Pediatric Infectious Disease

Register      Login

VOLUME 4 , ISSUE 2 ( April-June, 2022 ) > List of Articles

Original Article

Kikuchi-Fujimoto Disease: An Experience from a Tertiary Care Center in South India

Rachna S Mohite, Tripti Kaur, Vidya MN, Sagar Bhattad

Keywords : Familial Kikuchi disease, Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto disease, Lupus erythematosus, Recurrent Kikuchi disease, Systemic

Citation Information : Mohite RS, Kaur T, MN V, Bhattad S. Kikuchi-Fujimoto Disease: An Experience from a Tertiary Care Center in South India. Pediatr Inf Dis 2022; 4 (2):38-42.

DOI: 10.5005/jp-journals-10081-1345

License: CC BY-NC 4.0

Published Online: 01-06-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Introduction: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis is a benign, usually self-limiting condition, that usually affects females under the age of 30 years. The etiology remains unknown. Methods: Analysis of all cases of KFD diagnosed at our center from January 2019 to March 2021 was carried out. A total of seven cases with KFD were included in the study. The clinical and laboratory parameters, associated comorbidities, and treatment outcomes were studied and analyzed in detail. Results: The mean age of presentation was 11 years with a female predominance. Fever and lymphadenopathy were noted in the majority; while weight loss and fatigue were noted in 5/7. One patient had a recurrence of KFD, whereas two sisters presented with familial KFD. Most patients had leukopenia and raised ESR at presentation. ANA was positive in 5/7 (71%) of patients, of which two had systemic lupus erythematous at presentation. Steroids were used in all except one patient. Conclusion: We hereby report our experience in the diagnosis and management of KFD and re-emphasize that KFD must be considered as a possibility in febrile children with lymphadenopathy.

PDF Share
  1. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. Acta Haematologica Japan 1972; 35:379–380.
  2. Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis. A new clinicopathological entity. Naika 1972;30: 920–927.
  3. Tanaka T, Ohmori M, Yasunaga S, et al. DNA typing of HLA class II genes (HLA-DR, -DQ and -DP) in Japanese patients with histiocytic necrotizing lymphadenitis (Kikuchi's disease). Tissue Antigens. 1999;54(3):246–253. DOI: 10.1034/j.1399-0039.1999.540305.x
  4. Bagri PK, Beniwal S, Jakhar SL, et al. Kikuchi-Fujimoto disease: A diagnostic and therapeutic challenge. Clin Cancer Investig J 2014;3(3):254. DOI: 10.4103/2278-0513.132125
  5. Kapadia V, Robinson BA, Angus HB. Kikuchi's disease presenting as fever of unknown origin. Lancet 1989;2(8678–8679):1519–1520. DOI: 10.1016/s0140-6736(89)92956-5
  6. Chen JS, Chang KC, Cheng CN, et al. Childhood hemophagocytic syndrome associated with Kikuchi's disease. Haematologica 2000;85(9):998–1000.
  7. Yu HL, Lee SS, Tsai HC, et al. Clinical manifestations of Kikuchi's disease in southern Taiwan. J Microbiol Immunol Infect 2005;38(1):35–40.
  8. Lee KY, Yeon YH, Lee BC. Kikuchi-Fujimoto disease with prolonged fever in children. Pediatrics 2004;114(6):e752–e756 DOI: 10.1542/peds.2004-0485
  9. Deaver D, Horna P, Cualing H, et al. Diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control 2014;21(4):313–321. DOI: 10.1177/107327481402100407
  10. Zou CC, Zhao ZY, Liang L. Childhood Kikuchi-Fujimoto disease. Indian J Pediatr 2009;76(9):959–962. DOI: 10.1007/s12098-009-0194-y
  11. Kikuchi M, Takeshita M, Eimoto T, et al. Lymphoid malignancy: immunocytologic and cytogenetics.
  12. Guleria S, Gupta A, Pilania RK, et al. Kikuchi-Fujimoto Disease: an under recognized cause of fever with lymphadenopathy. Indian J Pediatr 2020;87(1):85. DOI: 10.1007/s12098-019-03070-8
  13. Kucukardali Y, Solmazgul E, Kunter E, et al. Kikuchi-Fujimoto disease: analysis of 244 cases. Clin Rheumatol 2007;26(1):50–54. DOI: 10.1007/s10067-006-0230-5
  14. Kang HM, Kim JY, Choi EH, et al. Clinical characteristics of severe histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto Disease) in lhildren. J Pediatr 2016;171:208–212. DOI: 10.1016/j.jpeds.2015.12.064
  15. Dumas G, Prendki V, Haroche J, et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. Medicine (Baltimore) 2014;93(24):372–382. DOI: 10.1097/MD.0000000000000220
  16. Park HS, Sung MJ, Park SE, et al. Kikuchi-Fujimoto disease of 16 children in a single center of Korea. Pediatr Allergy Immunol 2007;18(2):174–178. DOI: 10.1111/j.1399-3038.2006.00505.x
  17. Perry AM, Choi SM. Kikuchi-Fujimoto disease: A review. Arch Pathol Lab Med 2018;142(11):1341–1346. DOI: 10.5858/arpa.2018-0219-RA
  18. Sopeña B, Rivera A, Vázquez-Triñanes C, et al. Autoimmune manifestations of Kikuchi disease. Semin Arthritis Rheum 2012;41(6):900–906. DOI: 10.1016/j.semarthrit.2011.11.001
  19. Baenas DF, Diehl FA, Haye Salinas MJ, et al. Kikuchi-Fujimoto disease and systemic lupus erythematosus. Int Med Case Rep J 2016;9:163–167. DOI: 10.2147/IMCRJ.S106396
  20. Khanna D, Shrivastava A, Malur PR, et al. Necrotizing lymphadenitis in systemic lupus erythematosus: is it Kikuchi-Fujimoto disease? J Clin Rheumatol 2010;16(3):123–124. DOI: 10.1097/RHU.0b013e3181d56afb
  21. Smith LW, Gelber AC, Petri M. Diffuse lymphadenopathy as the presenting manifestation of systemic lupus erythematosus. J Clin Rheumatol 2013;19(7):397–399. DOI: 10.1097/RHU.0b013e3182a6a924
  22. Martínez-Vázquez C, Hughes G, Bordon J, et al. Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's disease, associated with systemic lupus erythemotosus. QJM 1997;90(8):531–533. DOI: 10.1093/qjmed/90.8.531
  23. Cheng CY, Sheng WH, Lo YC, et al. Clinical presentations, laboratory results and outcomes of patients with Kikuchi's disease: emphasis on the association between recurrent Kikuchi's disease and autoimmune diseases. J Microbiol Immunol Infect 2010;43(5):366–371. DOI: 10.1016/S1684-1182(10)60058-8
  24. Amir AR, Amr SS, Sheikh SS. Kikuchi-Fujimoto's disease: report of familial occurrence in two human leucocyte antigen-identical non-twin sisters. J Intern Med 2002;252(1):79–83. DOI: 10.1046/j.1365-2796.2002.01004.x
  25. Kikuchi M, Takeshita M, Eimoto T, et al. Histiocytic necrotizing lymphadenitis: clinicopathologic, immunologic, and HLA typing study. In: Hanaoka M, Kadin M, Mikata A, eds. Lymphoid Malignancy. New York: Field and Wood, 1990; 251–257.
  26. Atarashi K, Yoshimura N, Nodera H, et al. Recurrent histiocytic necrotizing lymphadenitis (Kikuchi's disease) in an human T lymphotropic virus type I carrier. Intern Med 1996;35(10):821–825. DOI: 10.2169/internalmedicine.35.821
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.