Kikuchi-Fujimoto Disease: A Case Report of Fever and Lymphadenopathy in a Young White Man

Abstract

Background: Kikuchi-Fujimoto disease (KFD) is a rare cause of rapidly evolving tender cervical lymphadenopathy. It is often initially misdiagnosed and managed as infectious lymphadenitis. Although most cases of KFD are self-limited and improve with antipyretics and analgesics, some are more refractory and may require corticosteroids or hydroxychloroquine therapy.

Case presentation: A 27-year-old White man presented for evaluation of fevers and painful cervical lymphadenopathy. He was found to have KFD on excisional lymph node biopsy. His symptoms proved challenging to manage with corticosteroids but eventually improved with hydroxychloroquine monotherapy.

Conclusions: KFD diagnosis should be considered irrespective of geographic location, ethnicity, or patient sex. Hepatosplenomegaly is a relatively rare manifestation of KFD that can make it especially difficult to distinguish from lymphoproliferative disorder, such as lymphoma. Lymph node biopsy is the preferred diagnostic approach to achieve a timely and definitive diagnosis. Although usually self-limited, KFD has been associated with autoimmune conditions, including systemic lupus erythematosus. Securing the diagnosis of KFD is therefore crucial to ensuring patients are monitored appropriately for the development of associated autoimmune conditions.

FIGURE 1

Computed Tomography of the Neck With Contrast Right cervical lymphadenopathy with cystic and necrotic changes can be seen on the axial (A) and coronal (B) computed tomography.

FIGURE 2

Hematoxylin and Eosin Stain of Cervical Lymph Node Lymphohistiocytic inflammation and necrosis can be seen, which are characteristic of Kikuchi-Fujimoto disease (original magnification ×40).