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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.
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PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
Subject(s)
Dendritic Cells , Histiocytic Necrotizing Lymphadenitis , Interferon-alpha , Lymph Nodes , Humans , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/blood , Histiocytic Necrotizing Lymphadenitis/immunology , Male , Interferon-alpha/blood , Female , Child , Adolescent , Adult , Dendritic Cells/immunology , Dendritic Cells/metabolism , Child, Preschool , Lymph Nodes/pathology , Myxovirus Resistance Proteins/genetics , Myxovirus Resistance Proteins/metabolism , Myxovirus Resistance Proteins/blood , Young Adult , Middle Aged , Lymphoma/diagnosis , Lymphoma/immunology , Lymphoma/blood , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/immunology , Mucocutaneous Lymph Node Syndrome/blood , Biomarkers/blood , Cytokines/blood , Cytokines/metabolismABSTRACT
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.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Recurrence , Humans , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/epidemiology , Histiocytic Necrotizing Lymphadenitis/pathology , Male , Female , Child , Retrospective Studies , China/epidemiology , Lymphadenopathy/etiology , Fever/etiology , Risk Factors , Child, Preschool , Adolescent , Meningitis, Aseptic/epidemiology , Meningitis, Aseptic/diagnosis , East Asian PeopleABSTRACT
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.
Subject(s)
Lymphadenopathy , Magnetic Resonance Imaging , Neck , Humans , Male , Female , Child , Lymphadenopathy/diagnostic imaging , Magnetic Resonance Imaging/methods , Neck/diagnostic imaging , Diagnosis, Differential , Child, Preschool , Adolescent , Retrospective Studies , Infant , Lymph Nodes/diagnostic imaging , Lymph Nodes/pathology , RadiomicsABSTRACT
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign condition affecting young Oriental-Asian females. It is characterized by fever and tender cervical lymphadenopathy with an unclear aetiology, and in most longitudinal reviews, KFD occurs before systemic lupus erythematosus (SLE). Herein, the case of a 28-year-old Kuwaiti female without any relevant past medical history, who was simultaneously diagnosed with KFD and SLE following an Ebstein-Barr virus infection, is reported. The patient was treated with oral prednisolone, hydroxychloroquine, cyclosporin, and belimumab and her response was clinically and biochemically favourable. Although KFD is prevalent in Asian populations, it may affect all races. Early diagnosis of KFD is difficult, particularly when simultaneously diagnosed with SLE, but crucial to preventing inappropriate therapy. Clinicians need to know about this rare disease, especially when patients present with fever and swollen lymph nodes, due to a risk of misdiagnosis with tuberculosis or lymphoma, as these are more often thought to be the cause of such symptoms.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Lupus Erythematosus, Systemic , Adult , Female , Humans , Arabs , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/drug therapy , Histiocytic Necrotizing Lymphadenitis/pathology , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Prednisolone/therapeutic use , Prednisolone/administration & dosage , KuwaitABSTRACT
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).
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Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare benign condition characterized by cervical lymphadenopathy and constitutional symptoms mimicking tuberculosis. We present the case of a 22-year-old male who presented with fever, dry cough, loss of appetite, multiple joint pains for 15 days, and loss of weight for one month. Physical examination revealed palpable cervical, occipital, axillary, and inguinal lymphadenopathy, and laboratory investigations were within normal limits except for raised erythrocyte sedimentation rate (ESR). Contrast-enhanced computed tomography (CECT) showed mediastinal lymphadenopathy with no pleuroparenchymal abnormality of the lung. Excision biopsy of a cervical lymph node confirmed necrotizing lymphadenitis consistent with KFD. The patient was treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and glucocorticoids, resulting in the resolution of symptoms and regression of lymphadenopathy. This case signifies the importance of considering KFD in the differential diagnosis of lymphadenopathy and highlights the significance of histopathological evaluation for accurate diagnosis and management guidance.
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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.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Lymphadenopathy , Female , Humans , Child , Histiocytic Necrotizing Lymphadenitis/complications , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/drug therapy , Lymphadenopathy/complications , Prednisolone/therapeutic use , Fever/etiology , PainABSTRACT
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.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Humans , Female , Histiocytic Necrotizing Lymphadenitis/diagnosis , Pregnancy , Adult , Pregnancy Complications/diagnosis , Hyperemesis Gravidarum/diagnosisABSTRACT
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.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Lymphohistiocytosis, Hemophagocytic , Adult , Humans , Child , Histiocytic Necrotizing Lymphadenitis/complications , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/drug therapy , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphohistiocytosis, Hemophagocytic/drug therapy , Positron Emission Tomography Computed Tomography/adverse effects , Lymph Nodes , Biopsy/adverse effectsABSTRACT
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, EpsteinâBarr virus was positive in these lymph nodes by in situ hybridization. This may suggest a trigger for the coexistence of the two diseases.
Subject(s)
Epstein-Barr Virus Infections , Histiocytic Necrotizing Lymphadenitis , Thyroid Neoplasms , Humans , Male , Histiocytic Necrotizing Lymphadenitis/complications , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/pathology , Thyroid Cancer, Papillary/pathology , Epstein-Barr Virus Infections/pathology , Herpesvirus 4, Human , Lymph Nodes/pathology , Thyroid Neoplasms/complications , Thyroid Neoplasms/pathologyABSTRACT
[Objective]To explore the etiology,pathogenesis and clinical treatment of histiocytic necrotizing lymphadenitis(HNL)based on the insidious pathogen warm disease theory.[Methods]To analyze the etiology,pathogenesis,characteristics of symptoms and transmission of the disease in Chinese medicine,and summarize the treatment principles based on the descriptions about insidious pathogen warm disease in ancient literature and modern researches on HNL,and cite a clinical case for verification.[Results]The pathogenesis of the HNL is mainly characterized by deficient healthy Qi leading to latent evil.The specific manifestation is that exogenous evils are latent in the Moyuan,which causes Yang Qi to be blocked and depressed and turns into heat.Evil heat spreads from the Moyuan to the Shaoyang tri-Jiao,leading to the generation of phlegm,static blood,turbid evil and toxin in the body,which in turn leads to disease.In terms of treatment,supporting healthy Qi and eliminating the evil is regarded as the law of treatment,and the emphasis is eliminating the evil.Aiming at the three pathogenetic links of latent evil,depressed heat and internal production of pathological products,the following treatment principles are formulated:expelling evils from Moyuan,dredging tri-Jiao and promoting the flow of Qi to make the evil heat go out,clearing away endogenous pathological products,removing toxin and dispersing knots.The focus of supporting healthy Qi is the protection of Qi and Yin as well as recuperation after recovery,which prevents evil Qi from remaining and causing the disease to reoccur.The medical case cited was a patient with HNL treated by applying the theory of insidious pathogen warm disease.Damp-heat and toxin brewing,phlegm combined with static blood was the traditional Chinese medicine(TCM)pattern of this case.The the prescriptions were based on Shengjiang Powder combined with Sanren Decoction,and Ganlu Xiaodu Pill successively,added and subtracted according to the syndrome,and the case achieved a significant effect.[Conclusion]The effect of treating HNL based on the insidious pathogen warm disease theory is quite good,which can provide new ideas and methods for the diagnosis and treatment of HNL.
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Kikuchi-Fujimoto disease (KFD), or Histiocytic Necrotizing Lymphadenitis, is a rare disease, with worldwide distribution but is best known in Japan and South Asia. The most common feature is cervical lymphadenopathy, accompanied by tenderness or high fever, with night sweats, but it can also be asymptomatic or with a very wide range of symptoms. The diagnosis is histopathological, on excisional biopsy. The Kikuchi-Fujmoto disease can mimic lymphoma but also tuberculosis and some autoimmune diseases, or be associated with them. Nephrologists need to be aware of it, considering the potential renal involvement. The association with systemic lupus erythematosus (SLE) is the most frequent but not the only one. Early diagnosis of this disease can prevent unnecessary investigations and aggressive therapies.
Subject(s)
Histiocytic Necrotizing Lymphadenitis , Lupus Erythematosus, Systemic , Humans , Histiocytic Necrotizing Lymphadenitis/complications , Histiocytic Necrotizing Lymphadenitis/diagnosis , Histiocytic Necrotizing Lymphadenitis/pathology , Diagnosis, Differential , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/pathology , Early Diagnosis , BiopsyABSTRACT
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.
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Kikuchi-Fujimoto disease is self-limited, has a good prognosis, is diagnosed histopathologically, is more common on the Asian continent, in women and in those under the age of 40, and is usually diagnosed by exclusion. We present the case of a 20-year-old male patient with no significant medical history who consulted due to persistent fever, enlarged lymph nodes, gastrointestinal symptoms and weight loss. He debuted with pancytopenia, elevated acute phase reactants and altered liver function, and underwent an excisional lymph node biopsy, whose findings were compatible with Kikuchi-Fujimoto disease. He was treated symptomatically with NSAIDs and was followed up one month after discharge, at which time there was no evidence of recurrent fever or other additional symptoms. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2553).
La enfermedad de Kikuchi Fujimoto es autolimitada, de buen pronóstico, de diagnóstico histo-patológico, cuya incidencia es mayor en el continente asiático en mujeres, en menores de 40 años y usualmente es un diagnóstico de exclusión. Presentamos el caso de un paciente masculino de 20 años sin antecedentes patológicos, quien consultó por fiebre persistente, adenopatías, síntomas gastrointestinales y pérdida de peso; debutó con pancitopenia, aumento de reactantes de fase aguda, alteración de función hepática y en quien se le practicó biopsia excisional de ganglio, la cual evidenció hallazgos compatibles con la enfermedad de Kikuchi Fujimoto. Se hace manejo sintomático con AINES y se realiza control al mes de egreso, donde no hay evidencia de recurrencia de picos febriles u otros síntomas adicionales. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2553).
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Objective:To investigate the diagnosis and treatment of histiocytic necrotizing lymphadenitis (HNL) complicated with hemophagocytic syndrome (HPS).Methods:The clinical characteristics, diagnosis, treatment process, and therapy response of a patient with HNL complicated with HPS admitted to the Institute of Hematology & Blood Diseases Hospital Chinese Academy of Medical Sciences in March 2019 were retrospectively analyzed, and the literature was reviewed.Results:This 17-year-old female patient had fever with bilateral cervical lymphadenopathy as the first presentation, accompanied by cough and expectoration. After admission, the disease progressed rapidly, and the serum ferritin increased progressively.The regimen of hormone and etoposide was used to control the disease condition. The bone marrow smear revealed atypical lymphocytes and hemophagocytic phenomenon, and the pathological features of HNL in lymph node biopsy were observed. This patient was finally diagnosed as HNL complicated with HPS. The patient's condition was stable at 3-month follow-up after discharge.Conclusions:The clinical manifestations of HNL patients complicated with HPS are similar to other hematologic malignant diseases, and application of multiple laboratory and pathological examination methods can help with early diagnosis. In the event of a progressive rise in serum ferritin, timely application of hormone therapy combined with etoposide if necessary can rapidly control the progression of the disease.
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El lupus eritematoso sistémico (LES) puede presentarse de muchas maneras y puede ser difícil de diagnosticar. En ocasiones puede ser difícil distinguir la enfermedad subyacente, ya que se sabe que el LES mismo causa síntomas gastrointestinales e incluso malabsorción. Este caso refleja los desafíos de diagnóstico que enfrentamos durante la larga y complicada hospitalización de una adolescente con compromiso sistémico, síndrome febril, malabsorción, marcadores inflamatorios elevados, hipoacusia, nefritis, linfadenitis necrotizante histiocítica
Systemic lupus erythematosus (SLE) can present in many ways and can be difficult to diagnose. It can sometimes be difficult to distinguish the underlying disease, as SLE itself is known to cause gastrointestinal symptoms and even malabsorption. This case reflects the diagnostic challenges we faced during the long and complicated hospitalization of an adolescent with systemic compromise, febrile syndrome, malabsorption, elevated inflammatory markers, hearing loss, nephritis, histiocytic necrotizing lymphadenitis
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RESUMEN ntroducción: la enfermedad de Kikuchi-Fujimoto es una causa rara de linfadenopatía cervical. Se caracteriza por una proliferación focal de células reticulares acompañadas de numerosos histiocitos y restos nucleares extensos. Objetivo: presentar un paciente con enfermedad de Kikuchi-Fujimoto diagnosticado por el departamento de Anatomía Patológica. Presentación del caso: paciente masculino con VIH, color de la piel negra, 33 años de edad, acudió a consulta por presentar un síndrome febril prolongado y adenopatías en la región del cuello. Con la administración de anestesia local y sedación se realizó escisión de adenopatías y se diagnosticó una linfadenitis necrotizante histiocítica o enfermedad de Kikuchi-Fujimoto. Conclusiones: la enfermedad de Kikuchi-Fujimoto es una entidad rara y de etiología incierta. El diagnóstico certero se obtiene del estudio histopatológico de una biopsia y el tratamiento incluye el uso de antipiréticos y antiinflamatorios no esteroideos.
ABSTRACT Introduction: Kikuchi-Fujimoto disease is a rare cause of cervical lymphadenopathy. It is characterized by a focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear remains. Objective: to present a patient with Kikuchi-Fujimoto disease diagnosed by the Department of Pathology. Case report: a 33-year-old, male, black race, HIV patient came to the clinic for presenting a prolonged febrile syndrome and lymphadenopathy in the neck region. With the administration of local anesthesia and sedation, adenopathies excision was performed and a histiocytic-necrotizing lymphadenitis or Kikuchi-Fujimoto disease was diagnosed. Conclusions: Kikuchi-Fujimoto disease is a rare entity with an uncertain etiology. The accurate diagnosis is obtained from the pathologic-histological study of a biopsy and the treatment included the use of antipyretics and non-steroidal anti-inflammatory drugs.
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RESUMO: A doença de Kikuchi-Fujimoto ou linfadenite histiocítica necrosante, de curso benigno, é prevalente em mulheres jovens, e associada à febre e leucopenia. Estudos recentes têm demostrado que sua etiologia ainda é incerta, sendo uma doença rara, com incidência de 0,5 a 5% de todas as adenopatias analisadas histologicamente. O diag-nóstico diferencial por imunohistoquímica foi decisivo, descartando outras hipóteses diagnósticas como: linfoma, tuberculose ganglionar e lúpus eritematoso sistêmico. Este relato de caso mostra as características da apresentação da doença em uma mulher, caucasiana e todas as etapas da investigação, destacando a importância dos diagnósticos diferenciais em adenopatias dolorosas e as dificuldades quando avaliamos portadores de doenças raras. (AU)
ABSTRACT: Kikuchi-Fujimoto disease or benign necrotizing histiocytic lymphadenitis is prevalent in young women and associated with fever and leukopenia. Recent studies have shown that its etiology is still uncertain, being a rare disease, with an incidence of 0.5 to 5% of all histologically analyzed adenopathies. Differential diagnosis by immunohistochemistry was decisive, ruling out other diagnostic hypotheses such as lymphoma, ganglion tuberculosis, and systemic lupus erythematosus. This case report shows the characteristics of the presentation of the disease in a Caucasian woman and all stages of the investigation, highlighting the importance of differential diagnoses in painful adenopathies and the difficulties when evaluating rare-disease patients. (AU)
Subject(s)
Humans , Female , Adult , Histiocytic Necrotizing Lymphadenitis , Rare Diseases , Ganglion Cysts , Diagnosis, Differential , Lupus Erythematosus, Systemic , LymphadenitisABSTRACT
OBJECTIVE@#To evaluate the clinical efficacy of medicinal penetration on acupoint combined with medication for histiocytic necrotizing lymphadenitis (HNL) of heat-toxin syndrome, and to explore the methods of improving the clinical effect.@*METHODS@#A total of 72 cases with HNL with heat-toxin syndrome were randomly divided into an observation group and a control group, 36 cases in each group. The patients in the control group were treated with oral administration of prednisone tablets for 40 days (first 5 days: 10 mg, three times a day; since then, reduced by 5 mg every 7 days). In the observation group, on the basis of the medication in the control group, the patients were treated with acupoint application and ultrasonic drug penetration therapy, once a day for 14 days. The acupoints of Waiguan (TE 5), Fengchi (GB 20) of affected side and points were selected. The changes of target lymph node swelling, visual analogue score (VAS), axillary temperature and total score of symptoms and signs were evaluated before treatment and 7, 14, 28 and 40 d into treatment; the changes of white blood cell (WBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and lactic dehydrogenase (LDH) were evaluated on 14 d and 40 d into treatment; the patients were followed-up for half a year.@*RESULTS@#① Fourteen days into treatment, the clinical cured rate in the observation group was 38.9% (14/36), which was superior to 16.7% (6/36) in the control group (0.05). ④ The recurrence rate in the observation group was 5.6% (2/36), which was similar to 16.7% (6/36) in the control group (>0.05).@*CONCLUSION@#The medicinal penetration on acupoint as adjunctive treatment could effectively relieve the discomfort symptoms of HNL patients with syndrome of heat and toxin, improve the clinical cured rate, and provide the research direction for shortening the course of medication.