},{"issue":"1","key":"457_CR6","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1046\/j.1365-2796.2002.01004.x","volume":"252","author":"A Amir","year":"2002","unstructured":"Amir A, Amr S, Sheikh S. Kikuchi\u2013Fujimoto's disease: report of familial occurrence in two human leucocyte antigen-identical non-twin sisters. J Intern Med. 2002;252(1):79\u201383.","journal-title":"J Intern Med"},{"issue":"3","key":"457_CR7","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1007\/BF01606059","volume":"418","author":"S Asano","year":"1991","unstructured":"Asano S, Akaike Y, Muramatsu T, Wakasa H, Yoshida H, Kondou R, et al. Necrotizing lymphadenitis: a clinicopathological and immunohistochemical study of four familial cases and five recurrent cases. 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Consent to participate was sought from the participant\u2019s parents using the consent form of the Sydney Children\u2019s Hospital Human Research Ethics Committee.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Consent for publication was gained from the participants\u2019 parents using our local institutional consent form from our ethics committee.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"62"}}