Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature
Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cau...
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2024-11-01
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| author | Arunima Deb Vielka Fernandez Ekim Kilinc Hisham F. Bahmad Nicholas S. Camps Vathany Sriganeshan Ana Maria Medina |
| author_facet | Arunima Deb Vielka Fernandez Ekim Kilinc Hisham F. Bahmad Nicholas S. Camps Vathany Sriganeshan Ana Maria Medina |
| author_sort | Arunima Deb |
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| description | Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cause remains unclear, infectious and autoimmune mechanisms have been implicated in the pathogenesis of the disease. In this case series, we aim to describe the histopathological features of KFD over a ten-year period at Mount Sinai Medical Center of Florida, and review the current understanding of its pathogenesis, clinical presentation, diagnosis, and management. A retrospective review of our pathology database between January 2013 and May 2024 was performed to identify patients diagnosed with KFD at our institution. Eight cases of KFD were identified, with a mean age of 35 years (range 24–49) and slight male predilection (5:3). Three patients exhibited leukopenia, and two had concurrent HIV infection. One patient developed systemic lupus erythematosus (SLE), and another developed IgA nephropathy during follow-up. Histopathological examination revealed the characteristic features of KFD, including lymph node architectural effacement, histiocytic infiltration, and necrosis. In conclusion, KFD remains a diagnostic challenge due to its overlapping clinical features with other infectious and autoimmune diseases, particularly SLE. While most cases resolve spontaneously, long-term follow-up is warranted due to the potential for recurrence and autoimmune associations. |
| format | Article |
| id | doaj-art-04c7eaa10604440a8c20f1f588bc9f94 |
| institution | Kabale University |
| issn | 2079-9721 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | MDPI AG |
| record_format | Article |
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| spelling | doaj-art-04c7eaa10604440a8c20f1f588bc9f942024-11-26T18:00:08ZengMDPI AGDiseases2079-97212024-11-01121127110.3390/diseases12110271Kikuchi–Fujimoto Disease: A Case Series and Review of the LiteratureArunima Deb0Vielka Fernandez1Ekim Kilinc2Hisham F. Bahmad3Nicholas S. Camps4Vathany Sriganeshan5Ana Maria Medina6The Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAThe Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAThe Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAThe Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USADepartment of Internal Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAThe Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAThe Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL 33140, USAKikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cause remains unclear, infectious and autoimmune mechanisms have been implicated in the pathogenesis of the disease. In this case series, we aim to describe the histopathological features of KFD over a ten-year period at Mount Sinai Medical Center of Florida, and review the current understanding of its pathogenesis, clinical presentation, diagnosis, and management. A retrospective review of our pathology database between January 2013 and May 2024 was performed to identify patients diagnosed with KFD at our institution. Eight cases of KFD were identified, with a mean age of 35 years (range 24–49) and slight male predilection (5:3). Three patients exhibited leukopenia, and two had concurrent HIV infection. One patient developed systemic lupus erythematosus (SLE), and another developed IgA nephropathy during follow-up. Histopathological examination revealed the characteristic features of KFD, including lymph node architectural effacement, histiocytic infiltration, and necrosis. In conclusion, KFD remains a diagnostic challenge due to its overlapping clinical features with other infectious and autoimmune diseases, particularly SLE. While most cases resolve spontaneously, long-term follow-up is warranted due to the potential for recurrence and autoimmune associations.https://www.mdpi.com/2079-9721/12/11/271Kikuchi–Fujimotohistiocytic necrotizing lymphadenopathyEBVHIVcase series |
| spellingShingle | Arunima Deb Vielka Fernandez Ekim Kilinc Hisham F. Bahmad Nicholas S. Camps Vathany Sriganeshan Ana Maria Medina Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature Diseases Kikuchi–Fujimoto histiocytic necrotizing lymphadenopathy EBV HIV case series |
| title | Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature |
| title_full | Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature |
| title_fullStr | Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature |
| title_full_unstemmed | Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature |
| title_short | Kikuchi–Fujimoto Disease: A Case Series and Review of the Literature |
| title_sort | kikuchi fujimoto disease a case series and review of the literature |
| topic | Kikuchi–Fujimoto histiocytic necrotizing lymphadenopathy EBV HIV case series |
| url | https://www.mdpi.com/2079-9721/12/11/271 |
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