Kikuchi-Fujimoto Disease: A Case Report and Literature Review

Abstract

Case. 38-year-old lady was referred to the ENT clinic with history of right-sided facial pain, otalgia, and odynophagia. Clinical examination revealed enlarged right-sided lymph nodes in the neck. Further radiological scans showed a mass near the carotid and enlarged level V lymph nodes. Lymphoma was initially suspected. Fine-needle aspiration and excision biopsy were undertaken. Histological analysis later suggested Kikuchi-Fujimoto disease, also known as histiocytic necrotising lymphadenitis. Literature Review. Kikuchi-Fujimoto disease (KFD) was described in 1972 as lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris. KFD, frequently found in East Asian countries, is rare in the UK. No definite aetiology of KFD is known despite autoimmune and infection factors being suggested. The diagnostic hallmark is histological findings from lymph nodes. Malignancy should be excluded. This condition is mainly self-limiting; hence, management is limited to supportive care. Steroid therapy could be used in severe cases. KFD is relatively unknown in the UK and this case report aims to highlight its occurrence in our population.

The Enigmatic Kikuchi-Fujimoto Disease: A Case Report and Review

“…The disease is more prevalent in Asian populations. This geographic predominance may be related to the presence of certain HLA alleles such as HLA class II alleles, HLA-DPA1 and HLA-DPB1 , which are more prevalent in Asian KFD patients [ 7 ]. These genes are extremely rare or absent among Caucasians [ 1 ].…”

“…Infectious agents such as Yersinia enterocolitica , Brucella , Bartonella henselae , Entamoeba histolytica , Mycobacterium szulgai , and Toxoplasma gondii were implicated but subsequent studies failed to support these findings [ 7 , 10 ]. Viruses such as Epstein-Barr virus, herpes virus, cytomegalovirus, parvovirus, paramyxovirus, parainfluenza virus, Rubella, hepatitis-B, human immunodeficiency virus (HIV), human T-lymphotropic virus type-1 (HTLV-1), and the Dengue virus have all been suggested as probable etiologies of KFD, but never convincingly demonstrated [ 7 ]. On the other hand, some authors hypothesized that KFD may reflect a self-limited autoimmune condition induced by virus-infected transformed lymphocytes.…”

“…They also have similar histological feature leading to speculation that KFD is a self-limited, SLE-like autoimmune condition caused by virus-infected transformed lymphocytes [ 10 ]. However, the association of KFD with SLE remains unclear [ 7 ].…”

Kikuchi-Fujimoto Disease: A Rare Cause of Fever in the Returning Traveller

“…The aetiology of KFD remains unknown. The geographic predominance in Asian countries may be related to the presence of the HLA class II alleles, HLA-DPA1 and HLA-DPB1 , which are prevalent in Asian KFD patients but extremely rare among Caucasians [ 11 ], an observation perhaps reflective of a genetic predisposition to the disease. An infectious cause, or at least trigger, to KFD has been postulated.…”

Kikuchi-Fujimoto Disease: A Rare Case Report Questioning the Diagnostic Utility of Fine Needle Aspiration Cytology

“…Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare cause of cervical lymphadenopathy affecting mainly young Asian women (1,2). Although many conditions or etiological agents such as autoimmunity or viral infections such as Epstein-Barr virus have been proposed, the etiology and pathogenesis remains unknown.…”