Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature

Abstract

Background: Kikuchi-Fujimoto Disease (KFD), also known as Histiocytic Necrotizing Lymphadenitis, is a rare cause of prolonged cervical lymphadenopathy in both the pediatric and adult populations. It was first reported in Japan in 1972, and since, this disease has been described worldwide, although most cases have been reported in Asia. The etiology of KFD is not fully understood, although there are 2 theories that are described in detail in this review. Kikuchi-Fujimoto Disease typically follows a benign course, with resolution of the lymphadenopathy within 6 months. It is important to recognize KFD as a cause of persistent lymphadenopathy, as it shares many characteristics with and must be differentiated from other causes of lymphadenopathy, including lymphoma, inflammatory disorders, autoimmune conditions, and infectious causes of lymphadenopathy. Case presentation: Here is presented a case of an 11-year-old male who was born in Vietnam, but subsequently adopted and raised in the United States, who presented to a private practice community-based Otolaryngology group. His chief complaint was a persistent neck mass of approximately 3 months duration. He underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD. Conclusion: The purpose of this article is not only to review the literature but also to contribute awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general Otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically Pediatricians, Infectious Disease Physicians, Rheumatologists, Pathologists, and Medical Oncologists.

Case Report of Kikuchi-Fujimoto Disease from Sub-Saharan Africa: An Important Mimic of Tuberculous Lymphadenitis

“…e sex ratio is variable in the pediatric age group with males affected more than females, which is reversed in adults [7]. e exact pathophysiology of KFD is not known, but the two theories postulated are infectious and autoimmune etiology [8]. Xu et al identified CMV,EBV, HHV-6, HHV-7, Parvovirus B19, and mycobacterial species from lymph node samples of 153 patients in China with KFD [9].…”

Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome – literature review based on a case report

“…Diagnosis is based on histopathological analysis with characteristic single or multiple areas within the Lymphadenopathy is also commonly caused by viruses (including, among others, EBV, CMV, HIV, rubella, rhinovirus and adenovirus), toxoplasmosis and various fungal infections [9,10,[21][22][23][24].…”

“…An estimated 3% to 4% of patients may experience recurrent episodes. Patients usually do not require treatment, except those with persistent fever or lymph node tenderness [24]. For symptomatic control NSAIDs are recommended.…”

“…Taking into consideration patients who were first diagnosed with KFD, there has been a higher recurrence rate than reported in the literature (3-4%). The literature search also revealed that patients with recurrences of KFD were more susceptible to autoimmune disease development [16,24].…”

Kikuchis sykdom

“…De hyppigste symptomene er lymfeglandelsvulst (100 %), feber (35 %), utslett (10 %), artritt (7 %), utmattelse (7 %) og hepatosplenomegali (3 %) (1). Sykdommen imiterer flere alvorlige tilstander og diagnostiseres ved typisk histopatologisk mønster i lymfeknutebiopsi og utelukking av andre årsaker til glandelsvulst, spesielt lymfom og systemisk lupus erythematosus (2). En studie fant ved regransking av lymfeknutebiopsier at 30 % av tilfellene av Kikuchis sykdom var feildiagnostisert som lymfom (3).…”