Cervical lymphadenopathy can be due to numerous causes. The common causes include reactive and infections conditions in children and malignancy in the elderly. Kikuchi-Fujimoto disease (KFD) is a rare cause of cervical lymphadenopathy. As viral vaccines contain viral antigens, they can trigger the development of KFD. The human papillomavirus (HPV) vaccine can trigger KFD. It is important to elicit a history of prior vaccination to identify the trigger in patients with necrotising histiocytic lymphadenitis suspected of having KFD. We hereby report a case of a 16-year-old female who was diagnosed with HPV vaccine induced KFD. Ours is the first case to be reported from India. Histopathology revealed necrotising histiocytic lymphoid hyperplasia and the absence of neutrophils, eosinophils, plasma cells, vessel wall vasculitis, haematoxylin bodies, and Reed-Sternberg cells, and negative aerobic, MTB cultures, anti-nuclear antibodies, clinched the diagnosis of KFD.

Kikuchi-Fujimoto Disease (KFD), also known as Kikuchi histiocytic necrotising lymphadenitis, is an extremely rare and benign condition that mostly affects young women. It is characterised by lymph node involvement with a predilection for the cervical region, commonly presenting with tender lymphadenopathy and a low-grade fever. The diagnosis requires excisional lymph node biopsy with immunohistochemical analysis. KFD is mostly self-limiting within a few weeks to months, with only some patients requiring symptomatic relief with non-steroidal anti-inflammatory drugs or corticosteroids, and a minority developing recurrent episodes of the disease. Importantly, it has been reported in association with systemic lupus erythematosus and, to a lesser extent, other immune-mediated inflammatory rheumatic diseases, such as Sjögren's syndrome, whose clinical presentation itself may include lymphadenopathy. In this paper, we present an unusual case of a woman with primary Sjögren syndrome (pSS) and a past medical history relevant for lymphoma, sarcoidosis, and thymoma, who later developed KFD, a particularly challenging diagnosis in this setting. We then performed a literature review of the association between KFD and pSS, gathering a total of 13 patients, and focusing epidemiological, clinical, and laboratory data.

Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting condition typically characterized by fever and lymphadenopathy. The exact etiology remains unclear but is suspected to be associated with viral infections and autoimmune responses. This report presents the case of a 32-year-old Chinese male who was admitted with recurrent high fever, lymphadenopathy, and hepatosplenomegaly. Initial treatment was ineffective, and a lymph node biopsy subsequently confirmed the diagnosis of KFD, with evidence of cytomegalovirus infection. Following treatment with corticosteroids, the patient's symptoms improved rapidly, and no relapse was observed during follow-up after discharge. This case highlights the diagnostic challenges of KFD, particularly in distinguishing it from lymphoma and systemic lupus erythematosus. Accurate and timely diagnosis is crucial to avoid unnecessary treatments, and long-term follow-up is recommended to monitor for potential disease progression.

We introduce a 16-year-old female who presented with tender cervical lymphadenopathy, prolonged fever, and hypothyroidism. After excluding common causes of fever of unknown origin, a surgical biopsy of cervical lymph nodes revealed Kikuchi-Fujimoto disease. The patient showed improvement with a short-term course of NSAIDs. An increased titre of thyroperoxidase antibody led to a diagnosis of Hashimoto's thyroiditis during stable condition. This report underscores the importance of considering Kikuchi-Fujimoto disease in the differential diagnosis of prolonged fever of unknown origin with lymphadenopathy and highlights the association with Hashimoto's thyroiditis, advocating for vigilance regarding hypothyroidism in long-term follow-up after Kikuchi-Fujimoto disease recovery.

Kikuchi-Fujimoto disease (KFD) is a rare benign condition associated with fever and lymphadenopathy and was first described by Kikuchi and Fujimoto independently in 1972 as histiocytic necrotizing lymphadenitis. The diagnosis is made by histopathology with immunohistochemistry. Limbic encephalitis is an extremely rare presentation of this uncommon disease, which has been described mainly in children. Available evidence is sparse in the form of case reports and case series in the form of 10 cases published till date. We report a case of an adult female with KFD with autoimmune limbic encephalitis, who had complete clinical and radiologic recovery with treatment, and a literature review of all the cases published till date.

Kikuchi-Fujimoto disease (KFD) is self-limiting, has an unknown origin, and predominantly affects the lymph nodes. KFD with aseptic meningitis is rare and diagnostically challenging. This retrospective observational study aimed to elucidate the clinical features and treatment outcomes of KFD, particularly in cases with aseptic meningitis.

We conducted this retrospective study to describe KFD to determine the characteristics of the disease, with a particular focus on cases involving aseptic meningitis.

Our study comprised 103 patients (33 men, 70 women) diagnosed with KFD at Peking University Third Hospital between January 2013 and March 2024. Diagnosis was based on histological examination of lymph node biopsies. The mean age was 25 (range: 16-66) years. Clinical manifestations included fever (100%), cervical pain (79.6%), fatigue (49.5%), headache (44.7%), myalgia (26.2%), and hepatosplenomegaly (23.3%). Biological signs included leukopenia (66.0%) and elevated lactate dehydrogenase (>250 U/L, 83.5%) and ferritin (>300 ng/mL, 44.6%) levels. Forty-three cases improved with nonsteroidal anti-inflammatory drugs (NSAIDs) as monotherapy, whereas 24 required corticosteroid therapy. Four of the 46 patients with headache underwent cerebrospinal fluid analysis, confirming aseptic meningitis. Notably, all four responded well to nonsteroidal anti-inflammatory drugs.

Our findings highlight the features and outcomes of KFD, particularly its association with aseptic meningitis, which has a favorable prognosis in the absence of corticosteroid therapy.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, self-limiting disorder characterized by fever typically lasting for 1 week up to 1 month and painful necrotizing lymphadenopathy, primarily affecting young adults of Asian ancestry. Although the exact cause remains unclear, infectious and autoimmune mechanisms have been implicated in the pathogenesis of the disease. In this case series, we aim to describe the histopathological features of KFD over a ten-year period at Mount Sinai Medical Center of Florida, and review the current understanding of its pathogenesis, clinical presentation, diagnosis, and management. A retrospective review of our pathology database between January 2013 and May 2024 was performed to identify patients diagnosed with KFD at our institution. Eight cases of KFD were identified, with a mean age of 35 years (range 24-49) and slight male predilection (5:3). Three patients exhibited leukopenia, and two had concurrent HIV infection. One patient developed systemic lupus erythematosus (SLE), and another developed IgA nephropathy during follow-up. Histopathological examination revealed the characteristic features of KFD, including lymph node architectural effacement, histiocytic infiltration, and necrosis. In conclusion, KFD remains a diagnostic challenge due to its overlapping clinical features with other infectious and autoimmune diseases, particularly SLE. While most cases resolve spontaneously, long-term follow-up is warranted due to the potential for recurrence and autoimmune associations.

Kikuchi-Fujimoto disease (KFD) is a rare self-limiting condition presenting as fever and cervical lymphadenopathy, with only two reported cases with isolated mediastinal lymphadenopathy. Lack of awareness about this condition often results in a high rate of misdiagnosis. We present a case of a 29-year-old Indian male with fever, mucocutaneous ulcers, weight loss, and mediastinal lymphadenopathy on CT. Extensive investigations ruled out malignancy and autoimmune and infectious causes. Endobronchial ultrasound-guided fine needle aspiration revealed necrotizing lymphadenitis, indicating KFD, followed by symptomatic management and reassurance. Awareness is crucial to avoid misdiagnosis and unnecessary treatment due to its potentially serious differentials. Long-term follow-up is imperative due to its association with autoimmune conditions.

Background Kikuchi-Fujimoto disease (KFD) is a benign lesion of the lymph nodes predominantly seen in younger women and is a condition associated with a good prognosis due to its indolent nature. Since KFD is chiefly a diagnosis of exclusion, it is liable to a higher degree of misdiagnosis. Aim In our study, we attempt to document and analyse the demographic, clinical, and pathological spectrum of cases diagnosed as KFD in a South Indian tertiary care centre over a period of 10 years. Methodology This study was conducted in a retrospective observational manner. Descriptive statistics, including simple frequency and percentage, were used to analyse and summarise the collected data. Results Among the 44 cases obtained, 36 patients were female and eight were males, with an age range of eight to 70 years. The mean age of the affected population was 26 years. Fever was a presenting complaint in 63.6% of cases, and 100% of the cases had lymphadenopathy, chiefly affecting the posterior cervical group of nodes (32/44 cases). Multiple lymph node groups were involved in 22 cases. Most of the nodes were larger than 1 cm in their greatest dimension. On analysis of peripheral smears, 43.2% of patients showed leukopenia. Anti-nuclear antibody (ANA) analysis showed a positive status among two patients, simultaneously establishing the diagnosis of systemic lupus erythematosus (SLE). A history of treated tuberculosis was present in four patients, and one patient had a history of non-compliance with TB treatment. Two patients had recently been diagnosed with TB and were on treatment. The involved nodes were subjected to fine-needle aspiration studies in 13 patients; 12/13 cases showed reactive lymphadenitis, while one case had atypical lymphoid cells. The involved nodes of all the patients were excised and sent for histopathological analysis. The diagnosis of KFD was confirmed on the biopsy. Conclusion In this study, we note the clinical presentations of all the patients diagnosed with KFD and elaborate on the investigations that were employed to verify the diagnosis via exclusion that enabled differentiation of KFD from its close mimickers, which have specific treatment. Keeping this entity in mind is essential in order to prevent overdiagnosis, as most often this condition resolves spontaneously, and in occasional cases, symptomatic treatment is sufficient for its management.

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.

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease, characterised by fever and cervical lymphadenopathy. Lymphadenopathy without cervical lymph node involvement is rare and may mimic lymphoma. Although KFD can be associated with extranodal involvement, muscle involvement has not been reported. Herein, we report a novel case of unilateral gluteal myositis associated with mesenteric KFD in a patient who presented with persistent fever and right hip pain. Radiological imaging revealed an inflammatory lesion on the right gluteal muscle and multiple enlarged abdominal lymph nodes. No cervical lymphadenopathy was observed. A mesenteric lymph node biopsy was performed, and the histopathological findings led to a diagnosis of KFD. By day 29, the patient's body temperature gradually returned to normal without any therapeutic intervention. Follow-up radiological imaging showed resolution of the gluteal lesion and a significant decrease in abdominal lymph node size. Considering the clinical course, the unilateral myositis may have developed as an extranodal involvement of KFD. Even if the clinical findings appear unrelated to those of KFD, a differential diagnosis that includes KFD should be considered in patients with unknown origin of fever.

Kikuchi-Fujimoto disease (KFD) is a benign self-limiting condition primarily affecting young females. It usually presents with fever and cervical lymphadenopathy of unknown aetiology with a preponderance of the Asian population. Histopathology is critical in making an accurate diagnosis. While the typical microscopic features include paracortical necrosis with debris, histiocytosis with immunoblasts, and absent neutrophils, rarely, KFD can show atypical features like marked immunoblastic proliferation mimicking lymphoma, demonstrate vasculitis mimicking lupus erythematosus, etc. The diagnosis is extremely challenging if such features occur in cases with generalised lymphadenopathy, which is infrequent in KFD. The study aims to describe the morphological, clinical, and immunohistochemical features of KFD and determine the frequency of the atypical features. We also analysed the subtle histological and immunohistochemical features that aid in the diagnosis of atypical cases. Cases reported as KFD over a period of 6 years were retrieved from the archives of histopathology. The morphological features were categorised as typical and atypical. In the atypical cases, the features that aided in the correct diagnosis of KFD were analysed. Out of the 42 cases evaluated, 23.9% (n=10) had generalised lymphadenopathy; 57.2% (n=24) were women with a median age of 25 years. Leukopenia was observed in 42% (n=13) of patients. Typical features were present in 76.2% (n=32) cases and 23.8% (n=10) presented with atypical features. Eight cases were antinuclear antibody-positive. Atypical features included five (50%) cases with vasculitis and panniculitis, and three (30%) cases with large, atypical cells for which immunohistochemistry (IHC) was performed. In two of these cases, the patent sinuses, absence of neutrophils, and IHC with CD68 aided the diagnosis. There is an overlap of clinical and histopathological features between KFD and malignant lymphomas and systemic lupus erythematosus. Given the fact that the atypical features (23.8%) are not rare occurrences in KFD, correlations with clinical findings and ancillary studies are essential to avoid misdiagnosis and inadvertent therapy.

Kikuchi-Fujimoto disease (KFD) is a self-limited disease that is more common in young Asian women. Typical presentations included fever and cervical lymphadenopathy. The etiology of KFD is unknown, and diagnosis is based mainly on lymph node biopsy. KFD has been reported to be associated with Mycoplasma pneumoniae infection. However, the role of antibiotic treatment is unclear. We reported 2 cases of KFD associated with Mycoplasma pneumoniae infection and were successfully treated with a macrolide.

Kikuchi-Fujimoto disease (KFD) also known as histiocytic necrotizing lymphadenopathy is an exceedingly rare cause of cervical lymphadenopathy, commonly accompanied by systemic symptoms such as fever, fatigue, night sweats, myalgia, skin rash.

In this paper, we report the case of a 22-year-old female patient who experienced a flare-up of leukocytoclastic vasculitis that was complicated by the appearance of a cervical lymph node with dysphagia, fever and nausea.

Infectious and autoimmune workup came back negative.

Excisional lymph node biopsy was done and the pathology results were consistent with histiocytic necrotizing lymphadenitis in keeping with Kikuchi-Fujimoto disease.

Patient improved on intravenous corticosteroids and was discharged on per os prednisone. Six month follow-up shows complete resolution of her symptoms.

KFD should be ruled out in patients with autoimmune or inflammatory diseases who develop lymphadenopathies.

The inflammatory response to viral infection is an important component of the antiviral response, a process that involves the activation and proliferation of CD8+ T, CD4+ T, and dendritic cells; thus, viral infection disrupts the immune homeostasis of the organism, leading to an increased release of inflammatory factors. Kikuchi-Fujimoto disease (KFD) is an inflammatory self-limited disorder of unknown etiology, and it is generally believed that the pathogenesis of this disease includes two aspects: viral infection and autoimmune response. Various immune cells, such as CD8+ T lymphocytes, CD4+ T lymphocytes, and CD123+ plasmacytoid dendritic cells, as well as the cytokines they induce and secrete, such as interferons, interleukins, and tumor necrosis factors, play a crucial role in the pathogenesis of KFD. In this article, we present a case study of a young female patient from China who exhibited typical symptoms of lymph node inflammation and fever. The diagnosis of KFD was confirmed through a lymph node biopsy. She presented with elevated ESR, IL-6, and IFN-γ. Viral markers showed elevated IgG and IgM of cytomegalovirus (CMV) and elevated IgG of Epstein-Barr virus (EBV), while changes occurred in the CD4+ T and CD8+ T cell counts. Eventually, the patient achieved disease relief through steroid treatment. Based on these findings, we conducted a comprehensive review of the involvement of viral infection-induced inflammatory response processes and autoimmunity in the pathogenesis of Kikuchi-Fujimoto disease.

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.

In this retrospective study, we aimed to investigate the frequency, trend, and nature of non-infectious diseases (non-IDs) as the final diagnosis for patients during an infectious disease (ID) consultation in an acute care hospital in Japan. This study included adult inpatients who underwent ID consultations between October 2016 and March 2018. The demographic data, clinical manifestations, and final non-ID diagnoses of cases were explored. Among the 502 patients who underwent ID consultations, 45 (9.0%) were diagnosed with non-IDs. The most common diagnoses were tumors (22.2%, n = 10), connective tissue and collagen vascular diseases (13.3%, n = 6), other inflammatory diseases (8.9%, n = 4), and drug-induced fever (8.9%, n = 4). Multiple logistic regression analysis showed that the presence of consultations for diagnosis (odds ratio [OR], 22.0; 95% confidence interval [CI], 10.1-48.2; p<0.01), consultations from the internal medicine department (OR, 2.5; 95% CI, 1.2-5.2; p = 0.02), and non-bacteremia cases (OR, 5.2; 95% CI, 1.4-19.3; p = 0.01) were independently associated with diagnosed non-IDs. Non-IDs after ID consultations were mainly tumor-related, inflammatory diseases, and drug fever. The presence of consultations for diagnosis, consultations from the internal medicine department and non-bacteremia cases were related to non-IDs among ID consultations. Further research is needed to explore the frequency and pattern of non-IDs to improve the quality of ID consultations in daily practice.

Kikuchi-Fujimoto disease (KFD) is a rare and self-limiting disorder that predominantly affects young individuals of Asian descent. This case report describes familial KFD in partially human leukocyte antigen (HLA)-matched siblings. An adolescent male presented with cervical lymphadenopathy and elevated lactate dehydrogenase (LDH) levels, diagnosed by biopsy as KFD; approximately one year later, his sister presented with similar symptoms. Both siblings were found to carry the HLA-DPB1*0202 allele, which is commonly associated with KFD. These cases highlight a genetic component in KFD and encourage further genetic research to delineate the pathogenesis of the disease.

Kikuchi Fujimoto disease (KFD) is a rare benign self-limiting condition described in young females characterized by lymphadenopathy and fever. It has been associated with several infective and autoimmune diseases, among which systemic lupus erythematosus (SLE) is relatively common. Kikuchi disease could occur either as a proceeding illness or as a coexisting illness with SLE. The presence of necrotizing lymphadenitis is appreciated in the histological specimen to confirm the diagnosis. Anti-nuclear antibody (ANA) positivity implicates a possible correlation with SLE or recurrence of the pre-existing disease. This clinical presentation needs to be evaluated thoroughly to prevent misdiagnosis and inappropriate treatment. Although Kikuchi disease generally warrants supportive treatment, steroids and immune therapy play a role in treating this persistent and recurrent disease. Long-term surveillance is mandatory for the early detection of sinister pathologies.

Kikuchi-Fujimoto disease is an uncommon systemic disease that mostly affects young women. Kikuchi-Fujimoto disease typically manifests as necrotizing lymphadenopathy, which frequently follows by a fever; however, Kikuchi-Fujimoto disease occurs rarely in extranodal regions. One of the most important accompaniments of Kikuchi-Fujimoto disease is its connection with autoimmune diseases such as systemic lupus erythematosus. This case presents a simultaneous occurrence of Kikuchi-Fujimoto disease with liver involvement and systemic lupus erythematosus in a young female patient.

We present a rare case of a 20-year-old white woman who presented with fever, joint pains, myalgia, and shortness of breath. Initial hospitalization and treatment for fever of unknown origin did not yield improvement. Physical examination revealed cervical and supraclavicular lymphadenopathy, and laboratory investigations showed abnormal blood counts, elevated inflammatory markers, and positive autoimmune serologies. Imaging studies revealed bilateral pleural effusion and liver lesions. Lymph node biopsy confirmed the diagnosis of Kikuchi-Fujimoto disease, and liver biopsy showed extranodal involvement. The patient was diagnosed with Kikuchi-Fujimoto disease-associated systemic lupus erythematosus and treated with hydroxychloroquine and corticosteroids. The patient showed gradual resolution of symptoms and lymphadenopathy with treatment.

Kikuchi-Fujimoto disease is a rare systemic condition primarily impacting young females. It is characterized by necrotizing lymphadenopathy, often accompanied by fever. Although Kikuchi-Fujimoto disease is predominantly seen in the lymph nodes, occurrences in non-nodal areas are infrequent. When diagnosing Kikuchi-Fujimoto disease, it is essential to screen patients for systemic lupus erythematosus. In this particular case, we observed liver involvement along with the presence of both Kikuchi-Fujimoto disease and systemic lupus erythematosus.

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign but rare disorder associated with febrile cervical lymphadenopathy in young adults. Here, we discuss a case of a young female patient presenting with left tender cervical lymphadenopathy that progressed bilaterally with a fever of unknown origin. Laboratory parameters showed persistent leukopenia, especially neutropenia, which fluctuated with the degree of symptom severity. Two months were taken to confirm the diagnosis of KFD based on the histological interpretation of the lymph node biopsy. Supportive management with analgesics and paracetamol formed the main treatment. This case highlights the challenges and importance of diagnosing KFD to exclude other serious conditions such as lymphoma, tuberculosis, or lupus lymphadenitis that share similar clinical manifestations as KFD.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon cause of protracted cervical lymphadenopathy in both children and adults. Although the majority of cases have been documented in Asia, this disease has been characterized globally since it was first identified in Japan in 1972. KFD's etiology is not entirely understood, although various theories have been postulated. Usually benign, Kikuchi-Fujimoto disease resolves within six months. KFD must be distinguished from other causes of chronic lymphadenopathy, such as lymphoma, inflammatory illnesses, autoimmune conditions, viral lymphadenopathy, and also from tuberculosis, especially in India, where it is still endemic. Here, we present the case report of a healthcare worker with a known case of hypothyroidism and autoimmune skin disorder who presented with prolonged fever, joint pain, and generalized lymphadenopathy post-COVID vaccination and was diagnosed as having KFD on biopsy, which can be associated with a very unusual presentation of this disease.

To explore the clinical and laboratory features, therapy and prognosis of Kikuchi-Fujimoto disease (KFD) in the cervical region.

We retrospectively reviewed the medical records of 134 patients who were diagnosed and treated with KFD from January 2000 to May 2022 in Fujian Medical University Union Hospital (Fujian, China). Their clinical characteristics, affected lymph node size, imaging examinations, and laboratory study results were analyzed.

The mean patient age was 24.9 years, and the male-female ratio was 1:1.73. Fever (55.2%, n = 74) was the most common clinical manifestation. Leukopenia (49.3%) was the commonest reported laboratory abnormality. A majority (65.7%) of the 134 patients presented with bilateral nodal involvement.

KFD should be considered as a possible diagnosis in a female patient under the age of 30 presenting with cervical lymphadenopathy, fever, leukopenia, and elevated LDH.

4.

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare benign disease that presents as cervical lymphadenopathy and fever.

A 25-year-old South Asian female dentist, recently married, presented to our emergency department due to two weeks of fever, sore throat, swollen neck, and cough. The patient initially presented to a private clinic and was prescribed antibiotics on two visits. On physical examination, her neck was swollen with palpable and tender right posterior and submandibular lymph nodes. Oropharyngeal examination revealed pharyngeal hyperemia without tonsillar enlargement, exudates, or mucocutaneous ulcers. Ultrasound imaging revealed enlarged neck and thoracic and abdominal lymph nodes. CBC showed leukopenia and anemia of chronic disease. B2 microglobulin, lactate dehydrogenase, and kappa light chains were elevated. Anti-ANA, anti-dsDNA, HIV polymerase chain reaction (PCR), Quantiferon-tuberculosis (TB), and rapid plasma reagin were all negative. A lymph node biopsy confirmed the diagnosis of Kikuchi-Fujimoto disease.

We believe this is the second case to be reported in the United Arab Emirates (UAE). Kikuchi-Fujimoto disease has a non-specific presentation that overlaps with several conditions including autoimmune, infectious, and malignant. Therefore, a thorough clinical approach and a high grade of clinical suspicion is required to rule out other possible differential diagnosis. Finally, Although Kikuchi-Fujimoto disease is mostly benign, clinicians should be careful as some patients might develop systemic lupus erythematosus (SLE), Still disease, or B cell lymphoma in the future.

Necrotizing lymphadenitis is a histological diagnosis that can arise from various conditions, including lupus lymphadenitis (LL), Kikuchi disease (KD), and infectious causes. Distinguishing between Kikuchi disease and lupus lymphadenitis can be challenging in clinical practice. In this report, we present the clinical scenario of a young female patient with lymphadenopathy and elucidate the process through which we ultimately arrived at a diagnosis of systemic lupus erythematosus (SLE) with macrophage activation syndrome. This case underscores the significance of recognizing Kikuchi disease as a condition that can mimic lupus and sheds light on the distinguishing features of necrotizing lymphadenitis, with a particular focus on Kikuchi disease and lupus lymphadenitis.

Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet's syndrome, Still's disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.

Kikuchi-Fujimoto disease (KFD) is an autoimmune condition that is more common in females and occurs in the third decade of life. The condition is usually benign and self-resolving and is characterized by fever, cervical lymphadenopathy, night sweats, myalgia, and rashes. The disease can be misdiagnosed as reactive follicular hyperplasia, tuberculous lymphadenitis, systemic lupus erythematosus, and malignant lymphoma. The diagnosis of KFD involves the excision of the affected lymph node. Although there is no specific treatment for the disease, usually symptomatic and supportive measures are effective; however, steroids and immunosuppressive therapies are considered in more severe cases. The disease lasts for around one to four months. The neurological complications include cerebellar ataxia, meningoencephalitis, and aseptic meningitis. Here, we describe the case of a 36-year-old male who presented with complaints of fever, malaise chills, anorexia, and fatigue associated with a tender right axillary lymph node. The patient underwent a biopsy which confirmed KFD and responded well to supportive therapy.

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited disease caused by subacute necrotizing regional lymphadenopathy. The etiology is unknown, although virus and autoimmune mechanisms have been proposed. Patients develop enlarged lymph nodes, fever, and, less frequently, extranodal signs. No specific laboratory test contributes to the diagnosis, and lymph node biopsy is the gold standard to define the diagnosis. The treatment is generally with supportive therapy and usually is spontaneously resolved within six months.  In this article, the authors present the case of a 41-year-old female with mild SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) infection 10 weeks before she was admitted to the emergency department (ED) due to cervical lymphadenopathies and fever lasting over three weeks. Physical examination revealed multiple lymphadenopathies on the submandibular and jugular regions, cutaneous rash, and hepatosplenomegaly. Blood tests showed elevated acute phase proteins, thrombocytopenia, and increased transaminases and lactate dehydrogenase (LDH). Computed tomography (CT) of the neck showed multiple adenopathies at levels I, II, III, and IV according to the Classification for Lymph Nodes from the American Head and Neck Society and American Academy of Otolaryngology on the right side. Excision biopsy was performed and histopathological examination confirmed KFD. A comprehensive analysis performed was unrevealing of an infectious or autoimmune cause and was assumed to be most likely triggered by SARS-CoV-2 infection given the timeframe correlation. KFD diagnosis is challenging and there are few reported cases of association with SARS-CoV-2 in the literature. Although further investigation is still needed to better understand the relation between them, it is important that physicians take SARS-CoV-2 infection and vaccination into consideration in KFD's differential diagnosis.

Systemic lupus erythematosus is a multisystem autoimmune disorder with a highly heterogeneous clinical presentation. The clinical phenotype varies from mild cutaneous and musculoskeletal manifestations to neurological involvement. Lymphadenopathy is a frequent manifestation of SLE, but the association is often not recognized, as lymphadenopathy is not a criterion for diagnosis. An unusual and seldom reported mimicker of lupus lymphadenitis is Kikuchi-Fujimoto Disease. This is a rare self-limiting disease of young adult females that presents with lymphadenopathy, fever, and systemic symptoms. Lupus lymphadenitis and KFD share some common clinical and pathologic features; but distinguishing between those two diseases can be challenging. We describe a 16-year-old Hispanic female who presented with axillary lymphadenopathy and was initially diagnosed with KFD based on an excisional lymph node biopsy; but later met the criteria for the 2019 European League Against Rheumatism/American College of Rheumatology classification criteria SLE. This case highlights the need for clinicians to be aware that patients with SLE may present with lymphadenopathy and to consider the association between Kikuchi disease and SLE to prevent misdiagnosis and allow for timely treatment to avoid complications.

Kikuchi-Fujimoto disease (KFD) is a rare cause of rapidly evolving tender cervical lymphadenopathy. It is often initially misdiagnosed and managed as infectious lymphadenitis. Although most cases of KFD are self-limited and improve with antipyretics and analgesics, some are more refractory and may require corticosteroids or hydroxychloroquine therapy.

A 27-year-old White man presented for evaluation of fevers and painful cervical lymphadenopathy. He was found to have KFD on excisional lymph node biopsy. His symptoms proved challenging to manage with corticosteroids but eventually improved with hydroxychloroquine monotherapy.

KFD diagnosis should be considered irrespective of geographic location, ethnicity, or patient sex. Hepatosplenomegaly is a relatively rare manifestation of KFD that can make it especially difficult to distinguish from lymphoproliferative disorder, such as lymphoma. Lymph node biopsy is the preferred diagnostic approach to achieve a timely and definitive diagnosis. Although usually self-limited, KFD has been associated with autoimmune conditions, including systemic lupus erythematosus. Securing the diagnosis of KFD is therefore crucial to ensuring patients are monitored appropriately for the development of associated autoimmune conditions.

Kikuchi-Fujimoto disease is a rare, benign, and self-limited disease of uncertain etiology, affecting mostly young female patients. It usually manifests as posterior cervical lymphadenopathy and fever. Its diagnosis is based on typical histopathological changes after the exclusion of other diseases such as lupus, lymphoma, or infectious lymphadenitis. The authors present a 47-year-old female patient with recurring episodes of high fever, urticarial rash, myalgia, arthralgia, fatigue, sore throat, and generalized lymphadenopathy. Blood tests showed increased inflammatory parameters and positive antinuclear antibodies. In the two times the patient was admitted to the hospital there were no infectious agents isolated. The patient didn't fulfill the criteria for diagnosis of lupus or any other autoimmune disease and there was also no evidence of lymphoma or other neoplastic diseases. A positron emission tomography/computed tomography (PET/CT) was performed at the first and second hospitalizations, showing generalized lymphadenopathy. At the first hospitalization, a mediastinal lymph node biopsy was obtained, excluding lymphoproliferative or granulomatous disease. During the course of the second hospitalization, a cervical lymph node was excised for biopsy, the histopathological changes of which suggested the diagnosis of Kikuchi-Fujimoto disease. The clinical course was complicated by hemophagocytic lymphohistiocytosis, with a significant increase in inflammatory markers and liver dysfunction. The patient was treated with prednisolone 1 mg/kg/day, with complete resolution of clinical and biochemical changes.

Although severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has caused many complications, the invention of coronavirus disease 2019 (COVID-19) vaccines has also brought about several adverse events, from common side effects to unexpected and rare ones. Common vaccine-related adverse reactions manifest locally or systematically following any vaccine, including COVID-19 vaccines. Specific side effects, known as adverse events of particular interest (AESI), are unusual and need more evaluation. Here, we discuss some of the most critical rare adverse events of COVID-19 vaccines.

Kikuchi-Fujimoto disease is a benign and self-limiting systemic disorder of unknown aetiology characterised by fever, superficial lymphadenopathy and leukopenia. In highly endemic & low-resource country like India, it is frequently misdiagnosed as tuberculosis.

Both the cases were diagnosed as necrotizing lymphadenitis by fine-needle aspiration cytology. Tuberculin skin prick test (TST) was positive for one case and negative for the other case. Cartridge based nucleic acid amplification test (CBNAAT) from lymph node aspirate was negative for mycobacterium tuberculosis in both the cases, later on histopathology of lymph node showed diagnosis of Kikuchi-Fujimoto disease.

Kikuchi Fujimoto is a self-limiting disease systemic disease of unknown aetiology. A definite diagnosis can be established by incisional/excisional biopsy of the lymph node. When dealing with cases of tubercular lymphadenitis, Kikuchi-Fujimoto disease should be kept as differential diagnosis.

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disorder characterized by regional lymphadenopathy. Clinical symptoms range from mild fever and tenderness to upper respiratory syndrome. A few cases have been observed during pregnancy or Hashimoto's disease. What we describe here is the first observed case of KFD in a pregnant woman with a history of Hashimoto's thyroiditis.

A 36-year-old woman presented to Aleppo University Hospital during the 13^th week of gestation with a painful cervical node on the right side of her neck. The patient's previous medical history confirmed Hashimoto's thyroiditis for several years. After histopathological examinations and radiological investigations, she was diagnosed with Kikuchi-Fujimoto disease and treated with corticosteroids. Although the patient did not adhere to the treatment very well due to her concerns for the fetus, the clinical picture improved after delivery. The patient now is on follow-up and continuing the current treatment with corticosteroids.

Further investigations need to be conducted to understand the possible autoimmune etiology of KFD when it is associated with Hashimoto's thyroiditis disease. It is also necessary to understand the relationship between this disease and pregnancy.

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.

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.

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.

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.

Kikuchi disease, also called histiocytic necrotizing lymphadenitis, is an idiopathic and generally self-limiting disease affecting young adults and children. Kikuchi disease does not commonly manifest neurological complications at its initial presentation. We herein report two cases of Kikuchi disease that initially presented with aseptic meningitis and encephalitis rather than the more common signs of lymphadenopathy, rash, and arthritis. A 15-year-old boy presented with aseptic meningitis with an extremely high intracranial pressure. A 28-year-old man presented with dysesthesia of the right lower extremity, coinciding with abnormal magnetic resonance imaging findings. In both cases, painful cervical lymphadenopathy was observed following the central nervous system symptoms. Both patients improved after treatment with steroids. Kikuchi disease occasionally affects the central nervous system, to which lymphadenitis may be observed subsequently. A repeated, careful physical examination of the cervical lymph nodes may be helpful for the diagnosis.

Kikuchi disease is common among Asians and women aged about 30 years. General symptoms include cervical lymphadenopathy and fever; 64% of the cases are self-limiting. However, this case is axillary lymphadenopathy, which is the main symptom in males. Thus, Kikuchi disease may be considered in the differential diagnosis of axillary lymphadenopathy.