• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.6
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• pp.548-553
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• 2007

Kikuchi-Fujimoto disease is a rare disease first described in 1972 by Kikuchi and Fujimoto et al. The disease is described as a benign and unusual self-limiting histiocytic necrotizing lymphadenitis of unknown origin, which is characterized histologically by necrotic foci surrounded by histiocytic aggregates. is usually manifested with lymphadenopathy and high fever. This disease mostly affects young Asian women between 20 and 30 years of age and has rarely been reported in children. Main symptoms are indolent or light tender, enlarged lymph nodes in the neck area. The correct diagnosis requires the histologic examination of the lymph node. Kikuchi-Fujimoto disease is easily confused histologically and clinically with lymphoma and systemic lupus erythematosis histologically and clinically. Although it is an uncommon cause of fever of unknown origin, early recognition of KFD is very important and will minimize potentially harmful and unnecessary evaluations and treatments. We reported a case, a 23-year old man who had Kikuchi-Fujimoto disease with a literature review.

• Childhood Kidney Diseases
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• v.16 no.2
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• pp.138-141
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• 2012

Rituximab, a chimeric anti-CD20 IgG1 monoclonal antibody, has been used as a rescue therapy for steroid-dependent or refractory nephrotic syndrome. However, the adverse effects of rituximab are yet to be investigated. We report a case of a 9-year-old boy with steroid-dependent nephrotic syndrome who developed Kikuchi-Fujimoto disease after several cycles of rituximab therapy. Kikuchi-Fujimoto disease is a benign, self-limited necrotizing histiocytic lymphadenitis of unknown etiology. In the present case, Kikuchi-Fujimoto disease developed when the peripheral blood B-cell count of the patient was at nadir, and the lesion regressed slowly but spontaneously after recovery of the B-cell count. To our knowledge, although the pathologic diagnosis of Kikuchi-Fujimoto disease was unavailable, this is the first report of Kikuchi-Fujimoto disease with clinical diagnosis as a possible adverse effect of rituximab.

• Clinical and Experimental Pediatrics
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• v.52 no.5
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• pp.622-626
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• 2009

Kikuchi-Fujimoto disease was initially described as a self-limiting histiocytic necrotizing lymphadenitis in Japan in 1972, and is predominantly observed in women under the age of 30 year and in Asian populations. The pathogenesis is still poorly understood but is thought to include infections, and autoimmune and neoplastic diseases. The most common clinical manifestations are fever and painless cervical lymphadenitis. Diagnosis is based on the histopathological findings, characterized by focal necrosis in the paracortical region with abundant karyorrhexis, aggregates of atypical mononuclear cells around the zone of necrosis, absence of neutrophils and plasma cells, and usually intact lymph node capsule. There is no specific therapy for the condition, and aseptic meningitis can occur as one of the complications. Here, we report the case of a patient with Kikuchi-Fujimoto disease accompanied with aseptic meningitis, which may be confused as a case of tuberculous meningitis and lymphadenitis.

• Journal of Yeungnam Medical Science
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• v.38 no.3
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• pp.245-250
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• 2021

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome-HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m²/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.

• Clinical and Experimental Pediatrics
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• v.55 no.11
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• pp.445-448
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• 2012

Kikuchi-Fujimoto disease (KFD) is a benign self-limiting disease characterized by fever and lymphadenitis. The etiology and pathogenesis of KFD is unclear. However, two hypotheses have been suggested: a viral infection hypothesis and an autoimmune hypothesis. Several KFD patients with various types of autoimmune diseases have been reported, and these reports support the hypothesis for autoimmune pathogenesis of KFD. Here, we report the case of a 17-year-old female patient diagnosed with KFD and autoimmune thyroiditis. This case serves as additional evidence that the etiology of KFD is autoimmune origin.

• Pediatric Infection and Vaccine
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• v.30 no.1
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• pp.39-46
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• 2023

Kikuchi-Fujimoto disease (KFD) is an acute febrile disease that mainly involves histiocytic necrotizing lymphadenitis in children and young adults. Diagnosis of KFD is even more difficult if image-guided percutaneous biopsy is technically challenging. We present a case of clinically diagnosed KFD in an 11-year-old boy who presented with fever, abdominal pain, and mesenteric lymphadenopathy, resulting in a diagnostic challenge. Additionally, we conducted a systematic review, and our goal was to describe the spectrum of disease, therapy, and outcomes. We identified 15 cases of KFD with symptoms that mimicked mesenteric lymphadenitis. Reports from the Americas, Europe, and Asia were also included. Most patients were male, exhibited leukopenia and elevated inflammatory markers, and recovered without significant sequelae or complications. A high index of suspicion of KFD should be maintained in children presenting with prolonged fever and unusual manifestations, such as mesenteric lymphadenitis.

• Korean Journal of Bronchoesophagology
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• v.4 no.1
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• pp.127-131
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• 1998

Histiocytic necrotizing lymphadenitis(NHL), or Kikuchi-Fujimoto disease, is of unknown origin that causes persistently enlarged cervical lymph nodes unresponsive to antibiotic therapy, usually in young women. Symptoms of NHL include tender cervical adenopathy, fever, weight loss, and night sweats. Laboratory studies usually reveal leukopenia and increased erythrocyte sedimentation rate. Lymph node biopsy reveals areas with frank cell necrosis, karyorrhexis, and absence of plasma cells. NHL follows a benign course, but shoud be differentiated from other diseases such as inflammatory, granulmomatous and malignant lymphadenopathies. We describe . three cases of NHL with a review of the literature.

• Pediatric Infection and Vaccine
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• v.10 no.1
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• pp.123-126
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• 2003

Kikuchi disease(subacute necrotizing lymphadenitis), first reported by Kikuchi and Fujimoto in 1972, is a benign self-limiting illness characterized by fever, neutropenia and cervical lymphadenopathy and develops predominantly in young women, especially in Asia. The cause of Kikuchi disease is unknown, but postinfectious(virus or bacteria) hyperimmune reaction has been suggested. Few pediatric cases have been reported. We experienced a case of Kikuchi disease associated with Yersinia pseudotuberculosis infection in 12-year-old girl. After she was admitted with cervical lymphadenopathy and headache, followed by sustained fever with leukopenia. All symptoms and signs did not seem to be responded to antimicobial treatment. Open biopsy of cervical lymph node was performed and showed findings consistent with subacute necrotizing lymphadenitis. Serologic studies were all negative except for Y. pseudotuberculosis. Fever subsided and lymphadenopathy improved after administration of oral steroid for 1 week.

• Pediatric Infection and Vaccine
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• v.11 no.2
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• pp.170-175
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• 2004

Purpose : We experienced 7 patients with Kikuchi-Fujimoto disease(KFD) who had presented a prolonged fever and lymphadenopathy. The clinical and the laboratory aspects of the patients were analyzed. Methods : The medical records of the 7 KFD patients, who were confirmed by excisional lymph node biopsy from January 1996 through December 2003, were retrospectively analyzed. Results : The mean age of the children was $11.4{\pm}2.8$ years(ranging from 8 to 15 years). The male to female ratio was 1.3 : 1. The median duration of the fever prior to admission and the total duration of the fever were 12 days(ranging from 5 to 65 days) and 27 days(ranging 9 to 75 days), respectively. One patient had supraclavicular lymphadenopathy, 5 had cercical involvement, and 1 had axillary lymphadenopathy. All the histologic findings of the lymph nodes biopsies showed the characteristic findings consistent with KFD such as paracortical necrosis with karyorrhexis and an increase in the number of phagocytic histiocytes and atypical lymphocytes. As for the laboratory findings, leukopenia($3,800{\pm}700/mm^3$), anemia(hemoglobin, $11.0{\pm}1.2g/dL$), an elevated erythrocyte sedimentation rate($42{\pm}17mm/hr$), and a relatively low C-reactive protein level($1.3{\pm}0.9mg/dL$) were noted. Four patients received conservative therapy with antipyretics, and 3 patients were treated with prednisolone. Conclusion : KFD is a rare disease, yet should be considered in the differential diagnosis for older children with prolonged fever and lymphadenopathy.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.987-991
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• 2008

Purpose : Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a self-limited disease characterized by cervical lymphadenopathy and fever. The etiology of KFD remains unknown; however, the self-limiting nature of HNL suggests the cause of this disease could be viral infection. For this reason, several viruses have been evaluated as possible etiologies of HNL, including Epstein-Barr virus (EBV), human herpesvirus 6 (HHV6), human herpesvirus 8 (HHV8), and cytomegalovirus (CMV). The aim of this study was to examine the relationship of EBV and HHV6 to HNL. Methods : Data pertaining to 51 cases with biopsy-confirmed HNL were collected between January 1999 and December 2005, from the Department of Pathology, College of Medicine, Pusan National University, Busan, Korea. The clinical records-including data regarding age, gender, duration of fever, and lymph node involvementwere reviewed retrospectively. The in situ hybridization (ISH) assay was performed by EBER PNA probe (Dako, Capinteria, CA, USA), and immunohistochemistry testing was performed with anti-HHV type 6 monoclonal antibodies (Chemicon, Temecula, CA, USA). Results : The HNL patients in this study were 24 males and 27 females, ranging in age from seven to 61 years (median: 25.9). ISH for EBV was positive in 8/51 (15.7%) biopsies, and immunohistochemistry for HHV6 was positive in 15/51 (29.4%) biopsies. Serologic analysis of EBV IgM was performed in 23 cases; only one patient was positive for EBV IgM and EBV ISH. Conclusion : Our study could not provide supportive evidence of a viral pathogenesis for HNL; therefore, cases of HNL may not have a dominant viral cause. However, some rare exceptional cases may have been caused by viral infection.