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This study aims to to establish a diagnosis model based on simple clinical features for children with cervical histiocytic necrotizing lymphadenitis or malignant lymphoma. Simple clinical features of pediatric patients were analyzed to develop a diagnosis model based on a comparison of classical machine-learning algorithms. This was a single-center retrospective study in a tertiary pediatrics hospital. Pediatric patients treated for cervical histiocytic necrotizing lymphadenitis or malignant lymphoma treated at our institution in recent 5 years were included. Demographic data and laboratory values were recorded and binary logistics regression analysis was applied to select possible predictors to develop diagnostic models with different algorithms. The diagnostic efficiency and stability of each algorithm were evaluated to select the best one to help establish the final model. Eighty-three children were included with 45 cases of histiocytic necrotizing lymphadenitis and 38 cases of malignant lymphoma. Peak temperature, white blood cell count, monocyte percentage, and urea value were selected as possible predictors based on the binary logistics regression analysis, together with imaging features already reported (size, boundary, and distribution of mass). In the ten-round random testing sets, the discriminant analysis algorithm achieved the best performance with an average accuracy of 89.0% (95% CI 86.2–93.6%) and an average AUC value of 0.971 (95% CI 0.957–0.995). Conclusion: A discriminant analysis model based on simple clinical features can be effective in differential diagnosis of cervical histiocytic necrotizing lymphadenitis and malignant lymphoma in children. Peak body temperature, white blood cell count, and short diameter of the largest mass are significant predictors. What is Known: • Several multivariate diagnostic models for HNL and ML have been proposed based on B-ultrasound or CT features in adults. • The differences between children and adults are nonnegligible in the clinical featues of HNL. What is New: • The study firstly report a large-sample diagnostic model between the HNL and MLin pediatric patients. • Non-imaging clinical features has also been proven with quite good diagnostic value. [ABSTRACT FROM AUTHOR]

Abstract Objectives To characterize the clinical features and to identify the predictors of recurrence of histiocytic necrotizing lymphadenitis (HNL) in Chinese children. Study design This study retrospectively analyzed the clinical characteristics, laboratory and pathological findings, and recurrence status of children diagnosed with HNL at a single center in China from January 2018 to May 2023. Logistic regression analysis was employed to identify predictors of HNL recurrence. Results 181 Chinese children with histopathologically confirmed HNL were enrolled (121 males and 60 females). The mean age was 9.3 ± 2.9 years. The most prominent clinical features were fever (98.9%) and cervical lymphadenopathy (98.3%). Aseptic meningitis was the most frequent complication (38.5%), while hemophagocytic lymphohistiocytosis and autoimmune disease were rare (1.7% and 1.2%, respectively). Recurrence occurred in 12.7% of patients. Erythrocyte sedimentation rate (> 30 mm/h) was the significant predictors of HNL recurrence, with odds ratios of 6.107, respectively. Conclusion Our study demonstrates that fever and cervical lymphadenopathy are the most frequent clinical manifestations of HNL in Chinese children, which often coexist with aseptic meningitis. HNL patients with risk factors require follow-up for recurrence.

Background: Cervical lymphadenopathy is common in children and has diverse causes varying from benign to malignant, their similar manifestations making differential diagnosis difficult. Objective: This study aimed to investigate whether radiomic models using conventional magnetic resonance imaging (MRI) could classify pediatric cervical lymphadenopathy. Methods: A total of 419 cervical lymph nodes from 146 patients, and encompassing four common etiologies (Kikuchi disease, reactive hyperplasia, suppurative lymphadenitis and malignancy), were randomly divided into training and testing sets in a ratio of 7:3. For each lymph node, 1,218 features were extracted from T2-weighted images. Then, the least absolute shrinkage and selection operator (LASSO) models were used to select the most relevant ones. Two models were built using a support vector machine classifier, one was to classify benign and malignant lymph nodes and the other further distinguished four different diseases. The performance was assessed by receiver operating characteristic curves and decision curve analysis. Results: By LASSO, 20 features were selected to construct a model to distinguish benign and malignant lymph nodes, which achieved an area under the curve (AUC) of 0.89 and 0.80 in the training and testing sets, respectively. Sixteen features were selected to construct a model to distinguish four different cervical lymphadenopathies. For each etiology, Kikuchi disease, reactive hyperplasia, suppurative lymphadenitis, and malignancy, an AUC of 0.97, 0.91, 0.88, and 0.87 was achieved in the training set, and an AUC of 0.96, 0.80, 0.82, and 0.82 was achieved in the testing set, respectively. Conclusion: MRI-derived radiomic analysis provides a promising non-invasive approach for distinguishing causes of cervical lymphadenopathy in children. [ABSTRACT FROM AUTHOR]

Objectives: To characterize the clinical features and to identify the predictors of recurrence of histiocytic necrotizing lymphadenitis (HNL) in Chinese children. Study design: This study retrospectively analyzed the clinical characteristics, laboratory and pathological findings, and recurrence status of children diagnosed with HNL at a single center in China from January 2018 to May 2023. Logistic regression analysis was employed to identify predictors of HNL recurrence. Results: 181 Chinese children with histopathologically confirmed HNL were enrolled (121 males and 60 females). The mean age was 9.3 ± 2.9 years. The most prominent clinical features were fever (98.9%) and cervical lymphadenopathy (98.3%). Aseptic meningitis was the most frequent complication (38.5%), while hemophagocytic lymphohistiocytosis and autoimmune disease were rare (1.7% and 1.2%, respectively). Recurrence occurred in 12.7% of patients. Erythrocyte sedimentation rate (> 30 mm/h) was the significant predictors of HNL recurrence, with odds ratios of 6.107, respectively. Conclusion: Our study demonstrates that fever and cervical lymphadenopathy are the most frequent clinical manifestations of HNL in Chinese children, which often coexist with aseptic meningitis. HNL patients with risk factors require follow-up for recurrence. [ABSTRACT FROM AUTHOR]

Kikuchi–Fujimoto disease (KFD) is rare during pregnancy. It is characterized by necrotizing lymphadenitis and often occurs in young Asian women. We report a case of KFD during pregnancy, which was difficult to diagnose. A 37‐year‐old pregnant female (gestational week [GW] 7+5) was admitted to our hospital because of hyperemesis gravidarum. On the eighth day of hospitalization (GW 8+6), she suddenly developed a fever (38.0°C) with skin rash and posterior pharynx redness. Blood tests showed pancytopenia and abnormal liver function. The patient was misdiagnosed with severe Epstein–Barr virus infection and administered with prednisolone. Subsequently, cervical lymphadenopathy was observed, and biopsy results led to the diagnosis of KFD. Thereafter, her symptoms improved, and she was discharged at GW 13+4. KFD must be included as a differential diagnosis for patients with fever, abnormal liver function, and pancytopenia during pregnancy. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD, histiocytic necrotizing lymphadenitis) is a rare, benign disease in which the presenting clinical and radiological features often result in misclassification as a malignant process. We present the first report of concurrent KFD in the draining lymph nodes of a malignant phyllodes tumour, adding to the growing number of reports of KFD occurring in the context of malignancy, further compounding the existing diagnostic difficulties. An increased awareness of this condition with consideration for inclusion in the differential diagnosis of lymphadenopathy is required for improved diagnosis of this under-recognized entity.

Shengfang Bao, Hua Huang, Yingying Jin, Fei Ding, Zhen Yang, Xuemei Xu, Chenxi Liu, Xiqiong Han, Liping Wang, Jingyi Lu, Jiani Ma, Yanliang Jin Department of Rheumatology & Immunology, Shanghai Children’s Medical Center, School of Medicine, Shanghai Jiao Tong University, Shanghai, 200127, People’s Republic of ChinaCorrespondence: Yanliang Jin, Email jinyanliang@scmc.com.cnAbstract: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limiting condition characterized by lymph node inflammation. While KFD is rarely associated with ocular manifestations, our case report highlights bilateral optic neuritis in a 13-year-old male patient with KFD. We also provide a comprehensive review of similar cases in the literature.Keywords: Kikuchi-Fujimoto disease, optic neuritis, neuromyelitis optica spectrum disorder, vision deficiency

Kikuchi disease, also called, Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare self-limiting illness with an unknown etiology and pathogenesis. It is predominantly seen among young females. The cardinal clinical features include fever and cervical lymphadenopathy. Skin eruptions have also been reported. In Oman, two cases have been reported to date, in 2005 and 2020, with only one case exhibiting skin manifestations. There is a paucity of disease in our country and worldwide. In this case report, we discuss the diagnosis of Kikuchi disease in a previously healthy 17-year-old Omani female who presented with fever, cervical lymphadenopathy, and malar rash. The clinical picture in this case resembled that of systemic lupus erythematosus. Due to the rarity of Kikuchi disease, particularly in our region, it is crucial to consider it as a differential diagnosis when a patient exhibits the aforementioned symptoms to prevent misdiagnosis and inappropriate treatment, as it can easily be misdiagnosed as systemic lupus erythematosus. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder that usually presents with regional cervical lymphadenopathy and fever. We report a case of a 12-year-old female who complained of fever, night sweating, significant weight loss, and tender right cervical lymph node enlargement for 2 months. A full workup including laboratory tests and imaging studies, an excisional biopsy, and histopathological analysis were done, and the diagnosis of KFD was confirmed. The patient was treated with analgesia and oral prednisolone, resulting in good improvement. A high degree of clinical suspicion is imperative for physicians, given the rarity of the disease and the associated diagnostic challenges. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease of unknown etiology. The diagnosis of KFD is challenging as it shares a resemblance with many viral, autoimmune, and neoplastic diseases. KFD generally presents with histiocytic necrotizing lymphadenitis involving multiple cervical lymph nodes. The authors present a rare case report of a recurrent case of KFD in a 49-year-old female patient. Intravenous dexamethasone, hydroxychloroquine, and nonsteroidal anti-inflammatory drugs were used for controlling the initial symptoms, and later, oral methylprednisolone was used as a maintenance therapy. Intravenous dexamethasone proved to be an effective drug in the treatment of KFD as it helps in faster recovery.

Abstract Histiocytic necrotizing lymphadenitis (HNL) is a benign, self-limiting disease that is rare clinically. The coexistence of HNL and tumor is rarer. We report a male patient who was preoperatively diagnosed with papillary thyroid carcinoma with cervical lymph nodes metastasis, and the postoperative pathological examination showed histiocytic necrotizing lymphadenitis combined with metastatic papillary thyroid carcinoma in the same single lymph node. More interestingly, Epstein‒Barr virus was positive in these lymph nodes by in situ hybridization. This may suggest a trigger for the coexistence of the two diseases.

Background: Histiocytic necrotizing lymphadenitis (HNL) is a self-limited inflammatory disease of unknown pathogenesis. A very small fraction of patients with HNL could develop hemophagocytic lymphohistiocytosis (HLH), a hyperinflammatory disorder. These patients are diagnosed as HNL with HLH (HNL-HLH). HNL-HLH in the pediatric population has been systemically studied, however, the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH remain to be explored. We aimed to explore the clinical, laboratory, and radiological features and outcomes of adult patients with HNL-HLH. Methods: We collected the clinical data of patients with HNL-HLH admitted to the First Affiliated Hospital of Nanjing Medical University from October 2010 to June 2015. All the patients underwent lymph node biopsy and have a pathological diagnosis of HNL. The age, gender, clinical presentation, lymph node signs, laboratory findings and imaging data, and pathological findings of the patients were collected. Results: In this study, we reported five adult patients with HNL-HLH. All five patients showed enlarged lymph nodes and prolonged fever. Laboratory findings were consistent with the diagnosis of HLH. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed enlarged lymph nodes with increased FDG uptake and splenic hypermetabolism could be present. All the patients responded well to corticosteroids and had a good prognosis. Two of the five patients were diagnosed with systemic lupus erythematosus during the follow-up. Conclusions: Our study demonstrated that adult patients with HNL-HLH showed distinct clinical, laboratory, and radiological features. And the prognosis is good and patients could be managed with steroids and supportive care. [ABSTRACT FROM AUTHOR]

Histiocytic necrotizing lymphadenitis (HNL) is a benign, self-limiting disease that is rare clinically. The coexistence of HNL and tumor is rarer. We report a male patient who was preoperatively diagnosed with papillary thyroid carcinoma with cervical lymph nodes metastasis, and the postoperative pathological examination showed histiocytic necrotizing lymphadenitis combined with metastatic papillary thyroid carcinoma in the same single lymph node. More interestingly, Epstein‒Barr virus was positive in these lymph nodes by in situ hybridization. This may suggest a trigger for the coexistence of the two diseases. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease of unknown etiology. The diagnosis of KFD is challenging as it shares a resemblance with many viral, autoimmune, and neoplastic diseases. KFD generally presents with histiocytic necrotizing lymphadenitis involving multiple cervical lymph nodes. The authors present a rare case report of a recurrent case of KFD in a 49-year-old female patient. Intravenous dexamethasone, hydroxychloroquine, and nonsteroidal anti-inflammatory drugs were used for controlling the initial symptoms, and later, oral methylprednisolone was used as a maintenance therapy. Intravenous dexamethasone proved to be an effective drug in the treatment of KFD as it helps in faster recovery. [ABSTRACT FROM AUTHOR]

Kikuchi disease, also called, Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare self-limiting illness with an unknown etiology and pathogenesis. It is predominantly seen among young females. The cardinal clinical features include fever and cervical lymphadenopathy. Skin eruptions have also been reported. In Oman, two cases have been reported to date, in 2005 and 2020, with only one case exhibiting skin manifestations. There is a paucity of disease in our country and worldwide. In this case report, we discuss the diagnosis of Kikuchi disease in a previously healthy 17-year-old Omani female who presented with fever, cervical lymphadenopathy, and malar rash. The clinical picture in this case resembled that of systemic lupus erythematosus. Due to the rarity of Kikuchi disease, particularly in our region, it is crucial to consider it as a differential diagnosis when a patient exhibits the aforementioned symptoms to prevent misdiagnosis and inappropriate treatment, as it can easily be misdiagnosed as systemic lupus erythematosus.

Immune checkpoint inhibitors have become one of the effective means of solid tumor treatment, among which anti-programmed death-1 (PD-1) antibodies are more maturely applied and can effectively inhibit tumor immune escape, thus enhancing the anti-tumor effect, but it can also lead to a series of immune-related adverse events (irAEs) in the process of clinical use. Here, we report a Patient with pancreatic solid pseudopapilloma treated with Sintilimab for the fifteenth cycles who developed chills, fever, and lymph node enlargement. Considering that the patient did not have infection, without history of autoimmune disease, we diagnosed the patient with Sintilimab-induced histiocytic necrotizing lymphadenitis (Kikuchi disease). The symptoms are alleviated after rapid use of glucocorticoids. Histiocytic necrotizing lymphadenitis (Kikuchi lymphadenitis) with anti-programmed death-1 (PD-1) antibody is a rare immune-related adverse events (irAEs).

Abstract Background Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. The diagnosis of KFD can be challenging for nonspecific symptoms, laboratory or imaging findings. In this study, we aimed to describe the clinical manifestations of patients with KFD and to access the potential role of serum cytokines in the diagnosis of this disease. Methods Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children’s Hospital. Clinical data were collected from inpatient or outpatient medical records. Serum cytokines were detected by the Flowcytomix technique. Serum levels of cytokines were compared between patients with KFD and SJIA, or patients with KFD and KD. The data of patients without MAS were further analyzed. A receiver operating characteristic (ROC) curve analysis was further performed to access the potential role of serum cytokines in the diagnosis of KFD. Results Serum cytokines were detected in 25 (43.8%, 25/57) patients with a histological diagnosis of KFD. Compared to SJIA or KD patients, the KFD group had a significantly higher IFN-γ/IL-6 ratio and much lower levels of serum IL-6. The median level of serum IFN-γ in KFD was 41.65 pg/ml (range, 21.04–70.74 pg/ml), which was much higher than that in SJIA (median: 3.33 pg/ml, p = 0.16) or KD (median: 2.6 pg/ml, p = 0.01). After excluding patients with MAS, there was statistical significance in all comparisons of serum IFN-γ, IFN-γ/IL-6 ratio, and serum IL-6. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from SJIA were > 8.48 pg/ml, 0.45, respectively. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from KD were > 8.56 pg/ml, 0.45, respectively. The specificity of all those cutoff values for differentiating KFD from SJIA or KD was ≥ 94.7%. Conclusions For patients with fever of unknown etiology and lymphadenopathy, after excluding HLH or MAS, serum IFN-γ > 8.56 pg/mL and IFN-γ/IL-6 ratio > 0.45 may highly suggest the diagnosis of KFD; serum IL-6 > 50.45 pg/mL indicates that the probability of KFD may be small, and sJIA, KD, and acute infection should be excluded first.

Abstract The clinical data of patients with Kikuchi–Fujimoto disease (KFD) combined with the involvement of the central system is limited, particularly for children. This study aimed to investigate the clinical features and prognosis of KFD associated with the central nervous system in children. We collected the clinical data of patients diagnosed with KFD combined with the involvement of the central system, hospitalized at Children's Hospital of Chongqing Medical University (CHCMU). Furthermore, we summarized the clinical features and outcomes in children by reviewing the published literature. Together with the two patients from CHMCU enrolled in this study, we identified 19 children diagnosed with KFD combined with the involvement of the central nervous system, including eight males and 11 females. The onset age ranges from 6 to 18 years (the median age was 14 and the interquartile range was 9–16). Sixteen patients were diagnosed with aseptic meningitis. Headache (78.9%, 15/19) was the most common symptom. Cerebrospinal fluid pressure was increased in 3 patients (60.0%, 3/5), cell count was elevated in 14 patients (73.7%, 14/19), glucose was decreased in 4 patients (21.0%, 4/19), and protein was elevated in 12 patients (63.1%, 12/19). A total of 9 patients had cranial imaging changes. Electroencephalogram (EEG) was performed on seven patients and 4 patients presented abnormal EEG (57.1%, 4/7). A total of 12 patients received steroid therapy; two were combined with antiepileptic treatment. In children, KFD complicated with the involvement of the central nervous system was rare and aseptic meningitis was the primary manifestation. Most patients had a good prognosis and a few had residual sequelae of neurological damage.

Background: Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. The diagnosis of KFD can be challenging for nonspecific symptoms, laboratory or imaging findings. In this study, we aimed to describe the clinical manifestations of patients with KFD and to access the potential role of serum cytokines in the diagnosis of this disease. Methods: Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children's Hospital. Clinical data were collected from inpatient or outpatient medical records. Serum cytokines were detected by the Flowcytomix technique. Serum levels of cytokines were compared between patients with KFD and SJIA, or patients with KFD and KD. The data of patients without MAS were further analyzed. A receiver operating characteristic (ROC) curve analysis was further performed to access the potential role of serum cytokines in the diagnosis of KFD. Results: Serum cytokines were detected in 25 (43.8%, 25/57) patients with a histological diagnosis of KFD. Compared to SJIA or KD patients, the KFD group had a significantly higher IFN-γ/IL-6 ratio and much lower levels of serum IL-6. The median level of serum IFN-γ in KFD was 41.65 pg/ml (range, 21.04–70.74 pg/ml), which was much higher than that in SJIA (median: 3.33 pg/ml, p = 0.16) or KD (median: 2.6 pg/ml, p = 0.01). After excluding patients with MAS, there was statistical significance in all comparisons of serum IFN-γ, IFN-γ/IL-6 ratio, and serum IL-6. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from SJIA were > 8.48 pg/ml, < 47.42 pg/ml, and > 0.45, respectively. The cutoff values of serum IFN-γ, IL-6, and IFN-γ/IL-6 ratio for differentiating KFD from KD were > 8.56 pg/ml, < 50.45 pg/ml, and > 0.45, respectively. The specificity of all those cutoff values for differentiating KFD from SJIA or KD was ≥ 94.7%. Conclusions: For patients with fever of unknown etiology and lymphadenopathy, after excluding HLH or MAS, serum IFN-γ > 8.56 pg/mL and IFN-γ/IL-6 ratio > 0.45 may highly suggest the diagnosis of KFD; serum IL-6 > 50.45 pg/mL indicates that the probability of KFD may be small, and sJIA, KD, and acute infection should be excluded first. [ABSTRACT FROM AUTHOR]

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a benign, idiopathic, self-limiting, and systemic disorder involving lymph nodes (LNs) of unknown etiology. The first case was reported in Japan, with an increasing incidence globally. The disease is a challenge because of the nonspecific clinical features, and the disease is easily mistaken for other forms of lymphadenitis leading to misdiagnosis and mistreatment. It is associated with many autoimmune disorders, of which systemic lupus erythematosus (SLE) is the most common association. The diagnosis is based on the histological examination of LNs, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 24-year-old pregnant Indian female patient without any relevant past medical history to demonstrate the correlation between KFD and SLE complicated with autoimmune hemolytic anemia. [ABSTRACT FROM AUTHOR]

Abstract Background Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS. Methods Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children’s Hospital. Clinical data were collected from inpatient or outpatient medical records. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis. Results Of 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p 679 IU/mL, respectively. Conclusions The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD. Trial registration Not applicable; this was a retrospective study.

Jee Yeon Baek,1 Ji-Man Kang,1,2 Ji Young Lee,1 Sung Min Lim,1 Jong Gyun Ahn1,2 1Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea; 2Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, KoreaCorrespondence: Jong Gyun Ahn, Department of Pediatrics, Severance Children’s Hospital, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Korea, Tel +82-2-2228-2057, Fax +82-2-393-9118, Email JGAHN@yuhs.acPurpose: Kikuchi–Fujimoto disease (KFD) is a rare, benign, and self-limited disease, characterized by cervical lymphadenopathy and fever. Herein, we analyzed the differences in its clinical manifestations and risk factors for recurrence between children and adults.Patients and Methods: We retrospectively reviewed the medical records of patients diagnosed with KFD at a tertiary referral hospital between 2005 and 2019. Patients were divided into two groups based on their age: children (< 19 years) and adults (≥ 19 years).Results: During the 14-year study period, 127 patients were diagnosed with KFD. Among these, 34 (26.8%) were children and 93 (73.2%) were adults. The fever duration was longer and the frequency of myalgia was higher in adults than in children; however, no other significant symptomatic differences were noted between the two groups. Lymph node evaluation was mainly performed using ultrasound in children (61.8%) and computed tomography in adults (78.5%). Moreover, the frequency of antibiotic use was higher in children than in adults (76.5% vs 54.8%, P = 0.027). In adults, multivariable logistic regression analysis revealed anti-nuclear antibody (ANA) positivity (titer ≥ 1:80) as a risk factor for recurrence (odds ratio: 7.813; 95% confidence interval = 1.818– 33.333; P = 0.006).Conclusion: The clinical features of KFD in children and adults were similar; however, the preferred imaging study and frequency of antibiotic use differed significantly between the two groups. Furthermore, in adults, ANA positivity was associated with KFD recurrence. Thus, patients with KFD who present with ANA positivity at diagnosis will benefit from a regular follow-up for monitoring KFD recurrence.Keywords: histiocytic necrotizing lymphadenitis, recurrence, child, adult

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Introduction: Fever with mediastinal lymphadenopathy warrants evaluation for various infective and noninfective causes. Kikuchi--Fujimoto disease (KFD) is a rare disorder of lymph node enlargement which is also known as histiocytic necrotizing lymphadenopathy. Case Report: We present the case report of a 35-year-old female presenting with fever and significant weight loss and the evaluation of the case up to the conclusive diagnosis of KFD. Discussion: KFD generally involves young females. The diagnosis can be confirmed by histopathological examination of lymph node which also classifies the stage of the disease. The treatment of KFD is generally conservative and it is a self-limiting disease. [ABSTRACT FROM AUTHOR]

Background: Kikuchi-Fujimoto disease (KFD) is typically a benign, self-limiting inflammatory disease. However, some patients may have a prolonged or recurrent disease course, or present with life-threatening complications such as macrophage activation syndrome (MAS). In this study, we aimed to describe the incidence and clinical features of MAS in KFD and to access potential laboratory markers for the diagnosis of KFD-associated MAS. Methods: Patients with KFD were retrospectively enrolled from January 2015 to November 2021 at Shenzhen Children’s Hospital. Clinical data were collected from inpatient or outpatient medical records. Data collected included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. Data were analyzed using GraphPad Prism 8.0 statistical software (GraphPad Software Inc., La Jolla, CA, USA). A receiver operating characteristic (ROC) curve analysis was further performed to access the potential predictors for the KFD-MAS diagnosis. Results: Of 58 patients with a histological diagnosis of KFD, 15 (25.9%) patients had MAS. Compared to patients without MAS, patients with KFD-MAS presented with a higher proportion of skin rash (26.7%, p = 0.01), glucocorticoid treatment (80%, p = 0.003), and disease recurrence (33.3%, p = 0.04). KFD-MAS patients had lower absolute peripheral white blood cell (WBC, p = 0.02), platelet (p = 0.002), serum albumin levels (p = 0.01), and lymphocyte count (p < 0.0001), and higher lactate dehydrogenase (LDH) levels (p < 0.0001). ROC curve analysis showed that the cutoff values of absolute lymphocyte count, an absolute platelet count, serum albumin level, and serum LDH level for KFD-MAS diagnosis were < 1235/μL, < 171 × 106/μL, < 35.6 g/L, and > 679 IU/mL, respectively. Conclusions: The presence of KFD-MAS in children may be more common than previously expected, especially in those with skin rash. KFD-MAS may be associated with a higher recurrence rate. An extremely elevated serum LDH level and moderate to severe lymphopenia may be useful diagnostic markers for MAS in KFD. Trial registration: Not applicable; this was a retrospective study. [ABSTRACT FROM AUTHOR]

Severe fever with thrombocytopenia syndrome (SFTS) is a severe systemic inflammatory response syndrome caused by a potentially lethal tick-borne SFTS virus. A rapid and robust extrafollicular B-cell response to fatal or sublethal SFTS virus infection is known to generate a robust extrafollicular B-cell response that is skewed to lambda-monotypic B-blasts. We present a case of fatal SFTS virus infection-associated histiocytic necrotizing lymphadenitis showing an effaced nodal architecture due to florid lambda-monotypic B-blasts that can raise a diagnostic pitfall in hematopathology. We examined histologic and immunophenotypic features of inguinal lymph node tissue of a 49-year-old Korean woman, who presented with a high fever and painful left inguinal lymphadenopathy associated with fatal SFTS virus infection. The nodal architecture of the excised lymph node appeared to be diffusely effaced by expanded paracortical zones by an extensive proliferation of immunoblast-like large lymphoid cells along with minor foci of necrosis associated with infiltration of phagocytosing histiocytes, featuring the proliferative phase of Kikuchi-Fujimoto disease. Immunophenotypically, however, the proliferating cells consisted predominantly of lambda-monotypic B-blasts. This misleading aberrant B-cell response should be recognized by pathologists to avoid misinterpretation of the lambda-monotypic B-blast proliferation in SFTS viral infection-associated histiocytic necrotizing lymphadenitis as diffuse large B-cell lymphoma. [ABSTRACT FROM AUTHOR]

Kikuchi Fujimoto Disease, originally discovered in 1972, is a rare lymphoproliferative disorder traditionally characterized by cervical lymphadenopathy, fevers, parotid gland enlargement, and several other nonspecific manifestations. Differentials include lymphoma, other viral diseases such as Epstein-Bar Virus, as well as other autoimmune conditions such as Systemic Lupus Erythematosus. Central nervous system involvement is exceptionally rare, with manifestations including meningitis as well as subdural effusions, as presented in this case. This review will summarize a case of a 24-year-old man with recurrent subdural effusions requiring intervention, subsequent relapse with abdominal lymphadenopathy, and possible IgG4 related disease. The background epidemiology, radiology, and potential pathophysiology will be reviewed.

Shan Tian,1,&ast; Bin Zhu,1,&ast; Youyou Tian,2 Junyuan Li,1 Cheng Peng1 1Department of Infectious Diseases, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430022, Hubei, People’s Republic of China; 2Department of Infectious Diseases, Taihe Hospital, Hubei Medical University, Shiyan, 442000, Hubei, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Cheng Peng, Department of Infectious Diseases, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1277 Jiefang Avenue, Wuhan, Hubei, People’s Republic of China, Email questionpc2005@163.comAbstract: Histiocytic necrotizing lymphadenitis (HNL) is a self-limiting inflammatory disease presenting with fever and cervical lymphadenopathy. However, no case of HNL with confirmed bacterial infection has been reported. A 38-year-old man was admitted to our hospital owing to an intermittent fever from 8 days. He was diagnosed with Graves hyperthyroidism 3 months prior and began taking methimazole tablets orally. Physical examination revealed superficial lymphadenopathy and goiter of the thyroid (grade II). Blood routine showed a decrease in peripheral blood cells, including significant reduction in leukocytes and platelets. A bone marrow culture identified a very rare pathogen, Cupriavidus pauculus, but the antibiotic effect of meropenem was unsatisfactory. Biopsy of the left cervical lymph node revealed HNL, and the patient had no fever after using glucocorticoids. This case report indicates that clinicians should be aware of the coexistence of HNL and bacterial infections, especially in patients with Graves hyperthyroidism.Keywords: histiocytic necrotizing lymphadenitis, leucopenia, Graves hyperthyroidism, Cupriavidus pauculus, glucocorticoid

Histiocytic necrotizing lymphadenitis (HNL) is an inflammatory disease of unknown etiology clinically characterized by painful lymphadenopathy. This study aimed to investigate the role of interferon (IFN)-α in the pathogenesis of HNL and the clinical significance of serum IFN-α levels for the diagnosis and monitoring of HNL disease activity. This study enrolled 47 patients with HNL and 43 patients with other inflammatory diseases that require HNL differentiation including malignant lymphoma (ML), bacterial lymphadenitis, and Kawasaki disease. Expression of IFN-stimulated genes (ISGs) and MX1 in the lymph nodes was measured by real-time quantitative reverse transcription polymerase chain reaction and immunofluorescence staining, respectively. Enzyme-linked immunosorbent assay was used to quantify serum cytokine levels. The results were compared with the clinical features and disease course of HNL. Patients with HNL had a significantly elevated ISG expression in the lymph nodes compared with those with ML. MX1 and CD123, a specific marker of plasmacytoid dendritic cells (pDCs), were colocalized. In patients with HNL, serum IFN-α levels were significantly elevated and positively correlated with disease activity. The serum IFN-α level cutoff value for differentiating HNL from other diseases was 11.5 pg/mL. IFN-α overproduction from pDCs may play a critical role in HNL pathogenesis. The serum IFN-α level may be a valuable biomarker for the diagnosis and monitoring of disease activity in patients with HNL. • Type 1 interferon signaling is enhanced in lymph nodes of patients with HNL. • IFN-α overproduction from pDCs may play a critical role in HNL pathogenesis. • The serum IFN-α level is a valuable biomarker for the diagnosis and monitoring of disease activity in patients with HNL. [ABSTRACT FROM AUTHOR]

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Background: Kikuchi-Fujimoto disease (KFD) is a rare, benign, and self-limited disease that presents with cervical lymphadenopathy and systemic symptoms. Histologic evaluation is often necessary to differentiate KFD from other entities. Methods: Electronic medical records and diagnostic material were reviewed for 14 children diagnosed with KFD and 6 children diagnosed with infectious mononucleosis (IM) from 2013-2021. Four cases of KFD were further characterized using targeted DNA-based next-generation sequencing. Results: Systemic symptoms were present in 86% (n = 12/14) of KFD patients, the most common being fever. Laboratory values worrisome for malignancy included cytopenia(s) (n = 9/12), elevated ESR and/or CRP (n = 9/12), elevated ferritin (n = 7/7), and elevated LDH (n = 7/10). Histologically, lymph nodes showed characteristic necrotic foci without neutrophils surrounded by MPO+ "crescentic" histiocytes. Immunoblasts and CD123+ plasmacytoid dendritic cells (pDCs) were also increased surrounding the necrosis. IM lymph nodes showed similar features when necrosis was present but increases in pDCs were patchy and rare neutrophils were seen in the necrotic foci. Molecular analysis of 4 KFD cases did not identify pathogenic variants. Conclusion: While the signs/symptoms of KFD are worrisome, there are pathologic features that help differentiate it from potential mimics. We did not identify characteristic molecular features to aid in the work-up of these cases. [ABSTRACT FROM AUTHOR]

Histiocytic necrotizing lymphadenitis (HNL), also known as Kikuchi disease, is a relatively rare sub-acute necrotic localized lymphadenitis. This benign, self-limiting condition is characterized by fever, painful lymphadenopathy, skin rash, and other systemic symptoms. Due to its nonspecific presentation, unclear etiology, and pathogenesis, HNL has a low incidence rate in clinical practice. Insufficient awareness among clinicians and pathologists can easily lead to misdiagnosis. This article reported a 27-year-old female patient who was admitted to the hospital with fever, neck pain, and enlarged lymph nodes in the neck. There were no special medical or personal histories, and postoperative pathology confirmed tissue necrotizing lymphadenitis. After treatment with steroids and symptomatic therapy, she recovered and was discharged from the hospital. Follow-up to date has shown no recurrence., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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Background: Kikuchi-Fujimoto disease (KFD) is a rare, benign condition of unknown etiology, presenting as cervical lymphadenitis. Lymphadenopathy is usually tender and maybe associated with systemic symptoms. Despite the extensive literature on this disease, it continues to be misdiagnosed owing to its misleading clinical presentation. Methods: A retrospective hospital-based descriptive cross-sectional study was conducted in tertiary care hospitals from 2011 to 2019. All patients with confirmed KFD diagnosis were included and after ethics committee approval the clinical details and histopathological data was retrieved from the medical records department and analyzed. Results: A total of 67 cases were included. The mean age was 26.9±11.3 years with a female: male ratio of 1.9:1. There were 50 patients with tender cervical lymphadenopathy which was the most common clinical presentation. The mean length and width of palpable lymph nodes were 2.3±1.0 cm and 2.2±0.7 cm respectively. Histology revealed proliferative stage in majority of patients ( n=40, 59.7%). Lymphadenopathy resolved in 83.6% within 2 months. There were 42 patients who had complete recovery with symptomatic treatment within a period of 9 months. Conclusions: KFD is prevalent in young, female patients of Asian descent and often presents as tender cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is symptomatic unless complicated, where steroid therapy is considered. KFD has an excellent prognosis with almost no risk of fatality.

Objective: The aim of this study is to present six cases of cervical lymphadenopathy, diagnosed as Kikuchi's disease and raise the index of suspicion for this disease. Study Design: Case Series. Setting: Histopathology and Otolaryngology/Head & Neck Surgery Department at Rehman Medical Institute (RMI) Peshawar. Period: July 2016 to March 2021. Material & Methods: Records of patients for cases diagnosed as "histiocytic necrotising lymphadenitis" were retrieved, their corresponding patient files accessed from the archive, and case notes analyzed. Results: In this series, a total of six cases of Kikuchi's disease are presented. A female predominance is evident from the present data (2:1 female to male ratio). All patients presented with palpable lymph nodes in the neck, predominantly on the left (66.7%). Majority (66.7%) presented with neuralgic pain (on/off). Only one patient did not show weight loss and night sweating, the remaining had all the symptoms. All of the patients recovered spontaneously within 6 months (range 2 to 6 months) with symptomatic treatment for neuralgic pain. Conclusion: Kikuchi's disease should be kept in the differential diagnosis in young females with persistent neck masses, palpable lymph nodes and suspicious fine needle aspiration cytology (FNAC). [ABSTRACT FROM AUTHOR]

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Histiocytic necrotizing lymphadenitis (HNL) is a rare idiopathic disorder. It is frequently underdiagnosed due to its nonspecific clinical features, mimicking like lymphomas, autoimmune diseases, and infectious reactive lymphadenopathy. Histological examination of the lymph node is the basis of diagnosis in HNL. We report a 40-year-old male patient who presented with fever for 4 weeks. After an extensive workup, laboratory investigations revealed progressive pancytopenia, gradual increase in serum lactate dehydrogenase, and ferritin with negative pyrexia profile. His radiological imaging including positron emission tomography/computed tomography showed multiple intra-abdominal, cervical, and axillary lymphadenopathies. Finally, the histological section from axillary lymph node core biopsy revealed a diagnosis of HNL. Subsequent autoimmune workup met diagnostic criteria for systemic lupus erythematosus. This case report alerts the clinicians regarding this rare disease and emphasizes the important role of morpho-histopathology in early recognition of this rare entity.

Abstract The authors urge clinicians to consider the possibility of Kikuchi‐Fujimoto Disease associated with autoimmune hemolytic anemia with possible correlation with systemic lupus erythematosus in patients presented with lymphadenopathy and fever.

Background: Kikuchi-Fujimoto disease (KFD) is a rare, benign condition of unknown etiology, presenting as cervical lymphadenitis. Lymphadenopathy is usually tender and maybe associated with systemic symptoms. Despite the extensive literature on this disease, it continues to be misdiagnosed owing to its misleading clinical presentation. Methods: A retrospective hospital-based descriptive cross-sectional study was conducted in tertiary care hospitals from 2011 to 2019. All patients with confirmed KFD diagnosis were included and after ethics committee approval the clinical details and histopathological data was retrieved from the medical records department and analyzed. Results: A total of 67 cases were included. The mean age was 26.9±11.3 years with a female: male ratio of 1.9:1. There were 50 patients with tender cervical lymphadenopathy which was the most common clinical presentation. The mean length and width of palpable lymph nodes were 2.3±1.0 cm and 2.2±0.7 cm respectively. Histology revealed proliferative stage in majority of patients (n=40, 59.7%). Lymphadenopathy resolved in 83.6% within 2 months. There were 42 patients who had complete recovery with symptomatic treatment within a period of 9 months. Conclusions: KFD is prevalent in young, female patients of Asian descent and often presents as tender cervical lymphadenopathy. Early diagnosis with excisional lymph node biopsy is crucial to avoid unnecessary investigations and treatment. Treatment is symptomatic unless complicated, where steroid therapy is considered. KFD has an excellent prognosis with almost no risk of fatality.

Introduction: Kikuchi-Fujimoto disease is considered a necrotizing histiocytic lymphadenitis of infrequent presentation. Its diagnosis is anatomopathological. In the current global context of the COVID-19 pandemic, this viral disease ranges from an asymptomatic condition to severe pneumonia, in addition to other atypical presentations. Patient information: white female, 34 years old, with health history and no toxic habits, who presented with fever between 38 and 390C, once or twice a day, without chills, which decreased with the administration of antipyretics. After the first 13 days of the onset of symptoms, the fever disappeared for six days and then reappeared with the same characteristics; for this reason it was decided to admit her to the hospital. The diagnosis of Kikuchi-Fujimoto disease was obtained after several complementary tests. Conclusions: the report of this case could announce the possible location of the new SARS-CoV-2 coronavirus among the etiologies of EKF. [ABSTRACT FROM AUTHOR]

Kikuchi disease is a type of benign, self-limiting necrotizing lymphadenitis that occurs most commonly in young women and usually manifests as palpable cervical lymph nodes and fever. Patients with an unusual location of lymph node involvement can be misdiagnosed with malignant disease. Here, we report a case of Kikuchi disease in a 15-year-old girl presenting with persistent fever for 2 weeks. Imaging studies, including ultrasonography, CT, and 18F-fluorodeoxyglucose PET/CT, revealed splenomegaly and enlarged lymph nodes in the neck, axilla, abdomen, retroperitoneum, and inguinal region. Laparoscopic excision of the celiac lymph nodes confirmed histiocytic necrotizing lymphadenitis, also known as Kikuchi disease. Conservative treatment with corticosteroids improved the patient’s condition.

Hyun Joo Jung,1 Il Jae Lee,2 Seung-Hyun Yoon3 1Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Gyeonggi-do, South Korea; 2Department of Plastic Surgery, Ajou University Hospital, Ajou University School of Medicine, Suwon, Gyeonggi-do, South Korea; 3Department of Physical Medicine and Rehabilitation, Ajou University Hospital, Ajou University School of Medicine, Suwon, Gyeonggi-do, South KoreaCorrespondence: Seung-Hyun Yoon Tel +82 31 219 5279Fax +82 31 219 5209Email yoonsh.md@gmail.comPurpose: Kikuchi disease (KD) is typically a benign disease. Recent studies reporting recurrence or serious cases suggest a possible association of KD with systemic autoimmune disorders. We performed a long-term analysis of the characteristics of KD in patients of all ages and assessed KD recurrence or progress to systemic autoimmune disorders.Patients and Methods: Electronic medical records of patients diagnosed with KD between April 1995 and May 2017 were reviewed for clinical and laboratory manifestations.Results: In total, 480 patients were confirmed to have KD based on histopathology findings. The mean age at KD diagnosis was 24.4 years. Recurrence occurred in 11.3% of patients; 2.7% developed autoimmune diseases after KD diagnosis. Patients who experienced recurrence had more extranodal symptoms, lymphopenia, and a longer lymphopenia-recovery duration. Patients who developed autoimmune diseases after KD were more likely to have extranodal symptoms, KD recurrence, and anti-nuclear antibody positivity.Conclusion: KD patients with risk factors need to be followed-up for KD recurrence and the development of systemic autoimmune diseases.Keywords: Kikuchi disease, histiocytic necrotizing lymphadenitis, recurrence, autoimmune disorders

Kikuchi-Fujimoto disease (KFD) is an infrequent benign self-limiting disease predominantly affecting young Asian women and has an excellent prognosis. Diagnosis of KFD is confirmed by characteristic immunohistology of lymph node biopsy revealing histiocytic necrotizing lymphadenitis with abundant CD 68+ plasmacytoid monocytes in the paracortex and karyorrhectic debris. SLE, in particular, has been reported to precede, occur simultaneously, or appear after KFD. Treatment modalities and clinical outcomes of both KFD and SLE are vastly different. Hence, it is imperative to make timely correct diagnoses of SLE and treat early to avoid multi-organ failure and potentially fatal outcomes. The case report is presented of a challenging case of a 26-year-old Asian male who was initially diagnosed with KFD, and subsequently met the diagnostic criteria for SLE. He was treated for SLE for 6 months and showed dramatic improvement symptomatically without any further recurrence.

Kikuchi-Fujimoto disease (KFD) is rare, and many infectious agents have been suspected for its etiology. This report presents an interesting case of KFD found with torque teno virus/torque teno minivirus (TTV/TTMV), which closely resembles the circovirus that causes necrotizing lymphadenitis in pigs. Three Korean patients showed several enlarged lymph nodes in their neck. Quantitative polymerase chain reaction (qPCR) and subsequent DNA sequencing for TTV/TTMV using formalin-fixed paraffin-embedded tissue were performed. Histologic examination demonstrated typical features of KFD. qPCR showed successful amplification of TTV/TTMV, and DNA sequencing confirmed the results. It is the first report of TTV/TTMV presence in three patients with KFD.

Kikuchi-Fujimoto disease is a very rare disease, basically involving young adults and ubiquitously distributed. It is characterized by fever and benign lymph node swelling. The distinguishing features of this disease are cervical lymphadenopathy, constitutional symptoms resembling tuberculosis, and its penchant to affect young people of Oriental or Asian descent, especially women. We describe an instance of a 42-year-old female who arrived with multiple neck swellings. On physical examination, there was palpable right-sided cervical lymphadenopathy, while laboratory investigations were essentially within normal limits except for raised erythrocyte sedimentation rate and anemia. After cefepime and nonsteroidal anti-inflammatory medications were administered, symptoms subsided, and lymphadenopathy receded in the patient. This case supports the importance of histological evaluation to reach an exact diagnosis and guide treatment and the need to consider Kikuchi-Fujimoto disease in the differential diagnosis of lymphadenopathy., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Sharma et al.)

Background: Little information exists regarding the differences in the clinical and laboratory characteristics of Kikuchi-Fujimoto disease (KFD) according to age.Objective: To evaluate the clinical and laboratory characteristics of KFD according to age.Methods: The relevance of sex, age, clinical features, laboratory findings, courses, and follow-up results were retrospectively evaluated in patients diagnosed with KFD at Pusan National University Hospital between 2010 and 2020.Results: Eighty patients (46 children and 34 adults) with a mean age of 21.5 ± 11.8 years (range, 3–49 years) were included in the study. Those aged 10–19 years accounted for the largest number of patients (42.5%). Among children, the male sex ratio was higher, especially for patients aged ≤ 9 years. In adults, the female sex ratio was higher, especially for patients aged 20–29 years. Fever, tenderness in the lymph node, and skin rashes were more common in children, while myalgia and weight loss were more common in adults. In children, the recurrence rate was significantly higher among boys than among girls (15.8 vs. 0.0%, P = 0.001); lower platelet count and higher CRP levels were observed among boys than among girls. EBV and ANA positivity rates were higher in boys than in girls. In adults, the recurrence rate was significantly higher in women than in men (18.2 vs. 0.0%, P = 0.005). ANA positivity rates were higher in women than in men.Conclusion: The clinical features, laboratory findings, and recurrence of KFD may differ depending on age and sex. Clinicians should be aware of this.

Background: Histiocytic necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease (KFD), is a self-limiting inflammatory disease with low incidence and high misdiagnosis rate in children. Furthermore, cases where the clinical presentation resembles acute appendicitis are very rare.Case Presentation: A 14-year-old boy was misdiagnosed as acute appendicitis and received operative treatment at his early visit. He suffered from abdominal pain, vomiting, diarrhea, fever, and lymphadenitis at the ileocecal junction, which were found by B-ultrasonography examination and surgery. Lymphadenectomy, as well as appendectomy, was performed, and KFD was identified by pathological examination. The patient was transferred to our hospital for further therapy because of recurrent fever and abdominal pain after the appendectomy. His temperature became normal after methylprednisolone was administered, and no recurrence was observed till now during follow-up.Conclusions: Necrotizing lymphadenitis involving mesenteric lymph nodes may cause acute-appendicitis-like symptom; KFD should be a diagnostic consideration for mesenteric lymphadenitis.