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.

Kikuchi-Fujimoto disease. An unusual disease

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).

Kikuchi Fujimoto disease: a rare case report

Kikuchi disease, also known as Kikuchi histiocyticnecrotizing lymphadenitis, was initially described in young Japanese women. It is a rare benign self-limiting disease of unknown cause usually characterized by fever and cervical lunphadenitis. It is diagnosed by doing excisional biopsy of affected lymph node. A case of young 20 years old female presented with complaint of fever and weight loss for 20 days along with cervical and axillary lymphadenopathy. Complete physical examination, radiological investigations and biochemical tests were done to rule outsystemic lupus erythematous, non-Hodgkin lymphoma and tuberculosis.Only symptomatic treatment was done along with corticosteroids. Its diagnosis is important as it can be easily mistaken for other form of lymphadenitis. Clinician and pathologists’ awareness of this disorder is very necessary.

Hemophagocytic syndrome as the first presentation of histiocytic necrotizing lymphadenitis: report of 1 case and review of literature / 白血病·淋巴瘤

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.

Reto Diagnóstico: Hipoacusia, Enteropatía Autoinmune y Linfadenopatia de Kikuchi ­ un Debut Inusual de LES / Diagnostic challenge: hearing loss, autoimmune enteropathy and Kikuchi lymphadenopathy - An unusual debut of SLE

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

Two Cases with Clinical Diagnosis of Subacute Necrotizing Lymphadenitis Successfully Treated with Shosaikoto / 日本東洋医学雑誌

We experienced 2 patients with clinical diagnosis of subacute necrotizing lymphadenitis who were successfully treated with shosaikoto. Case 1 is a 37-year-old woman. Left neck swelling and pain appeared from 12 days before, and temperature went up thereafter. Antibiotics were administered at another hospital, but she did not improve. There were multiple swollen lymph nodes of the left neck, and blood test showed decreased white blood cells and increased LDH. So we clinically diagnosed her as subacute necrotizing lymphadenitis. After administration of shosaikoto, fever resolved, and cervical swelling also markedly improved on the third day. On day 10, both symptom and findings disappeared. Case 2 is a 12-year-old man. Left neck swelling and pain appeared and temperature went up from 8 days before. An antibiotic was administered at another hospital, but he did not improve. There were multiple swollen lymph nodes of the left neck, and blood test showed decreased white blood cells and increased LDH. So we clinically diagnosed him as subacute necrotizing lymphadenitis, and administered shosaikoto. Then fever resolved on the third day, and cervical swelling diminished and tenderness disappeared on day 10. On day 18, both symptom and findings disappeared.

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

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.

Adenomegalia dolorosa: doença de Kikuchi entre os diagnósticos diferenciais / Painful adenomegaly: Kikuchi disease among differential diagnoses

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)

The correlations between traditional Chinese medicine syndromes of histiocytic necrotizing lymphadenitis and its pathological types / 国际中医中药杂志

Objective@#To investigate the interaction between traditional Chinese medicine (TCM) syndromes of histiocytic necrotizing lymphadenitis (HNL) and pathological types of HNL, so as to discover the distribution rule of TCM syndromes for indiction of intervention of HNL.@*Methods@#A total of 166 patients with HNL were included. The baseline data of the patients, clinical feature, the four diagnosis information of TCM and histopathological of cervical lymph node were collected. The syndromes of TCM were judged. The pathological types were confirmed by cervical lymph node biopsy. The Multi-way ANOVA was used to analyze the correlation between traditional Chinese medicine syndromes of HNL and its pathological types.@*Results@#Among the 166 patients, the ratio of different types are as follow: wind-heat and phlegm-toxin syndrome (57.83%, 96/166), heat-toxin exuberance syndrome (34.34%, 57/166), and phlegm and blood stasis syndrome (7.83%, 13/166). The pathological types include proliferative type (57.83%, 96/166), necrotizing type (35.54%, 59/166), and xanthomatous type (6.63%, 11/166). There were significant differences in targeted lymph node diameter, pain scores, body temperature (F value were 3.737, 34.484, 34.805, all Ps<0.05), while the difference of WBC (F=1.194, P>0.05) among three TCM Syndrome types were notsignificant. There were significant differences in pain scores, body temperature (F value were 12.153, 25.931, all Ps<0.05), but not for the targeted lymph node diameter, WBC (F value were 2.249, 0.671, all Ps>0.05) among three pathological types. The corresponding analysis result showed that, on point sets, wind-heat and phlegm-toxin syndrome were correlated with proliferative type, heat-toxin exuberance syndrome correlated with necrotizing type, and phlegm and blood stasis syndrome correlated with xanthomatous type (χ2=235.100, P<0.001).@*Conclusions@#There is a close correlation between syndrome types of TCM and pathological types of HNL. The pathological change of wind-heat and phlegm-toxin syndrometends toproliferative type, heat-toxin exuberance syndrome tends to necrotizing type, and phlegm and blood stasis syndrome tends to xanthomatous type.

Efficacy of medicinal penetration on acupoint as adjunctive therapy on histiocytic necrotizing lymphadenitis of heat-toxin syndrome: a randomized controlled trial / 中国针灸

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.

Piriformis Pyomyositis in a Patient with Kikuchi-Fujimoto Disease - A Case Report and Literature Review / Piomiosite do piriforme em um paciente com doença de Kikuchi-Fujimoto-relato de caso e revisão da literatura

Abstract Primary pyomyositis is a deep bacterial infection of the skeletal muscle. If left undiagnosed and untreated, the infection spreads, leading to sepsis, septic shock, and even death. The authors report a 23-year-old female presenting with piriformis pyomyositis during a treatment for Kikuchi-Fujimoto disease. Pyomyositis is a rare but potentially severe infection, which can lead to septic shock. The present case shows the need for a high degree of clinical suspicion for patients with compromised immune systems to begin treatment at an early stage. The literature demonstrates that outcomes of the treatment of piriformis pyomyositis are good.

Resumo A piomiosite primária é uma infecção bacteriana profunda do músculo esquelético. Quando não diagnosticada ou tratada, a infecção pode evoluir para sepse, choque séptico e até morte. Os autores relatam o caso de uma paciente do sexo feminino, 23 anos, apresentando piomiosite do músculo piriforme durante o tratamento da doença de Kikuchi-Fujimoto. A piomiosite é uma infecção rara, mas potencialmente grave, que pode levar ao choque séptico. Esse caso mostra a necessidade em se elevar o grau de suspeição clínica em pacientes com comprometimento do sistema imunológico, para que o tratamento seja iniciado em estágio precoce. A literatura mostra que os resultados do tratamento da piomiosite do piriforme são bons.

Acoustic radiation force impulse imaging of biopsy-proven Kikuchi disease: initial experiences for evaluating feasibility in pediatric patients

PURPOSE: This study evaluated the feasibility of acoustic radiation force impulse (ARFI) elastography and characterized the sonographic features of lymph nodes (LNs) with Kikuchi disease in pediatric patients. METHODS: Seventy-six cervical LN biopsies were performed for the diagnosis of cervical lymphadenopathy. ARFI imaging was performed, and the characteristic ultrasound features of the biopsied LNs and the contralateral LNs were analyzed. We also reviewed clinical and conventional ultrasonographic findings. RESULTS: On histology, 56 patients were diagnosed with Kikuchi disease. These LNs were large and elongated, with increased perinodal echogenicity and capsular thickening. In 38 of them, ARFI elastography was performed, and the median shear wave velocity (SWV) of the biopsied LNs with Kikuchi disease (2.19 m/sec; range, 1.45 to 4.57 m/sec) was higher than of the contralateral LNs (1.72 m/sec; range, 0.95 to 2.65 m/sec; P < 0.001). In patients with reactive hyperplasia, the mean SWV of the biopsied LNs (2.00 m/sec; range, 1.49 to 2.26 m/sec) was higher than that of the contralateral LNs (1.55 m/sec; range, 1.21 to 2.32 m/sec; P=0.031). CONCLUSION: The SWV of LNs with Kikuchi disease was significantly higher than that of the contralateral LNs. Morphologically, LNs with Kikuchi disease showed an enlarged, elongated, and oval shape, increased perinodal echogenicity, and capsular thickening. In addition to the conventional ultrasonographic findings, the application of ARFI is feasible even in pediatric patients for the evaluation of cervical lymphadenopathy.

Report of a rare case of histiocytic necrotizing lymphadenitis with bilateral pleural effusion diagnosed via cervical lymph node biopsy

ABSTRACT CONTEXT: Histiocytic necrotizing lymphadenitis (HNL) is a rare disorder that is often benign and self-limiting. There have been reports of co-occurrence of HNL with other diseases, including systemic lupus erythematosus, hemophagocytic syndrome and antiphospholipid syndrome. CASE REPORT: Here, we report a case in which a patient experienced unexplained fever, swelling of the cervical lymph node and bilateral pleural effusion and was ultimately diagnosed with HNL based on results from a lymph node biopsy. After treatment with glucocorticoid, the patient regained normal body temperature, the swelling of the lymph nodes disappeared and the pleural effusion was reabsorbed. CONCLUSIONS: The pathogenesis of HNL remains unclear, and pleural effusion is rarely reported in HNL patients. We presented this case to improve diagnostic awareness of this condition among clinicians and help reduce the likelihood of misdiagnosis.

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.

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.

Clinical Observations of Kikuchi-Fujimoto Disease in Children and Adolescents: A Single Center Experience / 이화의대지

OBJECTIVES: Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fever, and is usually self-limited. This study analyzed the clinical characteristics of pediatric patients with KFD. METHODS: This retrospective, observational, single-center study was conducted in South Korea from March 2008 to October 2015. KFD was diagnosed based on clinical, radiological or histological findings and excluded when there were any other causes of lymphadenopathy. Medical records were reviewed for clinical and laboratory manifestations. RESULTS: A total of 35 cases were included. The mean patient age was 12.1±2.9 years (range, 5 to 17 years); the male-to-female ratio was 1:0.8. The main clinical manifestations were cervical lymphadenopathy and fever in 34 cases (97%). The mean duration of fever was 12.2±8.3 days (range, 2 to 37 days). We noted enlargement of lymph nodes in the cervical, mesenteric (n=5, 14%), axillary (n=2, 6%), and inguinal (n=1, 3%) regions. Hepatosplenomegaly, loss of appetite, and rash were observed. On laboratory examinations, elevation of ferritin, leukopenia, and positivity for anti-nuclear antibodies were frequently observed. Twelve patients underwent biopsy and 23 cases were diagnosed by radiological findings. The mean duration of hospitalization for all cases was 7.9±2.9 days (range, 3 to 13 days) and steroids were administered in 10 cases. KFD recurrence was observed in 2 cases (5.7%) with the time to relapse of 7 months and 4 years. There were no cases with systemic lupus erythematous or other autoimmune disease. CONCLUSION: KFD should be considered in pediatric patients with lymphadenopathy and prolonged fever. Patients with KFD should be monitored for recurrence and the development of autoimmune disease.

Analysis of misdiagnosis of histiocytic necrotizing lymphadenitis / 白血病·淋巴瘤

The histiocytic necrotizing lymphadenitis (HNL) is a common benign lesion in the clinic. Because of its specificity, it is easy to be misdiagnosed as lymphoma or other benign lesions. The author analyzed the reasons of misdiagnosis as follows: (1) pathologists ignored the background of reactive hyperplasia and the specificity of clinical pathological changes; (2) isolate one-sided understanding of nuclear debris in necrotic foci; (3) a false negative result could be presented in HNL gene rearrangement analysis. The pathologists must fully grasp the clinical and histopathological features and immunophenotypic characteristics of HNL to prevent the misdiagnosis.

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.