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Fatal Kikuchi-Fujimoto disease: the lupus connection

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An Erratum to this article was published on 08 May 2003

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.

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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.

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Correspondence to A. Quintás-Cardama.

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An erratum to this article is available at http://dx.doi.org/10.1007/s00277-003-0656-7.

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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

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  • DOI: https://doi.org/10.1007/s00277-003-0611-7

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