Kakuchi-Fujimoto Disease Associated with Autoimmune Hepatitis and Systemic Lupus Erythematosus


Kakuchi-Fujimoto Disease Associated with Autoimmune Hepatitis and Systemic Lupus Erythematosus


Mithu Maheswaranathan, M.D.1 and Melissa Cunningham, M.D., Ph.D.2

From the Department of Internal Medicine1 and Division of Rheumatology2, Medical University of South Carolina, Charleston, SC, USA


International-Journal-of-Case-Reports-2d code

Systemic lupus erythematosus (SLE) is often associated with various systemic manifestations, from peripheral cytopenias to renal involvement or neuropsychiatric symptoms. Here, we present a patient in whom the cause of fever and altered mental status was unknown despite repeated lumbar punctures, but later became elucidated following the development of rash and lymphadenopathy with subsequent biopsies. This case demonstrates a unique presentation of Kakuchi-Fujimoto Disease, a necrotizing lymphadenitis manifesting in association with SLE and autoimmune hepatitis. Further, this case illustrates the proclivity of autoimmune disorders to occur concurrently.

Author contributions: Mithu Maheswaranathan – drafting of the manuscript; critical revision of the manuscript for important intellectual content; Melissa Cunningham – drafting of the manuscript; critical revision of the manuscript for important intellectual content, supervisory


Keywords: systemic lupus erythematosus, lymphadenopathy, autoimmune hepatitis, Kakuchi-Fujimoto Disease, necrotizing lymphadenitis, encephalopathy


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How to cite this article:
Mithu Maheswaranathan and Melissa Cunningham. Kakuchi-Fujimoto Disease Associated with Autoimmune Hepatitis and Systemic Lupus Erythematosus. International Journal of Case Reports, 2018 2:25. DOI: 10.28933/ijcr-2018-05-2802.


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