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

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

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

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto disease

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

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

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

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

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

Kikuchi-Fujimoto disease: pediatric case report.

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.

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.

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

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 (1.0mg/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.

Doença de Kikuchi-Fujimoto: relato de caso / Kikuchi-Fujimoto disease

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

Linfoadenitis histiocítica necrosante en una mujer joven / Necrosing histiocytic lymphadenitis in a young woman

Se describe el caso clínico de una paciente de 32 años de dad, que acudió a consulta por presentar cuadro febril y adénico desde hacía varias semanas, asociado a manifestaciones generales de anorexia y pérdida de peso. En el examen clínico se constató la presencia de adenopatías cervicales de 2 cm y orofaringe enrojecida. Los resultados de los exámenes complementarios efectuados y la evaluación histológica del ganglio linfático confirmaron que se trataba de una linfoadenitis histiocítica necrosante en fase proliferativa (enfermedad de Kikuchi Fujimoto). El estudio inmunohistoquímico no reveló malignidad.

The case report of a 32 year-old patient is described who attended the department for presenting adenoid and febrile condition for several weeks, associated with general manifestations of anorexy and weight loss. In the clinical examination the presence of 2 cm cervical adenopathies and reddened oropharynx was confirmed. The results of the complementary tests and the histological evaluation of the lymph node confirmed that it was a necrosing histiocytic lymphadenitis in proliferative stage, (Kikuchi Fujimoto disease). The immunological histological and chemical study did not reveal malignancy.

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.

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.(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)

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.(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)

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.

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 (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.

Linfadenitis histiocítica necrosante (enfermedad de Kikuchi-Fujimoto) / Histiocytic necrotizing lymphadenitis (Kikuchi -Fujimoto's disease)

Introducción. La enfermedad de Kikuchi-Fujimoto es una linfadenitis histiocítica necrosante poco frecuente, sobre todo en la edad pediátrica. Caso clínico. Niña de 9 años de edad con historia de fiebre, adenomegalias y pérdida de peso que requirió biopsia de ganglios, reportándose una linfadenitis necrosante (enfermedad de Kikuchi-Fujimoto); respondió a antiinflamatorios, pero presentó una recaída 5 años después. Conclusión. Debe sospecharse enfermedad de Kikuchi-Fujimoto en todo paciente con fiebre, adenomegalias, pérdida de peso y mal estado general, sobre todo cuando se ha descartado etiología infecciosa y un proceso oncológico. El diagnóstico definitivo es por medio de biopsia de alguno de los ganglios afectados.

Introduction. Kikuchi-Fujimoto's disease is a histiocytic necrotizing lymphadenitis, not frequent in children. Case report. Nine year-old girl with history of fever, adenomegaly and loss of weight that required a lymph node biopsy which was interpreted as necrotizing lymphadenitis (Kikuchi-Fujimoto's disease), she responded to anti inflammatory medication but presented a relapse 5 years later. Conclusion. Kikuchi-Fujimoto's disease should be suspected in the patients with fever, lymphadenitis, and weight loss after infectious and malignant etiologies have been ruled out. The definitive diagnosis is skin biopsy.

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.