Abstract
Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotizing lymphadenitis found mainly in young women. Patients typically present with cervical lymphadenopathy and fever and follow almost always a benign course with excellent outcomes. The etiology of KFD remains unknown and controversial although several viruses have been associated with this disease. One theory proposes that KFD may be a self-limiting form of systemic lupus erythematosus (SLE). This theory is strongly supported by the fact that microscopic features of KFD can be very similar to those found in lupus lymphadenitis. Despite its usually benign course, several cases with fatal outcomes have been reported. We report here a case of clinically aggressive KFD, which featured several autoimmune-related events and resulted in a fatal outcome. Autopsy studies showed characteristic findings of SLE, which suggests a remarkable link between SLE and KFD. Early and intensive immunosuppressive treatment may be the only option for patients who develop very aggressive forms of KFD in order to avoid a fatal outcome.
Similar content being viewed by others
References
Kikuchi M (1972) Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis. Nippon Ketsueki Gakkai Zasshi 35:379–380
Fujimoto Y, Kozima Y, Yamaguchi K (1972) Cervical subacute necrotizing lymphadenitis. A new clinicopathologic agent. Naika 20:920–927
Sire S, Djossou F, Deminiere C, et al. (1996) Kikuchi Fujimoto necrotizing lymphadenitis: a propose of 2 cases and review of the literature. Rev Med Interne 17:842–845
Pileri S, Kikuchi M, Helbron D, Lennert K (1982) Histiocytic necrotizing lymphadenitis without granulocytic infiltration. Virchows Arch 395:257–271
Chamulak GA, Brynes RK, Nathwani BN (1990) Kikuchi-Fujimoto disease mimicking malignant lymphoma. Am J Surg Pathol 14:514–523
Turner RR, Martin J, Dorfman RF (1983) Necrotizing lymphadenitis: a study of 30 cases. Am J Surg Pathol 7:115–123
Chan JKC, Wong K-C, Ng C-S (1989) A fatal case of multicentric Kikuchi's histiocytic necrotizing lymphadenitis. Cancer 63:1856–1862
O'Neill D, O'Grady J, Variend S (1998) Child fatality associated with pathological features of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Pediatr Pathol Lab Med 18:79–88
Feller AC, Lennert K, Stein H, Bruhn H-D, Wuthe H-H (1983) Immunohistology and etiology of histiocytic necrotizing lymphadenitis: report of three instructive cases. Histopathology 7:825–839
Dorfman RF, Berry GJ (1988) Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 5:329–345
Imumura M, Ueno H, Matsumura A, et al. (1982) An ultrastructural study of subacute necrotizing lymphadenitis. Am J Pathol 107:292–299
Litwin MD, Kirkham B, Henderson DR, Milazzo SC (1992) Histiocytic necrotizing lymphadenitis in SLE. Ann Rheum Dis 52:805–807
Quaini F, Manganelli P, Pileri S, et al. (1991) Immunohistological characterization of lymph nodes in adult onset Still's disease. J Rheumatol 18:1418
Trotta F, Dovigo L, Scapoli G, et al. (1993) Immunoblastic malignant lymphoma in adult onset Still's disease. J Rheumatol 20:1788
Tsang WY, Chan JKC, Ng CS (1994) Kikuchi´s lymphadenitis. A morphologic analysis of 75 cases with special reference to unusual features. Am J Surg Pathol 18:219–231
Tan EM, Cohen AS, Fries JF (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277
Eisner M, Amory J, Mullaney B, Tierney Jr L, Browner W (1996) Necrotizing lymphadenitis associated with systemic lupus erythematosus. Semin Arthritis Rheum 26:477–482
Acknowledgements
The authors are indebted to Dr. JKC Chan from the Queen Elizabeth Hospital (Hong Kong) for his kind revision of the biopsy samples and valuable comments about this case. We also want to thank the members of the Arkady M Rywlin Club whose very appropriate discussions greatly contributed to the preparation of the manuscript. A.Q-C is especially grateful to Amy Chang who contributed to the edition of the final version of the text.
Author information
Authors and Affiliations
Corresponding author
Additional information
An erratum to this article is available at http://dx.doi.org/10.1007/s00277-003-0656-7.
Rights and permissions
About this article
Cite this article
Quintás-Cardama, A., Fraga, M., Cozzi, S.N. et al. Fatal Kikuchi-Fujimoto disease: the lupus connection. Ann Hematol 82, 186–188 (2003). https://doi.org/10.1007/s00277-003-0611-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00277-003-0611-7