Kikuchi-Fujimoto Disease With Systemic Lupus Erythematosus and Systemic Sclerosis Overlap: A Unique Clinical Presentation

菊池-藤本病合并系统性红斑狼疮和系统性硬化症:一种独特的临床表现

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Abstract

Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis (HNL), is a rare self-limiting disorder presenting with fever and swollen lymph nodes. It is characterized by the focal proliferation of reticular cells, the presence of nuclear debris, and histiocytes. In advanced cases, it can present with hepato-splenomegaly and generalized lymphadenopathy. Historically, it has been associated with viral infections, as it frequently was found to be associated with upper respiratory symptoms. Alternative explanations include the immune response of T-cells leading to alteration in CD8-positive T-cell-mediated cell apoptosis. It is also speculated that KFD can be associated with rheumatological autoimmune diseases. We present a case of a 21-year-old African American female with a known diagnosis of systemic lupus erythematosus (SLE)-systemic sclerosis (SS) overlap presented with febrile lymphadenopathy and was diagnosed to have HNL on histological exam of lymph node biopsy.

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