Estenosis laringotraqueal recurrente asociada a Klebsiella pneumoniae subsp. rhinoscleromatis / Recurrent laryngotracheal stenosis associated with Klebsiella pneumoniae subsp. rhinoscleromatis

Introducción: Klebsiella rhinoscleromatis (KR) es una enterobacteria asociada con formación de granulomatosis crónica. Cuando este microorganismo afecta el tracto respiratorio se denomina escleroma, afectando principalmente la cavidad nasal; puede comprometer nasofaringe, laringe, tráquea y bronquios. Caso clínico: paciente femenina con antecedente de laringotraqueítis crónica con diagnóstico de estenosis traqueal y aislamiento en cultivos de Klebsiella pneumoniae ssp rhinoscleromatis multisensible, sin compromiso nasosinusal o extralaríngeo. Discusión: el escleroma puede afectar todo el tracto respiratorio y se deben tener presentes factores de riesgo asociados, como condiciones de hacinamiento, inmunosupresión y sexo femenino. El pilar del tratamiento es médico, basado en antibióticos; adicionalmente, se reserva manejo quirúrgico en la etapa esclerótica, donde hay ausencia del fenómeno inflamatorio. Conclusión: el escleroma es una patología rara con una evolución crónica y compromiso principalmente en cavidad nasal, que requiere alta sospecha diagnóstica para realizar manejo oportuno.

Introduction: Klebsiella rhinoscleromatis (KR) is an enterobacterium associated with the formation of chronic granulomatosis. When this microorganism affects the respiratory tract, it is called scleroma, the nasal cavity is the main one affected; additionally, it can involve nasopharynx, larynx, trachea, and bronchi. Clinical case: female patient with a history of chronic laryngotracheitis, with diagnosis of tra-cheal stenosis and isolation in cultures of multisensitive Klebsiella pneumoniae ssp rhinoscleromatis, without nasosinusal or extralaryngeal involvement. Discussion: scleroma can affect the entire respiratory tract, so associated risk factors should be taken into account, mainly overcrowding, immunosuppression, and female sex, in whom it is more common. The mainstay of treatment is medical, based on antibio-tics; additionally, surgical management is reserved for sclerotic stage, when there is no inflammatory phenomenon. Conclusion: scleroma is a rare pathology, with a chronic evolution, with involvement mainly in the nasal cavity, which requires a high diagnostic suspicion for its timely management.

Rhinoscleroma Revisited

ABSTRACT Rhinoscleroma (more appropriately 'scleroma') is a chronic, disfiguring inflammatory lesion that is rarely encountered in the present-day otolaryngology practice. The diagnosis often becomes difficult especially when it recurs and presents late with complications. This report illustrates the clinical and histologic features of rhinoscleroma in a defaulter patient who represents advanced-stage disease with orbital and intracranial extensions. Special emphasis has been provided on the computed tomography impressions such that the evolution of scleroma with time can be studied.

Histological correlation of nasal mass: a five year retrospective and prospective study.

Background: The presence of mass in the nose may seem to be a simple problem; however it raises several issues about the differential diagnosis. The aim of this study is to evaluate the histological profile of nasal masses in the environment. Methods: This is a retrospective and prospective study of nasal masses during a period of five years of patients presenting to the clinician in our hospital. Results: A total of 90 patients were analysed age ranging from 3 to 80 years. Majority of patients were in the second decade. Sex wise distribution showed a male to female ratio of 2.2:1. The main presenting symptom was nasal obstruction seen in 53.30% patients, while proptosis was seen only in malignant lesion. Among a total of 90 lesions in the nasal cavity 81.1% were benign, 15.5 % were malignant and 3.3% were inadequate sample The commonest non neoplastic lesion was nasal polyp which comprised about 83.33% followed by rhinoscleroma, while the commonest benign neoplastic lesion was papilloma and comprised about 42.10% and squamous cell carcinoma was the most common malignant neoplastic lesion in the nasal cavity and comprised about 42.85%. Most patients with benign lesion were in the second decade. Malignant lesions occurred mainly after fifth decade, while inadequacy of sample was seen in the second decade. Conclusion: Males are more commonly affected then females. Nasal obstruction is the commonest symptom Simple inflammatory nasal polyp is still the commonest histological pattern seen while papilloma being the most common benign lesion and squamous cell carcinoma the most common malignant lesion.

Rinoscleroma / Rhinoscleroma

INTRODUÇÃO: Rinoscleroma é uma doença infecciosa crônica do tipo granulomatosa causada pela bactéria Klebsiella rhinoscleromatis. Acomete a mucosa do trato respiratório, mais frequentemente o nariz. É considerada endêmica em determinadas regiões com África e América Central, porém é rara no Brasil. O acometimento nasal ocorre em 3 fases: catarral, granulomatosa e cicatricial. Em todo o seu curso a doença apresenta sintomatologia inespecífica, daí a dificuldade em ser diagnosticada. Seu diagnóstico é estabelecido através de cultura ou pelo encontro de células de Mikulicz ou corpúsculo de Russel no estudo anatomopatológico. O tratamento consiste em antibioticoterapia por longo período, associada ou não a cirurgia. OBJETIVO: Este relato tem por objetivo ilustrar um caso de rinoscleroma em uma paciente jovem com queixa de obstrução nasal bilateral de longa data e cefaleia. O intuito é alertar os otorrinolaringologistas para o diagnóstico desta doença rara, que se apresenta com sintomas inespecíficos e semelhantes a inúmeras patologias que acometem a região nasal.

INTRODUCTION: Rhinoscleroma is a chronic granulomatous infectious disease, caused by the bacterium Klebsiella rhinoscleromatis. Affects the respiratory tract mucosa, more frequent in the nose. Is considered endemic in certain regions like Africa and Central America, but is rare in Brazil. The nasal involvement occurs in 3 phases: catarrhal, granulomatous and sclerotic stage. In all its course the disease presents nonspecific symptoms, and because of that, the difficulty of being diagnosed. Its diagnosis is established by culture or by the meeting of Mikulicz cell or Russel corpuscles in the anatomopathological study. The treatment consists in a long-term antibiotic, associated or not to a surgery. OBJECTIVE: This report aims ilustrate a case of rhinoscleroma in a female young patient complaining of bilateral nasal obstruction, long standing and headache. The intent is alert the otorhinolaringologists in diagnosing this rare disease, which presents itself with nonspecific symptoms as like numerous pathologies that affect the nasal region.

Cytohistological features of rhinoscleroma.

Rhinoscleroma, also known as Mikulicz disease, is a chronic progressive disease caused by Klebsiella rhinoscleromatis. The disease is uncommon in Delhi. There are no cytological references of this disease. We describe the cytological features which should alert the pathologist to rule out this entity, which needs to be treated early. Immunohistochemistry on histopathological material is a useful adjunct to the diagnosis.

Rinitis atrófica como causa del síndrome de nariz vacía: reporte de caso / Atrophic rhinitis as cause of the empty nose syndrome: case report

La rinitis atrófica es una de las causas del Síndrome de nariz vacía, la cual está relacionada con colonización por gérmenes, inflamación crónica y posterior atrofia progresiva de la mucosa nasal. Típicamente produce síntomas obstructivos nasales, rinorrea y costras fétidas. Se presenta el caso en imágenes endoscópicas, tomográficas y de cultivo en una paciente femenina en la cual por sus comorbilidades el manejo fue exclusivamente médico.

Atrophic rhinitis is one of the causes of the empty nose syndrome that is related to colonization by germs, chronic inflammation and subsequent progressive atrophy of the nasal mucosa. Typically produces nasal obstructive symptoms, runny nose and fetid crusts. Endoscopy, tomography and culture images of a female patient is presented in which by her co-morbidities associated the treatment was medical exclusively.

Rhinoscleroma causing severe bilateral nasal obstruction

Rhinoscleroma is a chronic, infectious and granulomatous disease of the respiratory tract. There is often a delay in diagnosis due to unfamiliarity with the disease and also because culture is not always positive. We report a case in a 26-year-old woman with granular mass obstructing bilateral nasal cavities and causing breathing difficulty. Histopathological examination showed characteristic Mikulicz histiocytes containing numerous Gram-negative intracellular rod-shaped bacilli consistent with the diagnosis of rhinoscleroma. The patient was treated with gemifloxacin and tetracycline and remains asymptomatic over a year follow-up period. It is important to consider rhinoscleroma in cases of chronic nasal obstruction. As culture is not always positive, histopathological examination may be crucial to the diagnosis.

Rinoscleroma e linfoma não-Hodgkin nasal / Rhinoscleroma and nasal non-Hodgkin lymphoma

Introdução: O rinoscleroma é uma doença nasal granulomatosa rara, causada pela bactéria klebsiella rhinoscleromatis. O nariz representa a região primária de ocorrência. O linfoma não-Hodgkin nasal é uma rara neoplasia maligna, podendo ser do tipo T ou B. O rinoscleroma e o linfoma nasal não-Hodgkin apresentam como principal sintoma a obstrução nasal, e fazem parte do diagnóstico diferencial das granulomatoses nasais. Objetivo: Relatar a associação de rinoscleroma e linfoma não-Hodgkin nasal em um mesmo paciente, destacando a importância do diagnóstico diferencial das granulomatoses nasais. Relato do Caso: Paciente do sexo feminino, 49 anos, atendida com obstrução nasal progressiva há um mês. A rinoscopia evidenciava lesão aspecto papilomatoso em meato médio esquerdo. A imunohistoquímica confirmou rinoscleroma. A paciente foi corretamente tratada com remissão total da lesão. Dez meses após retornou com mesmos sintomas, mas histologia confirmou linfoma não-Hodgkin. Comentários Finais: Ambas as patologias podem causar sintomas nasais graves e complicações. O diagnóstico precoce possibilita a instituição do tratamento adequado e favorece o prognóstico. O estudo imunohistoquímico foi fundamental para a diferenciação diagnóstica.

Introduction: Rhinoscleroma, a rare nasal granulomatous disease, is caused by Klebsiella rhinoscleromatis. The nose is the primary occurrence region. Nasal non-Hodgkin lymphoma is a rare cancer, and could be of T or B type. The rhinoscleroma and the nasal non-Hodgkin lymphoma present with nasal obstruction as the main symptom, and are part of the nasal granulomatosis differential diagnosis. Objective: To report the association of rhinoscleroma and non-Hodgkin lymphoma in the same patient, by remarking the importance of the nasal granulomatosis' differential diagnosis. Case Report: A forty-nine year old female patient that appeared with a one-month progressive nasal obstruction. Rhinoscopy showed papillomatous feature lesion in left middle meatus. The immunohistochemical analysis confirmed rhinoscleroma. The patient was duly treated with total remission of the lesion. Ten months after, she returned with the same symptoms, but the histologic study confirmed non-Hodgkin lymphoma. Final Comments: Both pathologies may cause more severe nasal symptoms and complications. The early diagnostic enables the choice for the right treatment and contributes for the prognosis. The immunohistochemical study was essential for the diagnostic differentiation.

Rhinoscleroma Mimicking Malignancy.

Rhinoscleroma is an uncommon chronic granulomatous disease of the upper airways affecting nasal cavity, nasopharynx, larynx, trachea, and bronchi. The oral cavity, para nasal sinuses, and soft tissues of the lips and nose can be affected. In rare cases, rhinoscleroma spreads to the orbit. We present a case that was being evaluated for bilateral neck nodal mass, was confused with malignancy and subsequently, on thorough clinicopathological evaluation, was diagnosed as rhinoscleroma. This case underlines the importance of thorough clinical evaluation and diagnostic workup before instituting any sort of treatment in oncology.

Nasal Rhinoscleroma in a Nonendemic Area: A Case Report

Rhinoscleroma is a chronic, granulomatous infectious lesion most frequently affecting the respiratory tract mucosa. This disease is endemic to Africa, Central and South America, South Central and Eastern Europe, the Middle East, and China. We report an extremely rare disease of nasal rhinoscleroma in Korea. The patient was a 63-yr-old man, who suffered from chronic nasal obstruction and septal deviation. Characteristic histology from a nasal biopsy was proven and special stains for bacteria were employed: periodic acid-Schiff, Warthin-Starry silver, Giemsa, and Gram stains. Isolation of Klebsiella rhinoscleromatis from a culture of nasal discharge was failed, but ultrastructural examination of numerous phagocyzed bacilli in the Mikulicz cells confirmed the diagnosis. The histiocytic nature of the Mikulicz cells was confirmed, using CD 68 and alpha-1 antitrypsin, and by the ultrastructural features of Mikulicz cells. Here, we emphasize the recognition of this rare entity in nonendemic regions, frequently leading to delayed diagnosis.

Rhinoscleroma: A case report

Rhinoscleroma, or scleroma, is a chronic, slowly progressing granulomatous disease involving the upper respiratory tract, especially the nasal vestibules, choanae, pharynx, and larynx. Almost certainly the causative agent is Klebsiella rhinoscleromatis. The disease occurs frequently in Eastern Europe, the Middle East, and parts of Latin America, but it is hard to find such case in Korea. We prisent a case of rhinoscleroma involving the nasal vestibule in a 18-year-old male who was admitted due to nasal obstruction for 5 years and epistaxis for 2 months. The resected specimen was an irregular polypoid mass with relatively firm consistency and measured 3 cm in the largest diameter. Microscopically, the lesion was characterized by extensive fibrosis and inflammatory cell infiltration. The infiltrates consisted of predominantly lumphocytes, plasma cells, foamy or granular histiocytes which were singly scattered or grouped in clusters. In the cytoplasm of the histiocytes, round slightly basophilic bodies were noted. Warthin-Starry satin showed short positive rods within the cytoplasm of the cell (Mikulicz cell). Electron microscopically, the cytoplasm of Mikulicz cells contained large, round or irregular shaped clear vacuoles in which numerous Klebsiella bacilli attached to the boundaries of the vaculoes were noted. With higher magnifications, the bacilli were seen as roung or rod-shaped organisms.