Kikuchi-Fujimoto Disease: A Rare Cause of Cervical Lymphadenopathy

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare benign disease, clinically characterized by fever and tender cervical lymphadenopathy affecting the posterior cervical lymph nodes. This disease is usually accompanied by night sweats, rashes, and headaches. It generally affects young individuals, especially females, of Oriental-Asian origin. The etiology of KFD remains uncertain, but associations have been noted with viral diseases including Epstein-Barr virus (EBV), herpes simplex virus (HSV), and varicella-zoster virus (VZV), as well as autoimmune disorders including systemic lupus erythematosus (SLE) and Sjogren's syndrome. This review points out the etiology of KFD with cervical lymphadenopathy alongside its clinical presentation, histological highlights, lab investigations, complications, and treatment. Accurate diagnosis of this disease depends on lymph node excisional biopsy. Three histological patterns of KFD are recognized: proliferative, necrotizing, and xanthomatous. Distinction from lymphadenopathy-associated alternate disorders (e.g., SLE, malignancy, tuberculosis, or another infectious lymphadenitis) is essential to ensure appropriate therapy. This self-limited condition entails nonsteroidal anti-inflammatory drugs (NSAIDs) for pain relief with consideration of corticosteroids and hydroxychloroquine in severe cases.

Abdominal Kikuchi-Fujimoto lymphadenopathy: an uncommon presentation of a rare disease

“…KFD is a histiocytic necrotising lymphadenitis, rare in Western countries but prevalent in the Asiatic population. It has been reported most frequently in individuals below 40 years of age, with a slight female sex predisposition 2 8–10. Various pathogens have been proposed to elicit KFD through a hyperimmune response to infection, but most studies have failed to establish a proven causative infectious agent.…”

“…Corticosteroid therapy could be beneficial in severe cases of KFD with extranodal involvement, patients with prolonged symptoms, or in cases where the disease recurs. There are some reports of prompt improvement in response to hydroxychloroquine and intravenous immunoglobulin 2–4 17…”

“…Notably, serum hepatic transaminase concentrations were normal at admission to the hospital, but increased during the recovery phase from day 10, peaking at 442 U/L (GPT) and 200 U/L (GOT). Transient elevations in serum transaminases have been reported during KFD and are considered to be a ‘para-inflammatory’ phenomenon associated with a reactive hepatocellular inflammatory response 2 7. No new symptoms developed in the follow-up period subsequent to discharge, consistent with the diagnosis of KFD.…”

“…The aetiology of KFD remains a subject for debate. It has been attributed as a hyperimmune or autoimmune response to an infectious agent; an underlying genetic predisposition, associated with expression of specific human leucocyte antigen (HLA) type II allele variants has also been proposed 1 2…”

Comparison of Clinical Characteristics and Risk Factors for Recurrence of Kikuchi–Fujimoto Disease Between Children and Adult

“…Other symptoms include rashes, arthritis, and fatigue. 1 In KFD, lymph node biopsy usually reveals lymph node necrosis and histiocytic infiltration, which are used as the basis for diagnosis. 2 , 3 KFD is differentiated from other serious conditions (such as lymphomas, systemic lupus erythematosus [SLE], and tuberculosis) by analyzing the patient’s clinical manifestations, laboratory findings, and imaging or pathological findings.…”