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 of the xanthomathous type with cutaneous manifestations

Kikuchi's disease is a benign, self-limiting disease, whose pathogenesis remains unknown. Patients most often present with cervical lymphadenopathy, sometimes associated with fever and leukopenia. It has been reported that up to 40% of patients with Kikuchi's disease have also cutaneous eruptions, but no specific skin changes have been described. Kikuchi's disease can be subclassified into three histologic subtypes: a proliferative type, a necrotizing type and a xantomathous type. Most patients with Kikuchi's disease require no specific treatment, because the disease regresses spontaneously, within a few weeks to months. We report a case of a 31-year-old woman with xanthomatous type of Kikuchi's disease, whose first manifestation was the onset of erythematous papules with central suppuration on her face and on her left hand.

Comparison of Ultrasonographic Findings of Biopsy-Proven Tuberculous Lymphadenitis and Kikuchi Disease

OBJECTIVE: Although tuberculous lymphadenitis and Kikuchi disease are common causes of cervical lymphadenopathy in Asians and exhibit similar clinical manifestations, their treatment strategies are totally different. The purpose of this study was to identify ultrasonographic features that distinguish these two diseases. MATERIALS AND METHODS: This study was approved by the Institutional Review Board. The study included 77 patients with tuberculous lymphadenitis and 135 patients with Kikuchi disease. The sex and age distributions of the patients were analyzed. The size and shape of lymph nodes (LNs), presence of conglomeration, increased perinodal echogenicity, echogenic hilum, posterior neck involvement, internal calcification, patterns of internal necrosis, laterality of involved LNs, and hilar vascular patterns on ultrasonography were compared between the two groups. Multiple logistic regression analysis was conducted to identify independent findings to discriminate tuberculous lymphadenitis from Kikuchi disease. Finally, diagnostic accuracies were calculated using the independent findings. RESULTS: The presence of an echogenic hilum, internal calcification, patterns of internal necrosis, and LN hilar vascular structures on power Doppler ultrasonography were independent findings that discriminated tuberculous lymphadenitis from Kikuchi disease. The diagnostic accuracy of each of these four factors was 84.9% (181/212), 76.9% (163/212), 84% (178/212), and 89.2% (189/212), respectively. A combination of internal calcification and hilar vascular structures showed the best accuracy of 89.6% (190/212) (sensitivity, 86.7% [117/135]; specificity, 94.8% [73/77]) for diagnosing Kikuchi disease. CONCLUSION: The presence of an echogenic hilum, internal calcification, pattern of internal necrosis, and LN hilar vascular structures are useful ultrasonographic findings to differentiate tuberculous lymphadenitis from Kikuchi disease.

A Case of Kikuchi – Fujimoto Disease.

Kikuchi – Fujimoto Disease (KFD) or histiocytic necrotizing lymphadenitis is a rare benign, self-limited cervical lymphadenitis of unknown etiology. Kikuchi first described the disease in 1972 in Japan. Fujimoto and colleagues independently described Kikuchi’s disease in the same year. It pre-dominantly affects young women and can closely mimic infective and immunological disorders. In this article a case of KFD in a young female is described.

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto’s disease

La Enfermedad de Kikuchi-Fujimoto, también llamada Linfadenitis Histiocítica Necrosante, es un sindrome benigno y autolimitado descubierto por primera vez en 1972 por Kikuchi y por Fujimoto, dos patólogos japoneses. Es una enfermedad principalmente descrita en mujeres jóvenes hasta los 40 años de edad que se ha encontrado en todos los grupos étnicos y que puede presentarse en una proporción menor en hombres. La etiología es desconocida, aunque se ha asociado a ciertos microorganismos, y son las infecciones junto con el linfoma los principales diagnósticos diferenciales de esta enfermedad, caracterizada por linfadenopatía cervical con dolor, fiebre leve y síntomas sistémicos. El estudio histopatológico de los ganglios linfáticos permite diferenciar esta enfermedad de otras más graves, aunque es indistinguible histológicamente del Lupus Eritematoso Sistémico con el cual puede presentarse concomitantemente.

Kikuchi-Fujimoto’s disease, also called Histiocytic Necrotizing Lymphadenitis is a benign, self-limited syndrome. It is a disease mainly described in young women under 40 years of age, it has been found in all ethnic groups and may occur in a lesser extent in men. The etiology is not known, although it has been associated with some microorganisms. The differential diagnoses of this disease are infections and lymphoma. This disease is, characterized by cervical lymphadenopathy with pain, mildfever and systemic symptoms. Histopathological study of lymphnodes differentiate this disease from others more serious, although histologically it’s indistinguishable of Systemic Lupus Erythematosus with which can occur concomitantly.

Kikuchi-Fujimoto disease in fine needle aspiration smears: A clinico-cytologic study of 18 pediatric cases and correlation with 68 adult patients.

Objective: Kikuchi-Fujimoto disease (KFD) is a self-limiting disorder which usually affects young women. There are only a few studies on pediatric KFD patients and their fine-needle aspiration (FNA) cytodiagnosis. We report a series of pediatric KFD patients diagnosed by FNA cytology and compare them with adults. Materials and Methods: By routine FNA cytology and through retrospective review smears initially diagnosed as reactive hyperplasia of lymph node during the years 2004-2009, 18 pediatric and 68 adult KFD cases were detected. The clinico-cytologic features of these two groups were compared. Results: The age of the pediatric patients ranged from 6 to 18 years with a median of 13.5 years and adult cases were aged 19 to 54 years with a median of 30 years. Only 1 pediatric case (5.6%) and 20 (29.4%) adult cases were initially diagnosed as KFD (P = 0.0604). Arab:non-Arab ratios were 4.3:1 among the pediatric patients and 1:1.5 for the adults (P = 0.0043). FNA smears were highly cellular in 4 (22.2%) pediatric cases and 37 (54.4%) adult cases (P = 0.0180). More than 5% Kikuchi histiocytes was present in 8 (44.4%) pediatric and 49 (72.1%) adult cases (P = 0.0474). No significant difference was observed in male: female ratio, frequency of head & neck lymphadenopathy, time (season) of presentation, and presence of neutrophils and capillary networks, etc. Conclusions: Besides observation and interpretation errors, a significant difference between the two groups in respect of some clinico-cytomorphological features could have influenced the routine cytodiagnosis leading to lower pickup rate of pediatric KFD cases as compared to adults.

Enfermedad de Kikuchi Fujimoto / Kikuchi-Fujimoto disease

La enfermedad de Kikuchi Fujimoto o linfadenitis histiocitaria necrotizante es una afección benigna, autolimitada, de etiología desconocida y poco frecuente. Se presenta el caso de una mujer de 16 años sin antecedentes de jerarquía que consulta por fiebre y adenopatías cervicales. En el examen físico se constata paciente febril (38.5 °C) con adenopatías cervicales múltiples, bilaterales, duro-elásticas; orofaringe congestiva y esplenomegalia leve sin otros hallazgos patológicos de jerarquía. El laboratorio evidencia pancitopenia y elevación de la velocidad de eritrosedimentación, de la β2 microglobulina, las transaminasas y la lacticodeshidrogenasa. Se efectúan múltiples investigaciones serológicas que resultan negativas, excepto la de inmunoglobulina G para parvovirus, positiva. En la tomografía axial computarizada de cabeza, cuello, tórax, abdomen y pelvis se observan adenomegalias laterocervicales bilaterales, de 15 mm las de mayor tamaño, y axilares bilaterales de menos de 10 mm de diámetro; pequeñas condensaciones pulmonares basales posteriores con derrame pleural leve, esplenomegalia leve homogénea, escaso líquido libre en cavidad abdominal en el fondo de saco de Douglas. Se trata con ampicilina/sulbactam asociado a doxiciclina por posibles infecciones respiratoria y ginecológica. La paciente evoluciona con persistencia de los síntomas. Se realiza biopsia ganglionar que evidencia morfología compatible con enfermedad de Kikuchi Fujimoto. Se inicia tratamiento con prednisona con mejoría clínica y de los exámenes de laboratorio. La enfermedad de Kikuchi Fujimoto es probablemente subinformada y subdiagnosticada debido al bajo índice de sospecha. Al estar más informados, los médicos clínicos podrían investigar y diagnosticar los casos que hoy posiblemente no se detectan.

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare, benign, self-limited condition of unknown etiology. A 16-year-old woman with no relevant clinical history seeks medical attention for fever and cervical adenopathies. In a physical examination the patient is found to be febrile (38.5 °C) with multiple bilateral, hard-elastic cervical adenopathies, congestive oropharynx and mild splenomegaly with no other relevant pathological findings. The laboratory examination shows signs of pancytopenia and an increase in the erythrocyte sedimentation rate, β2-microglobulin, transaminases and lactate dehydrogenase. Several serological tests are carried out with negative results except for immunoglobulin G for parvovirus, which is positive. A computerized axial tomography scan of head, neck, thorax, abdomen and pelvis shows bilateral lateral cervical adenomegalies (the largest with a size of 15 mm) and bilateral axillary adenomegalies of less than 10 mm in diameter; small basal lung condensations with mild pleural effusion, mild homogeneous splenomegaly, and a small amount of free fluid in the abdominal cavity at the bottom of the Douglas cul-de-sac. Possible respiratory and gynecological infections are treated with ampicillin/sulbactam together with doxycycline. The patient progresses with persistence of the symptoms. A lymph node biopsy is then performed, revealing a morphology compatible with Kikuchi-Fujimoto disease. Treatment with prednisone is initiated with a clinical and laboratory improvement in the patient's condition. Kikuchi-Fujimoto disease is probably under-reported and under-diagnosed due to the low index of suspicion. Greater awareness of this illness would result in clinicians arriving at this diagnosis more often.

[Kikuchi-Fujimoto's disease]

The necrotizing histiocyte lymphadenitis is a disease of unknown cause. Clinicians should be aware of this uncommon disease in order to differentiate it from lymphoma, infectious adenitis, and collagenosis. A 24-year-old woman consulted for a right cervical mass which had developed rapidly with no associated symptom. Laboratory results were normal. The gangular biopsy confirmed the diagnosis of necrotizing hymphanditis. Necrotizing histiocyte lymphadenitis is generally observed in young women in their twenties. There has been no predominant ethnic backgrouns. Clinically, it is characterized by lymphadenitis of one or more lymph nodes, predominantly in the cervical region. The Laboratory tests are normal excepting for discrete signs of inflammation. Diagnosis anatomopathologic, the characteristic features include focal necrosis predominantly in the paracortical region with abundant karyorrhectic debris and atypical mononuclear cells around the necrotic zone [crescent-shaped histiocytes, plasmacytoid monocytes, and small lymphocytes and immunoblasts]. The clinical course is generally spontaneously favorable in six months. Relapse is uncommon. The clinicopathologic picture can be confused with a malignant blood disease particularly lymphoma. Evolution is benign and healing without treatment is the rule

Kikuchi-Fujimoto disease: an unusual association with acute renal failure

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis of unknown etiopathogenesis, is a self-limited disease which frequently appears as feverish lymphadenomegaly, thus creating the need for differential diagnosis with lymphoma, systemic lupus erythematosus (SLE), infectious mononucleosis, cat-scratch disease, and toxoplasmosis with lymphonodal impairment. However, there are cases in which it may evolve with complications such as aseptic meningitis, cerebellar ataxia, and aseptic myocarditis. We are presenting a case of a 24-year-old man who had an initial picture of arthralgia, evening fever and adenomegaly. Kikuchi disease was diagnosed through lymph node biopsy with immunohistochemistry and evolves with severe systemic manifestations, such as pericarditis with cardiac tamponade, pneumonitis, hepatitis, and acute kidney failure - the latter has not been reported in literature yet. There was significant improvement of the clinical picture with prednisone.

Kikuchi-Fujimoto disease: three case reports / Doença de Kikuchi-Fujimoto: relato de três casos

CONTEXT: Kikuchi-Fujimoto disease (KFD) manifests in most cases as unilateral cervical lymphadenomegaly, with or without accompanying fever. The disease mainly affects young women and has a self-limited course. It is more common in oriental countries, with few reports of its occurrence in Brazil. KFD should be included in the differential diagnosis of suspected cases of viral infections, tuberculosis, reactive lymphadenitis, systemic lupus erythematosus and metastatic diseases. It can be histologically confused with lymphoma. The disease is benign and self-limiting and an excisional biopsy of an affected lymph node is necessary for diagnosis. There is no specific therapy. CASE REPORTS: This study reports on three cases of non-Asian female patients with KFD who were attended at our service between 2003 and 2006. A review of the literature was carried out, with a systematic search on this topic, with the aim of informing physicians about this entity that is manifested by cervical masses and fever.

CONTEXTO: A doença de Kikuchi-Fujimoto (DKF) se manifesta na maioria das vezes como linfadenomegalia cervical unilateral, que pode ser acompanhada de febre. Afeta principalmente mulheres jovens, com evolução autolimitada. A prevalência desta doença é mais comum no oriente, havendo poucos relatos de sua ocorrência em nosso meio. DKF deve ser incluída no diagnóstico diferencial de casos suspeitos de infecções por vírus, tuberculose, linfadenite reacional, lúpus eritematoso sistêmico e doença metastática. Histologicamente pode ser confundida com linfoma. A doença é de caráter benigno, auto-limitada, a biópsia excisional do linfonodo acometido é necessária para o diagnóstico e não há tratamento específico. RELATO DE CASOS: Este trabalho relata três casos de pacientes não asiáticas do sexo feminino com DKF, atendidas em nosso serviço entre 2003 e 2006, e faz revisão da literatura com uma busca sistematizada sobre o assunto, com o objetivo de informar os médicos sobre essa entidade que se manifesta com massa cervical e febre.

Kikuchi's Disease in Children: Clinical Manifestations and Imaging Features

Previously published studies on Kikuchi disease (KD) have frequently addressed the computed tomography (CT) findings in the adult population, however, only a few studies have been reported for the pediatric age group. The purpose of this study is to analyze the clinical characteristics and imaging features of KD in children. Fifteen children (2-14 yr) who had a neck CT and pathology diagnosis of KD were included in this study. Clinical features, including the duration of lymphadenopathy and fever, prognosis, and laboratory values, were evaluated. We analyzed the sites, size, and lymph node pattern as seen on their CT scans. The median duration of fever was 10 days. Fourteen patients experienced improvement in their condition, although four of these patients experienced recurrent episodes of KD. All patients had affected cervical nodes at level V. Perinodal infiltrates were observed in the affected cervical nodes in 14 cases (93%), and non-enhancing necrosis was also noted within the affected cervical nodes in 10 cases (63%). In conclusion, the combination of imaging findings in conjunction with clinical findings of KD may help to determine whether or not to perform pathology analysis and follow-up studies.

Doença de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

A doença de Kikuchi-Fujimoto é caracterizada por febre e linfadenopatia, geralmente cervical. Esta doença acomete principalmente mulheres jovens. Pode ser confundida com linfoma, metástase de adenocarcinoma e tuberculose. Relatamos dois casos da doença de Kikuchi-Fujimoto. No primeiro caso, uma paciente de 28 anos havia tratado tuberculose há um ano e apresentava quadro clínico e histológico compatível com a doença de Kikuchi-Fujimoto. A segunda paciente, de 58 anos, recebeu tratamento inicialmente para granulomatose de Wegener e, posteriormente, para tuberculose. O exame histopatológico com estudo imunohistoquímico permitiu estabelecer o diagnóstico da doença de Kikuchi-Fujimoto nos dois casos. Após o diagnóstico definitivo, ambas foram tratadas sintomaticamente e melhoraram clinicamente dentro de um mês. Posteriormente, a segunda paciente desenvolveu lúpus eritematoso sistêmico.

Kikuchi-Fujimoto disease is characterized by fever and lymphadenopathy, usually localized in the cervical region. This disease principally affects young females. It can be confused with lymphoma, adenocarcinoma metastasis and tuberculosis. We report two cases of Kikuchi-Fujimoto disease. In the first case, a 28-year-old female had been treated for tuberculosis one year prior and presented with a clinical and histological profile compatible with Kikuchi-Fujimoto disease. The second patient, a 58-year-old female, initially received treatment for Wegener's granulomatosis and, subsequently, for tuberculosis. Histopathological examination followed by immunohistochemical analysis confirmed the diagnosis of Kikuchi-Fujimoto disease in both cases. After the definitive diagnosis had been made, both patients were treated symptomatically, and both presented clinical improvement within one month. Subsequently, the latter patient developed systemic lupus erythematosus.

Tumor cervical por enfermedad de Kikuchi: Una entidad clínica a la que no estamos habituados. Caso clínico / Histiocytic necrotizing lymphadenitis: Report of three cases

Histiocytic necrotizing lymphadenitis (HNL), also known as Kikuchi 5 disease is a rare condition of unknown etiology. Patients present with cervical lymph node enlargement, fever and malaise. The diagnosis is made by excision biopsy. However, this entity must be distinguished from both reactive processes and malignant tumors such as lymphoma. The clinical course is self limited with spontaneous resolution within a few months. We report three patients with the disease. A 37 year-old woman with a 4 months history of a painless submaxillary mass of 2.5 cm diameter, attached to the deep tissues of the neck. The mass was excised and the biopsy report was HNL. After 26 months of follow up, the patient is asymptomatic. A 30 year-old woman with a history of 2 months of a painless lateral cervical mass and aspiration biopsy was reported as suspicious for lymphoma. An excision biopsy was performed, that was reported as HNL. In both patients, lymphoma was ruled out by immunohistochemistry. A 33 year-old woman with a 3 weeks history of an asymptomatic lateral cervical mass. Biopsy was reported as HNL. This condition must be included in the differential diagnosis of cervical asymptomatic masses. The clinician must be aware of it to avoid long-term, costly treatments.

Two case reports of Kikuchi-Fujimoto disease

Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare but important etiology of cervical lymphadenopathy in young women. This disease is self limited and improves during 2-8 months. Diagnostic key is the pathology of involved lymphnode. Association of the disease with viral and autoimmune disease is not proved yet. In this article two cases of Kikuchi-Fujimoto disease are reported. The first case was a 31 years old female patient with sever fever and right axillary lymphodenpathy [size: 5x7cm] in which one month after the disease, diagnosis was proved by pathology studies. This patient has erythematous patches in her face and nose in several times that were improved spontaneously. The second case was a 20 years old female patient with fever, chiH, headache, weigh loss and cervical lymphadenopathy [size: 2x1 cm]. She received antibiotic at several times without any improvement. Finally after 5 months since the beginning of symptoms, the disease was diagnosed as Kikuchi-Fujimoto disease by surgical excision of lymhnode and pathology studies. After the surgery two small lymphnodes were appeared around the surgical site. In differential diagnosis of lymphadenpathy especially cervical in young women, Kikuchi-Fujimoto should be considered. Consumption of antibiotic in lymphadenopathy without clear diagnosis should be avoided. After diagnosis of Kikuchi-Fujimoto, long term work up for recurrence or the appearance of autoimmune disease symptoms is necessary

Enfermedad de Kikuchi-Fujimoto (linfadenitis histiocítica necrosante): Estudio clinicopatológico e inmunohistoquímico de 14 casos y su diagnóstico diferencial con otras linfadenitis necrosantes reactivas y neoplásicas / Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenopathy) clinicopathologic and immunohistochemical study of 14 cases and its differential diagnosis with other reactive and neoplastic necrotizing lymphadenopathies

Kikuchi-Fujimoto disease also known as histiocytic necrotizing lymphadenopaty (HNL) is a rare entity, originally described in Japanese population, although currently it has been described all over the world. It is more frequent in young women and it is usually located in cervical lymph nodes. We report 14 cases of HNL in Mexican population, their clinicopathological and immunohistochemical study as well as a comparative study with other necrotizing lymphadenopaties due to B or T-cell lymphomas, tuberculosis, Epstein Barr virus infection, and non-specific necrosis. In our study we found that there was more expression of the immunomarkers CD68, MPO, CD123 and antikerat in OSCAR in the cases of HNL in contrast with the lesser or even null expression of the same markers in the necrotized lymph nodes of the comparative study group.

La enfermedad de Kikuchi-Fujimoto o linfadenitis histiocítica necrosante (LHN) es una entidad poco frecuente, inicialmente descrita en población japonesa. Sin embargo, actualmente se ha descrito alrededor de todo el mundo. Es más frecuente en mujeres jóvenes y su localización habitual es en ganglios linfáticos cervicales. Presentamos estudio clinicopatológico de 14 casos de LHN en pacientes mexicanos, y los comparamos con linfadenitis necrosantes secundarias a linfomas B y T, tuberculosis, virus de Epstein Barr y necrosis inespecífica. Encontramos mayor expresión de los anticuerpos CD68, MPO, CD 123 y antiqueratina OSCAR en los casos de LHN comparados con la expresión baja o nula de los mismos anticuerpos en las necrosis ganglionares del grupo comparativo.

Kikuchi-Fujimoto disease: a rare cause of cervical lymphadenopathy.

Kikuchi-Fujimoto Disease (KFD) is a self-limiting, necrotizing, histiocytic lymphadenitis (NHL) of unknown etiopathogenesis. So far in Nepal not a single case of this disease in the cervical region leading to progressive inspiratory stridor has been reported till date. Here a case of 26 years old Nepalese house wife suffering from this disease, which is histopathologically proved as KFD with complete recovery is presented.

Enfermedad de Kikuchi-Fujimoto en pediatría: Reporte de un caso y revisión de la literatura / Kikuchi-Fijimoto disease in pediatric population: a case report and a review of literature

Un niño de 10 años de edad fue ingresado al Hospital Nacional de Niños "Dr. Carlos Luis Sáenz Herrera" con una masa cervical derecha de aproximadamente cuatro semanas de evolución. Se realizaron pruebas de gabinete y laboratorio que descartaron malignidad, infecciones o procesos inmunológicos. Una biopsia a cielo abierto reportó la presencia de granulomas con necrosis central, abundante cariorrexis, histiocitos, linfocitos y células gigantes multinucleadas, sin neutrófilos. Las tinciones especiales no mostraron ningún microorganismo. En valoraciones posteriores al mes, a los seis y 12 meses el niño continuaba asintomático. Por no tener etiología precisa, no se administró ningún medicamento. Con base en la evolución clínica, los hallazgos de la biopsia y al excluirse causas infecciosas, tumorales e inmunológicas, se concluyó que el paciente presentó una enfermedad de Kikuchi Fujimoto, siendo el primer caso pediátrico reportado en nuestro país.

A 10 year-old child, with a history of a right cervical mass, is admitted to the Costa Rican National Children's Hospital for workup. The mass appeared approximately 4 weeks before admission. Laboratory tests were performed and malignity, infection and immunologic causes were ruled out. A biopsy was performed revealing granulomas characterized by central necrosis with abundant karyorrhexis, surrounded by histiocytes, lymphocytes and giant multi-nucleated cells, without neutrophils. Special stains showed no microorganisms. Once infectious and immunologic causes were excluded, and based on the biopsy's result, treatment was ruled out. Twelve months later, the patient is still asymptomatic; therefore, the diagnosis of a Kikuchi-Fujimoto syndrome was proposed. This report constitutes the first pediatric case diagnosed in our country.