A familial case of Kikuchi-Fujimoto disease in dizygotic twins
Abstract Background Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970’s. Despite its rarity in the pediatric population, it is an important differential in persistent lymphadenopathy. Familial cas...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2020-08-01
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| Series: | Pediatric Rheumatology Online Journal |
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| Online Access: | http://link.springer.com/article/10.1186/s12969-020-00457-2 |
| _version_ | 1819276676038656000 |
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| author | Ashfaque Quadir Ken Peacock Peter Hsu Davinder Singh-Grewal Stephen Alexander |
| author_facet | Ashfaque Quadir Ken Peacock Peter Hsu Davinder Singh-Grewal Stephen Alexander |
| author_sort | Ashfaque Quadir |
| collection | DOAJ |
| description | Abstract Background Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970’s. 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. Case presentation 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 years 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 years 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. Conclusions 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. |
| first_indexed | 2024-12-23T23:44:00Z |
| format | Article |
| id | doaj.art-4ff2954cccbe427d9aafcb10df8a0254 |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-12-23T23:44:00Z |
| publishDate | 2020-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-4ff2954cccbe427d9aafcb10df8a02542022-12-21T17:25:33ZengBMCPediatric Rheumatology Online Journal1546-00962020-08-011811510.1186/s12969-020-00457-2A familial case of Kikuchi-Fujimoto disease in dizygotic twinsAshfaque Quadir0Ken Peacock1Peter Hsu2Davinder Singh-Grewal3Stephen Alexander4Department of General Medicine, The Children’s Hospital at WestmeadDepartment of General Medicine, The Children’s Hospital at WestmeadDepartment of Immunology, The Children’s Hospital at WestmeadDepartment of Rheumatology, The Sydney Children’s Hospitals NetworkDepartment of Nephrology, The Children’s Hospital at WestmeadAbstract Background Kikuchi-Fujimoto disease (KFD) or necrotizing histiocytic lymphadenitis, was described separately by both Kikuchi and Fujimoto in Japan in the early 1970’s. 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. Case presentation 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 years 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 years 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. Conclusions 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.http://link.springer.com/article/10.1186/s12969-020-00457-2Kikuchi-FujimotoTwinsFamilialLymphadenopathyNecrotizingHistiocytic |
| spellingShingle | Ashfaque Quadir Ken Peacock Peter Hsu Davinder Singh-Grewal Stephen Alexander A familial case of Kikuchi-Fujimoto disease in dizygotic twins Pediatric Rheumatology Online Journal Kikuchi-Fujimoto Twins Familial Lymphadenopathy Necrotizing Histiocytic |
| title | A familial case of Kikuchi-Fujimoto disease in dizygotic twins |
| title_full | A familial case of Kikuchi-Fujimoto disease in dizygotic twins |
| title_fullStr | A familial case of Kikuchi-Fujimoto disease in dizygotic twins |
| title_full_unstemmed | A familial case of Kikuchi-Fujimoto disease in dizygotic twins |
| title_short | A familial case of Kikuchi-Fujimoto disease in dizygotic twins |
| title_sort | familial case of kikuchi fujimoto disease in dizygotic twins |
| topic | Kikuchi-Fujimoto Twins Familial Lymphadenopathy Necrotizing Histiocytic |
| url | http://link.springer.com/article/10.1186/s12969-020-00457-2 |
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