Clinical features of histiocytic necrotizing lymphadenitis in children.

Due to its nonspecific clinical characteristics, histiocytic necrotizing lymphadenitis (HNL) is often misdiagnosed as a suppurative cervical lymphadenitis and lymphoma. Thus, this study aimed to investigate the clinical characteristics of HNL in pediatric patients. We retrospectively identified 61 patients with histopathologically confirmed HNL. Clinical and laboratory data, including age, sex, clinical manifestations, laboratory investigations, histological discoveries, treatment, and outcomes, were collected from the medical records to determine associations with extracervical lymph node (LN) involvement. The mean age of patients was 9.7 ± 2.8 years (range, 1.5-14.0 years), and the male-to-female ratio was 2.2:1. The most common systemic symptom was fever in all patients. The median pre-admission and total durations of fever were 13.0 (interquartile range [IQR]: 9.0-22.5 days) and 22.0 days (IQR: 17.0-33.0 days), respectively. Patients with temporary fever (< 2 weeks) had a higher peak temperature and were more likely to undergo LN biopsy after admission than those with a prolonged fever (≥ 2 weeks). Multivariate analysis revealed that peak temperature ≥ 40 °C was significantly associated with a longer fever duration (P = 0.023). Laboratory values showed leukopenia (68.9%), which presented more frequently in solitary cervical LNs than in extracervical LNs (82.4% vs. 52.9%, p = 0.027) in patients with prolonged fever. CONCLUSIONS: HNL is often misdiagnosed in older children with persistent fever and lymphadenopathy, leading to unnecessary diagnostic tests and evaluations, inappropriate antibiotic administration, and mismanagement. A multidisciplinary team, including primary care providers, rheumatologists, and pathologists, can improve patient outcomes by increasing their awareness of this rare condition. WHAT IS KNOWN: • Histiocytic necrotizing lymphadenitis (HNL) is characterized by fever, leukopenia, and neck lymphadenopathy with unknown etiology. • The lack of neutrophils or eosinophils in the histology, immunohistochemistry results help distinguish HNL from infectious causes. Although HNL is a self-limiting disease, antibiotics and steroid treatments were used inappropriately. WHAT IS NEW: • A fever peak ≥ 40 °C was associated with a longer fever duration in HNL patients. Leukopenia presented more frequently in solitary cervical lymph node (LNs) than in extracervical LNs inpatients with prolonged fever. • Steroids are not recommended as a routine treatment, however, in some severe or relapsing cases with persistent symptoms, prednisolone (5 mg twice a day for 2 days) or other steroids (an equivalent dose of prednisolone) responded favorably.

The diagnosis of nontuberculous cervicofacial lymphadenitis: A systematic review.

BACKGROUND: Nontuberculous mycobacterial cervicofacial lymphadenitis (NTMCL) is an uncommon condition detected in young immunocompetent children who typically present with a nontender neck mass. Various tests have been proposed to assist in the work-up of suspected NTMCL, with varying diagnostic utility. This systematic review investigates the sensitivity of the various diagnostic methods used in the work-up of pediatric NTMCL. METHODS: A systematic review in accordance with PRISMA guidelines was performed using the Pubmed, EMBASE, and Web of Science databases. Searches were filtered for English language studies published prior to 05/10/22. Studies meeting criteria included studies featuring 15+ pediatric patients with confirmed or suspected NTMCL. Studies with any reported diagnostic methodology used in the workup of NTMCL were included. RESULTS: Of 836 abstracts/articles reviewed, 21 studies met inclusion criteria. Diagnostic methods included culture(n = 11 studies), PPD-Tb(Tuberculin)(n = 12), PPD-Scrofulaceum, -Avium, or -Kansasii(n = 6), staining techniques(n = 4), IGRA(n = 3), and ultrasound(n = 2). All studies had an overall low risk of bias. Among patients confirmed to have NTMCL based on PCR and/or culture, the most sensitive tests were PPD-A(0.94, 95 % CI 0.91 to 0.97; n = 210 patients) and PPD-S(0.75, 95 % CI 0.68 to 0.81; n = 171). Auramine and Ziehl-Neelsen staining techniques had moderately high sensitivity(0.85 and 0.60 respectively), though were limited by low patient numbers(n = 20). PPD-Tb(0.45, 95 % CI 0.39 to 0.50; n = 300) and IGRA(0.02; 95 % CI 0 to 0.06; n = 48) demonstrated poor sensitivity. Among patients suspected to have NTM lymphadenitis based on global assessment, the most sensitive tests included combined PPD-S + A + K(0.92, 95 % CI 0.86 to 0.98; n = 85), PCR(0.82, 95 % CI 0.75 to 0.88; n = 136), and PPD-A(0.72, 95 % CI 0.62 to 0.81; n = 84). Culture showed a sensitivity of 0.54(95 % CI 0.50 to 0.58; n = 494). PPD-K, PPD-S, IGRA, and staining techniques demonstrated lower sensitivity. CONCLUSIONS: This systematic review is the largest study investigating the sensitivity of the various diagnostic methods used in the work-up of pediatric NTMCL. Patients with clinical suspicion for NTMCL and a positive PPD-Tb should first have tuberculous lymphadenitis ruled out with IGRA. Patients with a positive PPD-Tb and negative IGRA and high clinical suspicion for NTMCL can undergo presumptive surgical intervention. Patients with a negative PPD-Tb can undergo NTM antigen skin testing if available, or if high clinical suspicion exists, surgical intervention to reduce tissue burden and elicit additional tissue data.

Isolated unilateral hypoglossal nerve palsy from suppurative retropharyngeal lymphadenitis in an infant.

Retropharyngeal infections (RPIs) are uncommon in young infants and are difficult to diagnose due to their non-classical presentation. RPI can occasionally be complicated with multiple cranial nerve palsies but rarely in isolation. Isolated hypoglossal nerve palsy (HNP) due to RPI has been described in the literature but mostly in older children and adults. Assessment for hypoglossal nerve function is challenging in a young infant because the conventional signs of hypoglossal nerve dysfunction are difficult to elicit in this age group. Early recognition and treatment of RPI are associated with good HNP recovery. We present a case of a young infant with tongue deviation and difficulty with feeding attributed to an isolated HNP caused by suppurative retropharyngeal lymphadenitis. The infant underwent incision and drainage with complete recovery of the tongue function after 8 weeks.

Development and Evaluation of an NTM-IGRA to Guide Pediatric Lymphadenitis Diagnosis.

BACKGROUND: Diagnosis of nontuberculous mycobacteria (NTM) infections remains a challenge. In this study, we describe the evaluation of an immunological NTM-interferon (IFN)-γ release assay (IGRA) that we developed using glycopeptidolipids (GPLs) as NTM-specific antigens. METHODS: We tested the NTM-IGRA in 99 samples from pediatric patients. Seventy-five were patients with lymphadenitis: 25 were NTM confirmed, 45 were of unknown etiology but compatible with mycobacterial infection and 5 had lymphadenitis caused by an etiologic agent other than NTM. The remaining 24 samples were from control individuals without lymphadenitis (latently infected with M. tuberculosis , uninfected controls and active tuberculosis patients). Peripheral blood mononuclear cells were stimulated overnight with GPLs. Detection of IFN-γ producing cells was evaluated by enzyme-linked immunospot assay. RESULTS: NTM culture-confirmed lymphadenitis patient samples had a significantly higher response to GPLs than the patients with lymphadenitis of unknown etiology but compatible with mycobacterial infection ( P < 0.001) and lymphadenitis not caused by NTM ( P < 0.01). We analyzed the response against GPLs in samples from unknown etiology lymphadenitis but compatible with mycobacterial infection cases according to the tuberculin skin test (TST) response, and although not statistically significant, those with a TST ≥5 mm had a higher response to GPLs when compared with the TST <5 mm group. CONCLUSIONS: Stimulation with GPLs yielded promising results in detecting NTM infection in pediatric patients with lymphadenitis. Our results indicate that the test could be useful to guide the diagnosis of pediatric lymphadenitis. This new NTM-IGRA could improve the clinical handling of NTM-infected patients and avoid unnecessary misdiagnosis and treatments.

[Recurrent lymphadenitis was Kikuchi-Fujimoto disease]. / Lymfadenit som mystiskt återkom var Kikuchi­Fujimotos sjukdom.

Kikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is in most cases a benign disease which affects lymph nodes in the cervical region. Cervical adenopathy and fever are the most common symptoms, and young adults are mostly affected. Lymph node biopsy is the mode of diagnosis with demonstration of paracortical areas of apoptotic necrosis with abundant karyorrhectic debris and a proliferation of histiocytes, plasmacytoid dendritic cells, and CD8+ T cells in the absence of neutrophils. In most cases, the disease is self-limiting but it can be recurrent or evolve to SLE. Treatment varies from symptomatic to more systemic with cortisone and intravenous immunoglobulin.

Cervical lymphadenitis from Mycobacterium avium complex.

We present an instructive case of cervical lymphadenitis in a young man without a history of HIV infection. The patient developed spontaneous left-sided neck swelling that progressed over 4 months. CT imaging demonstrated a necrotic left-sided neck mass within the cervical lymph node chain. He was initially prescribed azithromycin and rifampin for presumed cat scratch disease with improvement but incomplete resolution of symptoms. Blood cultures ordered 2 months later grew Mycobacterium avium complex (MAC) and the patient had an excellent clinical response to MAC therapy. Here, we review the case, including presentation and management, and describe the implications for the immune status of the host and long-term considerations for treatment.

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.

BACKGROUND: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries. METHODS: Data of patients with PFAPA, SURF and uSAID were collected from 3 registries including detailed epidemiological, demographic and clinical data, results of the genetic testing and additional laboratory investigations with retrospective application of the modified Marshall and PRINTO/Eurofever classification criteria on the cohort of PFAPA patients and preliminary SURF criteria on uSAID/SURF patients. RESULTS: Clinical presentation of PFAPA is variable and some patients did not fit the conventional PFAPA criteria and exhibit different symptoms. Some patients did not meet the criteria for either PFAPA or SURF, highlighting the heterogeneity within these groups. The study also explored potential overlaps between PFAPA and SURF/uAID, revealing that some patients exhibited symptoms characteristic of both conditions, emphasizing the need for more precise classification criteria. CONCLUSIONS: Patients with recurrent fevers without molecular diagnoses represent a clinically heterogeneous group. Improved classification criteria are needed for both PFAPA and SURF/uAID to accurately identify and manage these patients, ultimately improving clinical outcomes.

A classic case of scrofula-cervical tuberculous lymphadenitis.

Tuberculosis (TB) is considered a common infection in developing countries and is caused by various strains of mycobacteria, usually Mycobacterium TB. TB remains to be one of the most important health threats. TB can have varied clinical presentations; Pulmonary TB affects the lungs and extrapulmonary TB (EPTB) can affect any part of the body. Cervical tuberculous lymphadenitis (CTL), cervical lymphadenitis, which is also referred to as scrofula is a case of EPTB that most frequently involves the cervical lymph nodes. In our report, a case of a young patient with CTL has been reported. This case's physical examination, evolution, diagnosis, and treatment have been discussed. Our case exemplifies the potential manifestations of an extrapulmonary tubercular lesion of the posterior pharyngeal wall, demonstrating that mycobacteria can infect practically any human organ. A high index of suspicion is critically required for the diagnosis of tuberculous lymphadenitis as mimics a number of pathological conditions.

Linfadenitis por Mycobacterium en pacientes inmunocompetentes y viviendo con VIH/sida / Lymphodenitis by Mycobacterium in immunocompetent and living with HIV/AIDS patients

Introducción: La coinfección del virus de inmunodeficiencia humana (VIH) y la tuberculosis ha alterado su presentación histológica, esto es particularmente frecuente en las linfadenitis. Objetivos: Realizar la caracterización etiológica de linfadenopatías producidas por el género Mycobacterium, destacar la importancia del diagnóstico precoz de esta enfermedad para evitar diseminación de la infección, tanto en pacientes inmunocompetentes como inmunodeficientes, específicamente con VIH/sida. Método: Se realizó estudio descriptivo-prospectivo entre enero de 2017 y enero de 2019. Durante este período se recibieron 5640 muestras, de estas 81 obtenidas a partir de tejido ganglionar; la toma de muestra mayoritariamente fue quirúrgica 74 (91,35 por ciento) y 7 (8,64 por ciento) por biopsia aspirativa (BAAF). Del total de muestras, 60 (74,07 por ciento) procedían de pacientes con VIH/sida, las muestras se descontaminaron por el método de ácido sulfúrico al 4 por ciento, se cultivaron en medio sólido Löwenstein-Jensen e incubaron a 37°C. Se realizaron lecturas semanalmente. Para identificar Mycobacterium tuberculosis se realizó la prueba rápida comercial inmunocromatográfica SD TB AgMPT64. Resultados: De 81 muestras analizadas se obtuvieron 22 (27,16 por ciento) aislamientos, 16 (72,72 por ciento) de Mycobacterium tuberculosis, y 6 (27,27 por ciento) de especies no tuberculosas. De estas, 18 (81,81 por ciento) procedían de pacientes con VIH/sida. Conclusión: Por todo lo antes expuesto es importante la vigilancia diagnóstica en este tipo de infección extrapulmonar, tanto para M. tuberculosis como para otras especies no tuberculosas y poder comenzar tempranamente el tratamiento específico evitando la diseminación de la infección, pues esta puede tener consecuencias fatales, sobre todo en pacientes con algún tipo de inmunosupresión, como aquellos con VHI/sida. Si un paciente mantiene fiebre prolongada, con linfadenopatías, sin síntomas respiratorios y no responde a los tratamientos con antibióticos, es necesario pensar en este tipo de infección(AU)

Introduction: The coinfection of human immunodeficiency virus (HIV) and tuberculosis has altered its histological presentation; this is particularly frequent in lymphadenitis. Objective: To carry out the etiological characterization of lymphadenopathies produced by the genus Mycobacterium, highlighting the importance of early diagnosis of this disease to avoid dissemination of the infection, both in immunocompetent and immunodeficient patients, specifically HIV / AIDS. Methods: A descriptive-prospective study was carried out between January 2017 - January 2019. During this period, 5640 samples were received, of these 81 obtained from lymph node tissue, the sample collection was mostly surgical 74 (91.35 percent) and 7 (8.64 percent) by aspiration biopsy (BAAF). Of the total samples, 60 (74.07 percent) were from HIV / AIDS patients, the samples were decontaminated by the 4 percent sulfuric acid method and cultured in solid Löwenstein-Jensen medium and incubated at 370C, the readings were made weekly. For the identification of Mycobacterium tuberculosis, the commercial SD TB AgMPT64 immunochromatographic rapid test was performed. Results: Of 81 samples analyzed, 22 (27.16 percent) isolates were obtained, 16 (72.72 percent) of Mycobacterium tuberculosis (MTB), and 6 (27.27 percent) of non-tuberculous species, of these 18 (81.81%) were from HIV / AIDS patients. Conclusion: For all the above, diagnostic surveillance is important in this type of extrapulmonary infection, both for M tuberculosis and for other non-tuberculous species and to be able to start specific treatment early, avoiding the spread of the infection, since it can have fatal consequences on all in patients with some type of immunosuppression, such as HIV/AIDS. If a patient maintains a prolonged fever, with lymphadenopathy, without respiratory symptoms and does not respond to antibiotic treatment, it is necessary to consider this type of infection(AU)

Sarcoidose como diagnóstico diferencial de mieloma múltiplo / Sarcoidosis as a differential diagnosis of multiple myeloma

A sarcoidose caracteriza-se como doença granulomatosa que acomete diferentes órgãos humanos, especialmente os pulmões, sendo sua patogênese pouco conhecida. No caso em questão, a paciente iniciou com sintomas inespecíficos, como fraqueza, perda ponderal e tosse seca esporádica, sendo internada para extensão da propedêutica. Sugeriu-se como hipótese diagnóstica inicial possível quadro de mieloma múltiplo, tendo em vista a anemia, a disfunção renal, a hipercalcemia e, sobretudo, as lesões osteolíticas apresentadas pela paciente. Todavia, o diagnóstico de sarcoidose foi selado a partir das biópsias de medula óssea e de linfonodo inguinal, que evidenciaram mielite e linfadenite granulomatosas, respectivamente. A terapêutica instituída baseou-se na administração de corticosteroides e em medidas de redução da calcemia. A paciente recebeu alta, com melhora do quadro clínico, para acompanhamento ambulatorial da doença. Conclui-se que a sarcoidose não possui tratamento curativo, mas a terapêutica imunossupressora é eficaz no controle da progressão da enfermidade, fazendo com que o paciente tenha um prognóstico favorável.

Sarcoidosis is characterized as a granulomatous disease that affects different human organs, especially the lungs, and its pathogenesis is little known. In this case, the patient started with nonspecific symptoms, such as weakness, weight loss, and sporadic dry cough, being hospitalized for extension of the propaedeutics. The initial diagnostic hypothesis suggested was a possible case of multiple myeloma, based on the anemia, renal dysfunction, hypercalcemia and, above all, the osteolytic lesions presented by the patient. However, the diagnosis of sarcoidosis was made after bone marrow and inguinal lymph node biopsies that showed granulomatous myelitis and lymphadenitis, respectively. The therapy instituted was based on the administration of corticosteroids and on measures to reduce the level of calcium. The patient was discharged, with clinical improvement, for outpatient follow-up of the disease. It is concluded that sarcoidosis has no curative treatment, but immunosuppressive therapy is effective in controlling the progression of the disease, giving the patient a favorable prognosis.

Tuberculosis ganglionar / Ganglionar tuberculosis

Introducción: La tuberculosis es una enfermedad que aún se diagnostica en Cuba. Aunque la forma pulmonar predomina, se presentan en ocasiones diversas formas localizadas a otros órganos y tejidos, dentro de ellas la forma ganglionar. Caso clínico: Se presenta una joven de 21 años con fiebre de 15 días de evolución y aumento de volumen no doloroso de los ganglios del cuello y preauricular izquierdo. Luego de tratamiento antibiótico la fiebre desaparece pero las adenopatías persisten. Se hace una primera exéresis ganglionar la cual arroja una adenitis crónica agudizada con abscedación. Se realiza Mantoux el cual arroja un resultado de 32 mm. El Rx de tórax y la tomografía axial computadorizada tóraco-abdominal no arrojaron ninguna alteración. Se hace una nueva exéresis ganglionar cuyo estudio anatomopatológico informa la presencia de granulomas caseificados. El estudio microbiológico del tejido arrojó Mycobacterium tuberculosos, codificación 8. Comentarios: La tuberculosis ganglionar es la primera forma de tuberculosis extrapulmonar en aquellos países con baja incidencia de esta enfermedad. Es más frecuente en mujeres y en la localización cervical. La cutirreacción de Mantoux hiperérgica es orientadora en el diagnóstico, pero se requiere del estudio histológico de un ganglio con la presencia de granulomas caseificados y la demostración del bacilo en este tejido. Se presenta este caso para recordar que esta entidad debe ser tenida en cuenta en el estudio de todo síndrome adénico febril y que es necesario que en el estudio histológico de toda exéresis ganglionar deben realizarse las técnicas necesarias para llegar a este diagnóstico(AU)

Introduction: Tuberculosis continues to be a disease diagnosed in Cuba. Although the pulmonary form continues to predominate, several localized forms are sometimes presented to other organs and tissues, including the ganglionic form. Clinical case: A 21-year-old girl presented with a fever of 15 days of evolution and a non-painful volume increase of the neck and left preauricular lymph nodes. After antibiotic treatment the fever disappears but the adenopathies persist. A first lymph node excision is performed, which results in acute chronic adenitis with abscess. Mantoux is performed with a result of 32 mm. The chest X-ray and the thoraco-abdominal CT scan did not show any alteration. A new lymph node excision is performed, whose anatomopathological study reports the presence of caseified granulomas. The microbiological study of the tissue resulted tuberculous Mycobacterium, coding 8. Comments: Lymph node tuberculosis is the first form of extrapulmonary tuberculosis in those countries with low incidence of this disease. It is more frequent in women and in the cervical location. The hyperergic Mantoux is a guide in the diagnosis, but the histological study of a ganglion with the presence of caseified granulomas and the demonstration of the bacillus in this tissue is required. This case is presented to remember that this entity must be taken into account in the study of any febrile adenic syndrome and that it is necessary that in the histological study of any lymph node excision the necessary techniques must be performed to reach this diagnosis(AU)

Fiebre periódica con estomatitis aftosa, faringitis, y adenitis / Periodical fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome)

RESUMEN Se presenta un caso de fiebre periódica con estomatitis aftosa, faringitis, y adenitis síndrome de fiebre periódica con estomatitis aftosa, faringitis, y adenitis, entidad aparentemente infrecuente de la cual no se recoge reporte en el país. La etiología no es todavía bien comprendida, se observa con mayor frecuencia entre los 2 y 5 años, aunque se han descrito casos en adultos. Se caracteriza por fiebre periódica con estomatitis aftosa, faringitis, y adenitis de carácter periódica con una dramática respuesta al uso de esteroide en la mayoría de los casos. El caso que se presenta es un paciente de 5 años de edad, sexo masculino, con cuadros de fiebre periódica asociado a amigdalitis con exudado, adenitis y lesiones aftosas, con respuesta en la fase aguda a la prednisona y con respuesta al tratamiento de mantenimiento con cimetidina. En la actualidad lleva 11 meses en remisión (AU).

ABSTRACT We present a case of periodical fever with aphthous stomatitis, pharyngitis and adenitis (PFAPA syndrome), entity that is apparently infrequent and its report in the country was not found. Its etiology is not still clearly understood. It appears with higher frequency in the ages between 2 and 5 years, though it has been described in adult population. It is characterized by periodical fever with aphthous stomatitis, pharyngitis and adenitis of periodical character, with a dramatic answer to the use of steroids in most of the cases. The case that is presented is the one of a male patient aged 5 years with reiterative periodical fever associated to tonsillitis with exudate, adenitis and aphthous lesions, with answer, in the acute phase, to prednisone, and to cimetidine at the maintenance therapy. Nowadays, he is already 11 months in remission (AU).

Síndrome celulitis-adenitis, una forma infrecuente de presentación de la sepsis neonatal tardía: A propósito de dos casos / Adenitis-cellulitis syndrome, an infrequent form of presentation of the late-onset neonatal septicemia: Report of two cases

La sepsis es la principal causa de mortalidad neonatal. La forma precoz, habitualmente, está relacionada con la colonización recto-vaginal u otros factores de riesgo materno. En la forma tardía, es difícil establecer su origen; por lo general, es nosocomial o de la comunidad. El Streptococcus agalactiae (Streptococcus beta-hemolítico del grupo B) es el germen implicado con más frecuencia en la sepsis neonatal en países desarrollados. La forma tardía, generalmente, se presenta con septicemia y meningitis, y, en ocasiones, pueden detectarse infecciones osteoarticulares o de piel y tejidos blandos. El síndrome celulitis-adenitis en la región cervical, forma poco frecuente de presentación, es causado por Staphylococcus aureus y, ocasionalmente, por Streptococcus agalactiae. Se reportan 2 casos de sepsis neonatal tardía con clínica de celulitis-adenitis cervical causados por Streptococcus beta-hemolítico del grupo B, con una evolución satisfactoria con terapia antibiótica de amplio espectro.

Septicemia is the main cause of neonatal mortality. The early-onset neonatal sepsis is usually related to maternal factor risks including recto-vaginal colonization. In the late-onset neonatal septicemia it is more difficult to establish the etiology because the majority of the cases are nosocomial or community related. The Streptococcus agalactiae (beta-hemolytic Streptococcus) is the most frequent germ associated with neonatal sepsis in developed countries. The late-onset form usually occurs with septic symptoms and meningitis and, in a few cases, with osteoarticular, skin and soft tissue infection. Adenitis-cellulitis syndrome is rarely seen, and its main cause is Staphylococcus aureus, followed by Streptococcus agalactiae. We report two cases of group B Streptococcus late-onset neonatal septicemia, both of them with adenitis-cellulitis syndrome. Patients recovered uneventfully after an adequate antibiotic therapy.

Histoplamosis diseminada en una paciente con hipotiroidismo / Disseminated histoplasmosis in a patient with hypothyroidism

La histoplasmosis es una infección causada por el hongo Histoplasma capsulatum, la incidencia de la enfermedad oscila entre 0.1 a 0.29 casos por cada 100 000 habitantes y de estos 1 de cada 2 000 casos se presenta de forma diseminada, manifestándose en personas con inmunosupresión y de forma infrecuente en personas inmunocompetentes. Objetivo: mostrar la relación existente entre hipotiroidismo e histoplasmosis diseminada. Presentación del caso clínico: paciente de 33 años, procedente de Talanga, Francisco Morazán, Honduras, tratada por hipotiroidismo de 3 años de evolución con levotiroxina 100ug cada día, con tumoración en axila izquierda de hace 2 años, la cual 3 meses previos al ingreso fue tratada por adenitis aguda de forma ambulatoria sin mostrar mejoría. Al examen físico se encontró adenopatía de 2 sospecha de histoplasmosis confirmándose con tinción de Grocott; el examen para virus de inmunodeficiencia humana fue negativo y el perfil inmunológico resultó normal. Conclusión : En este caso la histoplasmosis diseminada se acompañó de hipotiroidismo, no se ha demostrado que conlleve a inmunosupresión, y aún faltan pruebas que establezcan el vínculo de hipotiroidismo como factor predisponente a esta enfermedad...(AU)

Diretrizes de conduta e tratamento de síndromes febris periódicas: síndrome de febre periódica, estomatite aftosa, faringite e adenite / Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos.

Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.

Extensive cervical lymphadenitis mimicking bacterial adenitis as the first presentation of Kawasaki disease / Extensa linfadenite cervical mimetizando adenite bacteriana como primeira manifestação da doença de Kawasaki

Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient’s symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.

Adenite cervical >1,5cm é o critério menos frequentemente observado em pacientes com doença de Kawasaki e manifesta-se habitualmente em associação com os demais sintomas da fase aguda. Entretanto, linfadenite febril isolada com intensos sinais flogísticos e flegmão é raramente observada como primeira manifestação da doença de Kawasaki. Assim, relatou-se aqui um caso de uma criança que apresentou linfadenite cervical com celulite adjacente e flegmão mimetizando adenite bacteriana como primeira manifestação da doença de Kawasaki. Paciente previamente hígido, 7 anos, masculino, iniciou quadro de febre, adenite cervical com celulite adjacente e cefaleia intensa, sendo prescrita cefadroxila devido ao diagnóstico clínico de linfadenite bacteriana. Por se manter febril e com piora dos sinais flogísticos, após 1 dia foi internado para receber antibioticoterapia endovenosa (oxacilina e ceftriaxona). Tomografia computadorizada da região cervical mostrou processo infeccioso/inflamatório primário. No quarto dia, apresentou lábios ressecados e descamativos, sendo a oxacilina substituída por clindamicina devido à persistência da febre e sinais flogísticos. No nono dia, iniciou hiperemia ocular não exsudativa. No décimo dia de febre, apresentou exantema em tronco, membros, mãos e pés. Recebeu gamaglobulina endovenosa (2g/kg/dose), evoluiu com resolução dos sintomas e, após 2 dias, recebeu alta hospitalar. No 14odia, apresentou descamação lamelar dos dedos das mãos. Portanto, doença de Kawasaki deve ser considerada no diagnóstico diferencial das linfadenites cervicais febris na infância não responsivas à antibioticoterapia empírica, mesmo que esteja presentes celulite adjacente e flegmão.