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OBJECTIVE: To outline the characteristics of Kikuchi-Fujimoto disease (KFD) in children and analyze factors associated with severe and recurring courses. METHODS: Electronic medical records of children histopathologically diagnosed with KFD at Seoul National University Bundang Hospital from March 2015 to April 2021 were retrospectively reviewed. RESULTS: A total of 114 cases (62 males) were identified. The mean patient age was 12.0 ± 3.5 years. Most patients came to medical attention with cervical lymph node enlargement (97.4%) and fever (85%); 62% had a high-grade fever (≥39°C). Prolonged fever (≥14 days) was seen in 44.3% and was associated with a high-grade fever (P = .004). Splenomegaly, oral ulcer, or rash was present in 10.5%, 9.6%, and 15.8%, respectively. Laboratory findings showed leukopenia, anemia, and thrombocytopenia in 74.1%, 49%, and 24%, respectively. Sixty percent of cases had a self-limited course. Antibiotics were initially prescribed in 20%. A corticosteroid was prescribed in 40% of patients and was associated with oral ulcer (P = .045) and anemia (P = .025). Twelve patients (10.5%) had a recurrence with a median interval of 19 months. No risk factor for recurrence was identified in multivariable analysis. Clinical characteristics of KFD were similar between our current and previous studies. However, antibiotics use decreased (P < .001); nonsteroidal anti-inflammatory drugs use increased (P < .001), and, although statistically not significant, corticosteroid treatment also increased. CONCLUSIONS: Over a span of 18 years, the clinical characteristics of KFD did not change. Patients presenting with high-grade fever, oral ulcer, or anemia may benefit from corticosteroid intervention. All patients should be monitored for recurrence.

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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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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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OBJECTIVE: To analyze the clinical characteristics and prognosis of pediatric hemophagocytic lymphohistiocytosis (HLH) associated with histiocytic necrotizing lymphadenitis (HNL). STUDY DESIGN: We retrospectively collected the clinical data of all children with HNL-HLH enrolled in Beijing Children's Hospital from 2007 to 2019. The control patients with Epstein-Barr virus-associated HLH and simple HNL (not associated with HLH) were case matched (1:2). The clinical features and prognosis were analyzed by case-control study. Cases of HNL-HLH in the literature were reviewed. RESULTS: The male-to-female ratio of the 13 patients in our center was 9:4. The mean age of the patients at disease onset was 8.1 ± 1.2 years, younger than that of the 16 patients in the literature (P = .017). Clinical presentations, especially rash and splenomegaly, and laboratory examination of HNL-HLH group were statistically different from Epstein-Barr virus-HLH group, simple HNL group, and patients reported in the literature (P < .05). Three patients were treated with immunosuppressive drugs or chemotherapy owing to poor control of HLH. One patient died, and all 12 remaining patients survived, 2 of which developed autoimmune diseases. Kaplan-Meier survival curves showed no statistical difference among the 3 groups (P > .05). CONCLUSIONS: HNL-HLH is more common in school- and preschool-age children. Most patients have a favorable prognosis. Some patients suffer from relapses or develop autoimmune diseases. Prolonged follow-up should be carried out for patients with HNL-HLH.

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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)

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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.

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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.

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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.

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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.

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The Kikuchi-Fujimoto disease is a rare disease that occurs mostly in young adults, although some cases have been reported in children. It is usually characterized by fever and cervical lymphadenopathy. The etiology of the disease remains unknown. Its course is usually benign and self-limited. It has special histopathological features that allow the differential diagnosis with other entities, which from a clinical point of view can be very complicated. We report a 9 years 11 months old girl with lymphadenopathy and fever with five months evolution, which is the longest evolution among the cases reviewed by the authors in world literature. Given that the presentation of this disease in children is very rare, we estimate that the knowledge of this disease is relevant and pediatricians must consider it in the differential diagnosis of fever of unknown origin in children.

La enfermedad de Kikuchi-Fujimoto es una entidad rara que se presenta principalmente en adultos jóvenes, aunque se han descrito algunos casos en la edad pediátrica. Se caracteriza por adenopatías generalmente cervicales y fiebre. La etiología de la enfermedad permanece desconocida. Su curso es generalmente benigno y autolimitado. Tiene características histopatológicas especiales que permiten el diagnóstico diferencial con otras entidades, lo que desde el punto de vista clínico puede ser muy complicado. Presentamos el caso de una niña de nueve años y 11 meses que cursó con adenopatías y fiebre de cinco meses de evolución. Este tiempo es el más prolongado entre los casos revisados por los autores en la literatura mundial (búsqueda en PubMed y SciELO por “Kikuchi-Fujimoto Disease”, “histiocytic necrotizing lymphadenitis”). Dado que la presentación de esta enfermedad en la edad pediátrica es muy rara, estimamos pertinente su conocimiento y consideración en el diagnóstico diferencial de la fiebre de origen desconocido en niños.

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Se presentó un caso de una gestante de 26 años, de raza negra y residente de Nueva Gerona en el municipio especial la Isla de la Juventud. Esta paciente fue diagnosticada hace 11 años con un antecedente patológico personal de la enfermedad Kikuchi Fujimoto en el Hospital Clínico Quirúrgico "Hermanos Ameijeiras" de Ciudad de la Habana. Tiene como antecedente obstétrico de primigrávida, fecha de última menstruación 27 de julio de 2012; tuvo un parto eutócico simple y espontáneo, con 39,4 semanas de embarazo el 29 de marzo de 2013. Fue necesaria una estrecha atención prenatal y vigilancia durante el parto y puerperio, por tratarse de una embarazada con la enfermedad Kikuchi Fujimoto, poco conocida e infrecuente; resultó el primer caso en la Isla de la Juventud. La evolución fue favorable, apoya el carácter benigno y autolimitado de la enfermedad y su tratamiento exclusivamente sintomático.

A case of a 26 year-old, black pregnant woman from Nueva Gerona special municipality of Isla de la Juventud (Youth Island) is presented. The patient was diagnosed 11 years before with personal pathological history of Kikuchi-Fujimoto disease at Hermanos Ameijeiras University Hospital in Havana city. As obstetric history she was a primigravida, last date of period July 27th, 2012; she had a spontaneous eutocic delivery with 39, 4 weeks of pregnancy on March 29th, 2013. As she suffered from Kikuchi-Fujimoto disease, a rare and infrequent entity; prenatal follow-up and supervision during delivery and puerperium was carried out. It was the first case report of the Youth Island. The patient had a favorable evolution, supporting the benign and self-limited features of the disease, and the symptomatic treatment exclusively.

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Histiocytic necrotizing lymphadenitis (also known as Kikuchi-Fujimoto's disease) is an uncommon, benign and self-limiting lymph disease. We report three patients aged 14, 22 and 50 years, who presented with fever and cervical lymphadenopathy, accompanied by skin lesions and joint pain in two of the three cases. One of the patient's sister suffered from histiocytic necrotizing lymphadenitis. Laboratory abnormalities varied and findings included leucopenia, relative neutrophilia, elevated C reactive protein, erythrocyte sedimentation rate and ferritin. Basic laboratory screening tests were performed on all patients to rule out autoimmune and infectious diseases. Lymph node biopsy and subsequent pathological examination were essential to establish the diagnosis. All patients received antibiotics at some point of their hospital stay. Two patients required glucocorticoid treatment, while the remaining case experienced a spontaneous recovery. Its pathogenesis is still unknown, but clinical and histopathological studies suggest a connection with autoimmune diseases. There is no established treatment, but apparently the disease responds to the administration of glucocorticoids.

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A doença de Kikuchi-Fujimoto é uma linfadenite necrosante rara de bom prognóstico, cujo diagnóstico diferencial inclui principalmente as neoplasias linfoides. Relata-se um caso peculiar de doença de Kikuchi-Fujimoto em paciente jovem, do sexo masculino, com acometimento inguinal bilateral e diagnóstico histopatológico.

The Kikuchi-Fujimoto disease is a rare necrotizing lymphadenitis of good prognosis, differential diagnosis includes mainly lymphoid neoplasms. We report a peculiar case of Kikuchi-Fujimoto disease in a young patient, male, with bilateral inguinal involvement and histopathological diagnosis

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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.

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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.

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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.(AU)

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 patients 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.(AU)

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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.(AU)

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 patients 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.(AU)

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RESUMO

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.

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RESUMO

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.

Assuntos