Kikuchi–Fujimoto disease: A rare cause of cervical lymphadenopathy

Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare and generally self-limiting disease of uncertain etiology that presents with fever and cervical lymphadenopathy in both the pediatric and adult populations. Here, we present the case of a 5-year-old male who presented with acute onset of fever and cervical lymphadenopathy which was initially diagnosed and managed as staphylococcal lymphadenitis. He underwent an excisional biopsy when symptoms persisted, which confirmed the diagnosis of KFD and was managed conservatively. This case study emphasizes awareness of this entity in the differential diagnosis of fever with persistent lymphadenopathy. Due to its characteristic overlap with other disorders such as tuberculous lymphadenitis, and lymphoma, KFD remains an arduous diagnosis for physicians. Therefore, awareness about its symptoms helps in minimizing potentially harmful unnecessary evaluations and thereby preventing misdiagnosis and inappropriate treatment.

Clinicopathologic study of Kikuchi's disease in children in a tertiary hospital in South India

Background: The aim of this study was to evaluate the clinical and laboratory characteristics, treatment modalities and outcome of children with Kikuchi's disease.Methods: A retrospective cross-sectional study was conducted among all children, histopathologically diagnosed with KFD.' Clinical, laboratory data and treatment outcomes were analysed.Results: During the study period, 53 children histopathologically confirmed as KFD were enrolled in the study. There were 36 males and 17 females. The lymph node involvements were mostly cervical with bilateral predisposition (63.5%), firm (88%), matted (30.8%) and tenderness (38.5%). Fever, headache, vomiting, chills, myalgia and rash were other common presentations other than cervical lymphadenopathy. The associated laboratory findings include anemia (71.2%), leukopenia especially lymphopenia (31.4%), monocytosis (21.6%), thrombocytopenia (16.3%), elevated CRP (53.1%), ESR (83.7%), LDH (100%) and elevated liver enzymes. Most of the children were managed conservatively (49.1%). Corticosteroids were administered for (22.6 %) of patients. Recurrence occurred in 4 children (7.5 %) and 13 children (24.5%) had other associated diseases.Conclusions: KFD should be suspected in well children with febrile cervical lymphadenopathy, especially with leukopenia, monocytosis, and elevated CRP, ESR, LDH, Liver enzymes. KFD in children can have rarely atypical presentations and coexist with other diseases.

A Case of Kikuchi-Fujimoto Disease Associated with Mycoplasma Pneumoniae Infection / 임상소아혈액종양

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease characterized by subacute necrotizing lymphadenitis. This benign disease is frequently associated with prolonged fever and mostly occurs in young Asian women. KFD is generally diagnosed using a biopsy of affected lymph nodes and spontaneously resolves in several months. Although the causative agent is believed to be infectious, the etiology remains unknown. Some cases of KFD are associated with viral infections, including Epstein-Barr virus, human herpes virus 6, and parvovirus B19 infection. Herein, we report a case of KFD associated with Mycoplasma pneumoniae infection.

Kikuchi-Fujimoto Disease in a Filipino Boy: A Case Report

@#<p><strong>OBJECTIVE:</strong> To report a case of a 12-year-old boy with Necrotizing Lymphadenitis (Kikuchi-Fujimoto disease) presenting as lymphadenitis secondary to multiple diagnosis.</p><p> </p><p><strong>METHODS:</strong></p><p><strong>Design :</strong> Case Report</p><p><strong>Setting:</strong> Tertiary Private Hospital</p><p><strong>Patient:</strong> One</p><p> </p><p><strong>RESULTS:</strong> A 12-year-old boy consulted for two-month history of cervical lymphadenopathy with the underlying cause remaining unclear despite multiple consults, diagnosis and medical treatment. Lymphoma was considered and excision biopsy with further investigations confirmed a diagnosis of Kikuchi-Fujimoto disease. Supportive management was given with no recurrence of symptoms noted on 18 months of follow up. </p><p> </p><p><strong>CONCLUSION:</strong>  Kikuchi-Fujimoto disease in this case was a diagnosis of exclusion. Even with a proper history and physical examination, experts in otolaryngology can be misled to manage this case as malignant. Awareness of the disease and appropriate examinations including immunohistochemistry are important for a timely diagnosis and proper intervention.</p><p> </p><p><strong>KEYWORDS: </strong>lympadenitis; Kikuchi-Fujimoto disease; lymphoma; cervical lymph nodes</p>

Enfermedad de Kikuchi-Fujimoto, manifestación junto con siliconosis / Kikuchi-Fujimoto disease in presentation with silicosis

Resumen La enfermedad de Kikuchi-Fujimoto, también llamada linfadenitis necrotizante histiocítica, es un padecimiento raro y benigno de causa desconocida; se distingue por linfadenopatía cervical y fiebre frecuentemente en mujeres jóvenes previamente sanas. Las manifestaciones clínicas, el antecedente de lupus eritemaso sistémico y el análisis histopatológico sugieren una respuesta inmunitaria celular mediada por células T e histocitos como parte de la fisiopatología. El diagnóstico de enfermedad de Kikuchi-Fujimoto requiere biopsia por escisión de los ganglios linfáticos afectados en los que se evidencia necrosis con infiltrados de histiocitos y característicamente ausencia de neutrófilos. No existe tratamiento efectivo contra la enfermedad de Kikuchi-Fujimoto, el cuadro se alivia de manera espontánea en uno a cuatro meses; ante síntomas persistentes y severos se administran glucocorticoides y antiinflamatorios no esteroides. La siliconosis es una condición en la que el silicón, componente de implantes mamarios, actúa como coadyuvante inmunogénico provocando una respuesta local y sistémica autoinmunitaria con síntomas inespecíficos que constituyen el síndrome autoinmunitario inducido por coadyuvantes. El tratamiento de este síndrome se basa en la eliminación del estímulo externo y en la mayoría de los casos se observa una respuesta favorable a largo plazo sin necesidad de iniciar tratamiento inmunomodulador. Se necesitan más casos para poder establecer a la siliconosis como una probable causa de enfermedad de Kikuchi-Fujimoto y conocer más a fondo la relación entre ambos padecimientos.

Abstract Kikuchi-Fujimoto disease, also called histiocytic necrotizing lympha-denitis, is a rare and benign condition of unknown etiology, characte-rized by cervical lymphadenopathy with fever that frequently occurs in previously young healthy women. The clinical manifestations, a history of systemic lupus erythematous and histopathologic analysis suggest an immune response of T cells and histiocytes as a part of the pathophysiology. The diagnosis of Kikuchi disease requires lymph node biopsy, in which there is evidence of necrosis with histiocyte infiltrates and characteristically absence of neutrophils. No effective treatment exists for Kikuchi disease, it is self-limited into 1-4 weeks; with severe and persistent symptoms, glucocorticoids and nonsteroidal anti-inflammatory drugs are prescribed. Silicosis is a condition in which silicone, a component of breast implants, acts as an immunogenic adjuvant, causing a local and systemic autoimmune response with non-specific symptomatology constituting the adjuvant-induced autoimmune syndrome (ASIA). The treatment of ASIA is based on the elimination of the external stimulus and in most cases a favorable long-term response is observed without initiating immunomodulatory treatment. More cases are needed in order to establish silicosis as a cause of Kikuchi-Fujimoto disease and to know more about the relationship between these conditions.

Kikuchi's disease (histiocytic necrotizing lymphadenitis): A rare presentation with acute kidney injury, peripheral neuropathy, and aseptic meningitis with cutaneous involvement

Kikuchi's disease (KD) also known as histiocytic necrotizing lymphadenitis is rare, idiopathic, generally self-limited cause of lymphadenitis. We present a case of twenty year young female who presented in critically ill state with fever, cervical and axillary lymphadenopathy, rash, vomiting and altered sensorium and found to have neurological, hepatic, renal and dermatological involvement. Kikuchi's disease should be considered in differential diagnosis of fever and lymphadenopathy and though benign can sometimes present with multi-organ involvement. It is because of rarity of this disease with unusual complications, present case is reported.

Systemic Lupus Erythematosus Associated Pitfalls on ¹⁸F-FDG PET/CT: Reactive Follicular Hyperplasia, Kikuchi-Fujimoto Disease, Inflammation and Lymphoid Hyperplasia of the Spleen Mimicking Lymphoma / 대한핵의학회잡지

Systemic lupus erythematosus (SLE) is associated with a variety of inflammatory processes that can affect the lymph nodes, brain, kidneys, and spleen. We present two patients with SLE in whom SLE-associated conditions complicated interpretation of ¹⁸F-fluoro-2-deoxy-D-glucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) imaging of the lymph nodes and the spleen. The imaging findings mimicked lymphoma, but histopathological evaluation showed benign processes including reactive follicular hyperplasia in the lymph nodes, Kikuchi-Fujimoto disease in perisplenic lymph nodes, and inflammatory changes and lymphoid hyperplasia in the spleen.

Bilateral Frosted Branch Angiitis in Kikuchi-Fujimoto Disease

PURPOSE: A case of frosted branch angiitis in Kikuchi-Fujimoto disease is reported. CASE SUMMARY: A 33-year-old male complained of a sudden decrease in visual acuity that developed in both eyes 5 days prior. He suffered from a headache, chills, myalgia, and flank pain 1 week before. The initial best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.2 in the left eye. On slit lamp examination, no inflammatory finding was observed in the anterior chamber and vitreous body of both eyes. On fundus examination, a diffuse vascular sheathing-like frosted branch was found in the retinal vessels, and retinal hemorrhage was observed. Fluorescein angiography showed staining and leakage of dye along the vascular sheathing. Serological findings were negative, showing no evidence of an autoimmune disease or viral infection. Neck ultrasonography revealed non-tender left cervical lymph node enlargement >1 cm in diameter. Ultrasound-guided fine needle aspiration cytology showed findings compatible with Kikuchi-Fujimoto disease, including necrotic changes and pronounced karyorrhexis, plus histiocyte and lymphocyte infiltration without neutrophils. We started systemic steroid therapy. One month after treatment, the BCVA of both eyes improved to 1.0. CONCLUSIONS: In patients with frosted branch angiitis, systemic disease such as Kikuchi-Fujimoto disease should be considered.

Association Kikuchi disease with Hashimoto thyroiditis: a case report and literature review

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto’s thyroiditis.

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

Resumen: La enfermedad de Kikuchi-Fujimoto es poco común, de curso natural con alivio espontáneo, de causa desconocida, que se manifiesta por linfadenopatías, fiebre y síntomas menos específicos como diaforesis nocturna, pérdida de peso, cefalea, fatiga, náusea y artralgias. La única forma de establecer el diagnóstico es mediante los hallazgos histopatológicos, caracterizados por necrosis coagulativa con abundantes restos de cariorrexis en zonas paracorticales. El tratamiento es sintomático y debe realizarse diagnóstico diferencial con procesos infecciosos y neoplasias. Se comunica el caso de una mujer de 37 años de edad en la que se integró el diagnóstico de enfermedad de Kikuchi-Fujimoto y se realiza una revisión de la bibliografía.

Abstract: Kikuchi-Fujimoto disease is a rare, self-limiting disease of unknown etiology, manifested by lymphadenopathy, fever and less specific symptoms such as nocturnal diaphoresis, weight loss, headache, fatigue, nausea and arthralgias. The only way to establish the diagnosis is through the histopathological findings, characterized by coagulative necrosis with abundant remains of karyorrhexis in paracortical zones. The treatment is symptomatic, and a differential diagnosis must be made with infectious and neoplastic processes. We present the case of a 37-year-old woman in whom the diagnosis of Kikuchi-Fujimoto disease was integrated, and a review of the literature is made.

A Case of Recurrent Steroid-dependent Kikuchi-Fujimoto Disease Successfully Treated with Hydroxychloroquine / 임상소아혈액종양

Kikuchi-Fujimoto disease (KFD) is known as a self-limiting disease. The most common symptoms include fever, cervical lymphadenopathy, and pain, but nonspecific symptoms such as joint pain, nausea, chills, diaphoresis, and diarrhea may also be present. Its clinical course is generally benign, and symptoms including fever disappear within several months without special treatment. Thus, there is no specific recommended treatment for patients with KFD. However, some patients suffer from prolonged fever or systemic symptoms such as splenomegaly, skin rash, arthralgia, and aseptic meningitis. Many studies have reported the effectiveness of corticosteroids in patients with prolonged fever and systemic symptoms. Our patient also responded favorably to steroids; however, the disease relapsed while tapering. Recently, some studies reported the effectiveness of hydroxychloroquine (HC) in patients with KFD. Herein, we report successful treatment with HC in an adolescent patient with recurrent KFD dependent on steroids without any symptoms of autoimmune disease.

Kikuchi Fujimoto disease: A series of three cases

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.<br /><strong>CASE:</strong> The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.<br /><strong>CONCLUSION:</strong> The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.</p>

Kikuchi Fujimoto disease: A series of three cases

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.CASE: The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.CONCLUSION: The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.

Kikuchi Fujimoto disease: A series of three cases

INTRODUCTION: Kikuchi-Fujimoto disease (KFD) is a  rare self-limited disorder manifested by painful cervical lymphadenopathies commonly associated with fever and night sweats.This is a series of three female patients presenting with fever and lymphadenopathies diagnosed with KFD.CASE: The first case is a 34-year-old female admitted due to fever of 10 days associated with lymphadenopathies and joint pains.Excision biopsy done showed necrotizing histiocytic lymphadenitis consistent with KFD.Other laboratories showed hypocomplementemia, positive ANA and anti-dsDNA.Patient was discharged improved with low dose oral corticosteroid and hydroxychloroquine.The second case is a 53-year-old female with fever,lymphadenopathies,polyarthritis and morning stiffness.Biopsy of the cervical lymph node was done showing KFD and lupus serologies (ANA 1:640 speckled, anti-dsDNA and anti-Smith) revealed positive results as well.Patient was then diagnosed with SLE and was started on low dose oral corticosteroid and hydroxychloroquine which resulted to resolution of fever and gradual resolution of lymph nodes on out-patient follow up.The last case is a 45-year-old female admitted due to persistent fever, painful lymphadenopathies and headache. Serological work-up including autoantibody tests for SLE were all unremarkable but showed associated iron deficiency anemia. Biopsy of the cervical lymph node showed Kikuchi's disease. Patient was discharged with oral methylprednisolone.CONCLUSION: The rarity of KFD makes defining an autoimmune etiology a challenge to clinicians.Careful disease course follow up is then recommended for patients who initially lack parameters for SLE diagnosis.

A Case of Recurrent Steroid-dependent Kikuchi-Fujimoto Disease Successfully Treated with Hydroxychloroquine / 임상소아혈액종양

Kikuchi-Fujimoto disease (KFD) is known as a self-limiting disease. The most common symptoms include fever, cervical lymphadenopathy, and pain, but nonspecific symptoms such as joint pain, nausea, chills, diaphoresis, and diarrhea may also be present. Its clinical course is generally benign, and symptoms including fever disappear within several months without special treatment. Thus, there is no specific recommended treatment for patients with KFD. However, some patients suffer from prolonged fever or systemic symptoms such as splenomegaly, skin rash, arthralgia, and aseptic meningitis. Many studies have reported the effectiveness of corticosteroids in patients with prolonged fever and systemic symptoms. Our patient also responded favorably to steroids; however, the disease relapsed while tapering. Recently, some studies reported the effectiveness of hydroxychloroquine (HC) in patients with KFD. Herein, we report successful treatment with HC in an adolescent patient with recurrent KFD dependent on steroids without any symptoms of autoimmune disease.

Una relación potencialmente peligrosa: lupus eritematoso sistémico y enfermedad de Kikuchi - Fujimoto / A dangerous relation: Systemic lupus erythematosus disease and Kikuchi Fujimoto disease

Resumen La enfermedad de Kikuchi-Fujimoto (EKF) es conocida como una linfadenitis necrotizante histiocítica, tiene una presentación benigna y autolimitada, afecta principalmente a mujeres jóvenes y generalmente resuelve sin tratamiento en los primeros seis meses de los síntomas. Presentamos el caso de una mujer de 48 años quien consultó por un cuadro de dolor abdominal, mialgias y adenopatía cervical al examen físico. Se le encontró en los exámenes paraclínicos anticuerpos antinucleares 1:640, anti DNA de doble cadena asociado a consumo de complemento y anticuerpos anticardiolipina positivos. Se realizó biopsia de ganglio cervical que mostró necrosis cortical y población de histiocitos y linfocitos. Al iniciar el manejo con esteroides y cloroquina se comprobó mejoría franca y posteriormente la paciente fue dada de alta, continuando controles de manera ambulatoria por los servicios de reumatología y medicina interna.

Abstract Kikuchi disease, also known as histiocytic necrotizing lymphadenitis, isa benign and self-limited condition that mainly affects young women andresolve without treatment within six months of symptoms. It was first describedin Japan in 1972 and is more common in Asian population. We report the case of a woman of 48 years who consults for abdominal pain, myalgia, and cervical lymphadenopathy on physical examination. He found himself in the presence of antinuclear antibodies paraclinical 1: 640 positive DNA double chain associated with complement consumption and positive cardiolipin antibodies We perform cervical node biopsy that showed the presence of cortical necrosis and histiocytes and lymphocytes population. When you start handling steroid and chloroquine, a high frank and subsequent improvement was observed, continuing control rheumatology and internal medicine.

Recurrent Kikuchi's Disease Treated by Hydroxychloroquine / 감염과화학요법

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disease, with a specific histopathology. It can be diagnosed clinically, and specific symptoms include fever and cervical lymphadenopathy. The histological finding of KFD in cervical lymph nodes includes necrotizing lymphadenitis. KFD needs conservative treatments. If KFD persists for a long period, steroids or nonsteroidal antiinflammatory drugs can be used to control symptoms. Previous studies have reported the treatment of KFD with hydroxychloroquine (HC) in patients unresponsive to steroids. Herein, we report a case of a 25-year-old female patient diagnosed with KFD unresponsive to steroids, and was successfully treated with HC.

Kikuchi-Fujimoto Disease Misdiagnosed as Relapse of the Infection after Treatment of Periprosthetic Hip Joint Infection / 대한고관절학회지

Periprosthetic joint infection (PJI) of the hip can be difficult to treat and can lead to a number of problems including: i) severe functional decline of the hip joint and ii) increasing financial burden for patients due to long treatment periods and the need for repeated surgical interventions. Because there is risk of inadequate control of infection or relapse of a preexisting infection following the treatment of PJI through surgery, it is important to closely observe clinical symptoms such as systemic fever. Kikuchi-Fujimoto disease is usually a self-limiting disease characterized by fever and cervical lymphadenopathy. We report one case of Kikuchi-Fujimoto disease, with literatures review, that was mistaken for an infection relapse after surgical treatment of the PJI due to sustained fever postoperatively.

A Case of Kikuchi Disease Involving an Intrapulmonary Lymph Node Mistaken for a Pulmonary Nodule / 대한내과학회지

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon idiopathic, self-limiting disease characterized by cervical lymphadenopathy. Patients with KFD may present with a wide variety of nonspecific symptoms, including fever, night sweats, and weight loss. Although KFD can affect all lymph nodes of the body, involvement of the intrathoracic lymph nodes is relatively rare. In particular, isolated involvement of the intrapulmonary lymph nodes is extremely unusual. We herein report a case involving a 45-year-old man who presented with symptoms of myalgia, fatigue, and fever. Computed tomography performed during follow-up showed a slowly growing nodule in the upper lobe of the left lung. Results of laboratory tests did not reveal any evidence of infection or autoimmune disease, including systemic lupus erythematosus. Results of excisional biopsy by video-assisted thoracoscopic surgery revealed KFD in an intrapulmonary lymph node. His symptoms improved after a trial of nonsteroidal anti-inflammatory drugs.