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)

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.

Enfermedad de Kikuchi-Fujimoto en una paciente con lupus eritematoso sistémico y linfadenopatía generalizada. Reporte de caso / Kikuchi-Fujimoto disease in a patient with systemic lupus erythematosus and generalised lymphadenopathy. Case report

Resumen La enfermedad de Kikuchi-Fujimoto es un trastorno benigno que afecta principalmente a mujeres jóvenes, se caracteriza por adenopatías de predominio en la región cervical, asociadas a fiebre y leucopenia. Aunque de etiología desconocida, hay evidencia de que una infección viral o una enfermedad autoinmune puede desencadenar la enfermedad. Se reporta un caso infrecuente en Colombia de enfermedad de Kikuchi-Fujimoto en una paciente con lupus eritematoso sistémico.

Abstract Kikuchi-Fujimoto disease is a benign disorder that mainly affects young women, and is characterised by predominantly cervical lymphadenopathy associated with fever and leukopenia. Although of unknown aetiology, there is evidence that a viral infection or autoimmune disease can trigger the disease. An uncommon case in Colombia is presented of Kikuchi-Fujimoto disease in a patient with Systemic Lupus Erythematosus.

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.

Doença de Kikuchi-Fujimoto antes do diagnóstico de lúpus eritematoso sistêmico juvenil / Kikuchi-Fujimoto disease prior to childhood-systemic lupus erythematosus diagnosis

A doença de Kikuchi-Fujimoto (DKF) é uma linfadenite necrosante histiocítica autolimitante de origem desconhecida. É digno de nota que a DKF era apenas pouco frequentemente comunicada em pacientes com lúpus eritematoso sistêmico (LES), com rara ocorrência em pacientes com LES juvenil (LESJ). Até onde vai nosso conhecimento, ainda não foi estudada a prevalência de DKF na população pediátrica lúpica. Assim, em um período de 29 anos consecutivos, 5.682 pacientes foram acompanhados em nossa instituição e 289 (5%) satisfaziam os critérios de classificação do American College of Rheumatology para LES; um sofria DKF isolado (0,03%) e apenas um padecia de DKF associada a diagnósticos de LESJ; este caso foi descrito no presente artigo. Uma jovem com 12 anos de idade apresentava-se com febre alta, fadiga e linfadenopatia cervical e axilar. Os anticorpos antinucleares (ANA) estavam negativos, com imunologia positiva para IgM e IgG antivírus do herpes simples tipos 1 e 2. As imagens obtidas por tomografia por emissão de pósitrons com flúor-18-fluoro-desoxi-glicose/tomografia computadorizada (PET/TC) demonstraram linfadenopatia difusa. A biópsia dos linfonodos axilares demonstrou linfadenite necrosante com presença de histiócitos, sem doença linfoproliferativa, compatível com DKF. Transcorridos 30 dias, a paciente apresentou regressão espontânea, não havendo necessidade de tratamento. Nove meses depois, a paciente exibia erupção malar, fotossensibilidade, úlceras orais, linfopenia e ANA 1:320 (padrão nuclear homogêneo). Nessa ocasião, a aplicação do Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) (Índice de Atividade de Doença/LES 2000) teve um escore igual a 10, e a jovem foi tratada com prednisona (1,0 mg/kg/dia) e hidroxicloroquina, demonstrando melhora progressiva dos sinais e sintomas. Em conclusão, DKF é doença benigna e rara em nossa população lúpica pediátrica. Também queremos enfatizar a relevância do diagnóstico de doenças autoimunes durante o acompanhamento de pacientes com DKF.

Kikuchi-Fujimoto disease (KFD) is a self-limiting histiocytic necrotizing lymphadenitis of unknown origin. Of note, KFD was infrequently reported in adult systemic lupus erythematosus (SLE), with rare occurrence in childhood-SLE (C-SLE) patients. To our knowledge, the prevalence of KFD in the paediatric lupus population was not studied. Therefore, in a period of 29 consecutive years, 5,682 patients were followed at our institution and 289 (5%) met the American College of Rheumatology classification criteria for SLE, one had isolated KFD (0.03) and only one had KFD associated to C-SLE diagnoses, which case was reported herein. A 12 year-old female patient had high fever, fatigue and cervical and axillary lymphadenopathy. The antinuclear antibodies (ANA) were negative, with positive IgM and IgG herpes simplex virus type 1 and type 2 serologies. Fluorine-18-fluoro-deoxy-glucose positron emission tomography/computed tomography (PET/CT) imaging demonstrated diffuse lymphadenopathy. The axillary lymph node biopsy showed necrotizing lymphadenitis with histiocytes, without lymphoproliferative disease, compatible with KFD. After 30 days, she presented spontaneous regression and no therapy was required. Nine months later, she developed malar rash, photosensitivity, oral ulcers, lymphopenia and ANA 1:320 (homogeneous nuclear pattern). At that moment the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 10 and she was treated with prednisone (1.0 mg/kg/day) and hidroxychloroquine showing progressive improvement of hers signs and symptoms. In conclusion, KFD is a benign and rare disease in our paediatric lupus population. We also would like to reinforce the relevance of autoimmune diseases diagnosis during the follow-up of patients with KFD.

Tuberculosis ganglionar cervical simulando una enfermedad de Kikuchi: reporte de un caso y revisión de la literatura / Cervical lymph node tuberculosis simulating Kikuchi's disease: a case report and review of the literature

La linfadenitis cervical tuberculosa es la manifestación más común de tuberculosis en cabeza y cuello. La enfermedad de Kikuchi es una enfermedad rara que consiste en una linfadenitis necrotizante histocitiaria. La presentación clínica en ambas entidades suele consistir en una o más masas cervicales dolorosas, que pueden ser duras o fluctuantes. Los hallazgos por imagen no permiten distinguir de manera absoluta la linfadenitis tuberculosa de la enfermedad de Kikuchi. Por lo tanto es imprescindible para interpretar los hallazgos por imagen no sólo conocer el origen demográfico del paciente, sino su estado inmunológico con respecto a la tuberculosis y el estudio histopatológico y microbiológico de las adenopatías.

Tuberculous cervical lymphadenitis is the most common manifestation of tuberculosis in the head and neck. Kikuchi's disease is a rare disease that is histocitiaria necrotizing lymphadenitis. The clinical presentation in both entities is usually a painful neck masses or more, which can be hard or fluctuating. The imaging findings do not distinguish in absolute tuberculous lymphadenitis Kikuchi's disease. Therefore it is essential to interpret the imaging findings not only know the origin of the patient population, but their immune status with regard to Tuberculosis and histopathological and microbiological lymphadenopathy.

Linfadenitis necrotizante histiocitaria: Comunicación de 3 casos / Histiocytic necrotizing lymphadenitis: report of 3 cases

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.

Enfermedad de Kikuchi-Fujimoto: a propósito de un caso / Kikuchi-Fujimoto disease: A propos of a case

La enfermedad de Kikuchi Fujimoto es una forma infrecuente de linfadenitis. En la actual provincia de Mayabeque antes provincia de La Habana no se recoge ningún caso con el referido diagnóstico. Se comunica una paciente femenina de 25 años, color de la piel negra, con diagnóstico de enfermedad de Kikuchi-Fujimoto, que presentaba adenopatías predominantemente en región cervical izquierda. Se realizaron estudios de hematología general, serología viral, exéresis y biopsia del ganglio. La biopsia ganglionar mostró abundantes histiocitos, inmunoblasblos, necrosis celular con polvo nuclear y cariorrexis, así como ausencia de neutrófilos y eosinófilos. Evolucionó favorablemente y actualmente se sigue en consulta de hematología

Kikuchi-Fujimoto disease is a rare form of lymphadenitis. In the current province of Mayabeque formerly Havana Province no cases have been taken up with the referred diagnosis. It is communicated a female patient of 25 years old, black skin color, diagnosed with Kikuchi-Fujimoto disease, who predominantly presented adenopathies in the left cervical region. Studies of general hematology, viral serology, exeresis and ganglion biopsy were performed. ganglionar biopsy showed abundant histiocytes, immunoblasts, cell necrosis with nuclear dust and karyorrhexis, as well as absence of neutrophils and eosinophils. The patient progressed favorably and is currently being monitored in hematology consultation

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.

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.

Linfadenitis necrotizante en lupus eritematoso sistémico y enfermedad de Kikuchi Fujimoto / Necrotizing lymphadenitis in systemic lupus erythematosus and Kikuchi Fujimoto disease

La enfermedad de Kikuchi Fujimoto se caracteriza histológicamente por la presencia de linfadenitis necrotizante, que igualmente se encuentra descrita en Lupus Eritematosos Sistémico, con características clínicas y patológicas comunes que pueden sugerir una posible relación entre estas dos enfermedades. ¿Es la enfermedad de Kikuchi Fujimoto una manifestación del Lupus Eritematoso Sistémico?. A continuación se presenta un caso de una mujer con Lupus Eritematoso Sistémico con linfadenitis necrotizante y linfadenopatía generalizada.

Kikuchi Fujimoto is histologically characterized by the presence of necrotizing lymphadenitis, which is also described in Lupus Erythematosus with common clinical and pathological features that may suggest a possible relationship between these two diseases. Is Kikuchi Fujimoto disease a manifestation of systemic lupus erythematosus? We report a case of a woman with Systemic Lupus Erythematosus with necrotizing lymphadenitis and lymphadenopathy.

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.

Enfermedad de Kikuchi-Fujimoto: Presentación de un caso pediátrico / Kikuchi-Fujimoto disease: Presentation of a pediatric case

Se comunica un paciente masculino de 14 años, blanco, con diagnóstico de enfermedad de Kikuchi-Fujimoto, que presentaba adenopatías predominantemente en región cervical izquierda, fiebre de corto tiempo de evolución y compromiso del estado general sin pérdida de peso. Se hicieron estudios de hematología general, serología viral y biopsia por aspiración con aguja fina de un ganglio cervical. El aspirado fue sospechoso de células neoplásicas. La biopsia ganglionar mostró abundantes histiocitos, inmunoblasblos, necrosis celular con polvo nuclear y cariorrexis, así como ausencia de neutrófilos y eosinófilos. Evolucionó favorablemente con desaparición de la fiebre a los 20 días del inicio de sus manifestaciones con regresión paulatina de las adenopatías.

This is the case of a white patient aged 14 diagnosed with Kikuchi-Fujimoto disease presenting adenopathies prevailing in left cervical region, short-term course fever and involvement of its general condition without weigh loss. Authors made studies of general hematology, viral serology and fine needle aspiration biopsy of a cervical ganglion. There were suspicions of neoplasic cells in aspirate. Ganglion biopsy showed abundant histiocytes, immunoblasts, and cellular necrosis with nuclear powder and caryorrhesis, as well as a lack of neutrophils and eosinophils. There was a favorable course with disappearance of fever at 20 days from the onset of its manifestations with a gradual regression of adenopathies.

Enfermedad de Kikuchi-Fujimoto: presentación de un caso clínico / Kikuchi-Fujimoto disease: case report

La enfermedad de Kikuchi-Fujimoto es una enfermedad de los ganglios linfáticos, habitualmente benigna y autolimitada, que afecta principalmente a adultos jóvenes y se caracteriza poradenopatías (en general cervicales) e hipertermia persistente. Debe diferenciarse de otras causas, infecciosas o tumorales, que cursan con cuadro clínico similar. Su diagnóstico es anatomopatológico (linfadenitis necrotizante histiocitaria) y su tratamiento sintomático. Se presenta un caso clínico de enfermedad de Kikuchi-Fujimotoen una niña de 12 años y se revisa la bibliografía mundial.

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.