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.

Patología infecciosa del vestíbulo nasal / Infectious diseases of the nasal vestibule

El vestíbulo nasal corresponde a la primera porción de la fosa nasal, éste se encuentra delimitado lateralmente por los cartílagos alares y medialmente por el borde caudal del septum nasal y la columela. Las enfermedades infecciosas del vestíbulo nasal son patologías frecuentes en la práctica clínica; su diagnóstico se realiza en base a sospecha clínica y examen físico, requiriendo habitualmente solo manejo médico ambulatorio. Desde el punto de vista etiológico pueden ser virales, bacterianas y fúngicas. Las principales especies bacterianas involucradas corresponden a Staphylococcus coagulasa negativa, S. epidermidis, S. hominis y S. haemolyticus, difteroides spp y S. aureus. Su manejo es esencialmente médico con casos excepcionales requiriendo manejo quirúrgico. En la actualidad existe escasa información epidemiológica al respecto, lo que dificultad la clasificación de los dichos cuadros clínicos. Se realizó una revisión de la literatura sobre cuadros infecciosos que afectan el vestíbulo nasal para lograr sistematizar y clarificar las distintas patologías y sus tratamientos.

The nasal vestibule corresponds to the first portion of the nasal passage, limited laterally by the lateral crus and medially by the caudal edge of the nasal septum and columella. Infectious diseases of the nasal vestibule are frequent in clinical practice, diagnosis is made based on clinical suspicion and physical examination, usually requiring only ambulatory medical management. In terms of etiology, they can be viral, bacterial and fungal. The main bacterial species involved correspond: Coagulase-negative Staphylococcus, S. epidermidis, S. hominis and S. haemolyticus, difteroides spp and S. aureus. Management is essentially medical and only exceptionally requires surgery. Currently, there is a lack of epidemiological information in this regard, which makes it difficult to classify these clinical conditions. A review of the literature on infectious conditions that affect the nasal vestibule was performed, to systematize and clarify the different pathologies and their management.

Respiratory scleroma: A clinicopathologic study of 51 cases from Guatemala.

OBJECTIVES: To evaluate clinical and pathologically cases of respiratory scleroma diagnosed in a 30-year period in Guatemala. MATERIAL AND METHODS: Fifty-one cases of respiratory scleroma diagnosed from 1988 to 2018 in a single pathology service in Guatemala were confirmed using Warthin-Starry staining. Immunohistochemical reactions against CD68, LCA, CD20, CD3, and CD138 were performed to illustrate the inflammatory infiltrate. Scanning electron microscopy (SEM) was performed to illustrate bacteria morphology. RESULTS: All 51 cases affected patients from poor areas of Guatemala, particularly women (66.7%), with a mean age of 31 years (range 7-66 years). Nose was affected in most cases (96.1%). Other sites involved included pharynx, larynx, palate, maxillary sinuses, and upper lip. Depending on the stage, the disease manifested as ulcerations, nasal deformities, or laryngeal stenosis. Nasal obstruction, epistaxis, dysphonia, fetid discharge, and pain were the main symptoms. Mikulicz cells (CD68+) in a plasma cell-rich inflammatory background (CD138+, CD20+, CD3+/-) were the typical microscopic presentation. In SEM, each macrophagic vacuole contained few to dozens of Klebsiella rhinoscleromatis diplobacilli. Treatment consisted of long-term trimethoprim and sulfamethoxazole, with adequate control of disease. CONCLUSION: Respiratory scleroma is a rare infectious disease affecting the upper respiratory tract, in poor regions of the world, including Guatemala.

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.

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.

[Laryngeal stenosis as late manifestation of rhinoscleroma. Case report]. / Estenosis subglótica como manifestación tardía de un rinoescleroma. Presentación de un caso.

Rhinoscleroma is a chronic, progressive, granulomatous disease resulting from infection with Klebsiella rhinoscleromatis. It is generally uncommon, but endemic in certain regions of the world. It typically affects the nasal cavity, but also other parts of the respiratory system, such as the laryngotracheal region, which may be life-threatening if compromised. We present a 46-year-old male patient with a history of rhinoscleroma diagnosed and treated 15 years ago, with subglottal stenosis as a late sequela of this disease.

Infección por Klebsiella rinoescleromatis: revisión de una entidadotorrinolaringológica de importancia creciente: [revisión] / Klebsiella rhinoscleromatis infection: review of an otolaryngologic entity of increasing relevance: [review]

La infección por Klebsiella rinoescleromatis es una condición infrecuente en nuestro país, pero endémica en ciertas regiones del mundo. Se presenta como una entidad crónica, progresiva y de aspecto granulomatoso. Causa típicamente rinoescleroma, pero puede comprometer otras partes del sistema respiratorio. Especial atención requiere el compromiso laríngeo y traqueal pues puede representar un riesgo para la vida del paciente. Su diagnóstico se basa en hallazgos como deformidad nasal u obstrucción de la vía aérea con disnea, disfonía y estridor respiratorio. Se apoya en técnicas de imagen como la tomografía computada y la resonancia magnética, pero el diagnóstico confirmatorio lo entrega la histopatología. Su tratamiento incluye el uso de antibióticos y cirugía, la que se puede realizar por diversas técnicas. En suma, es una enfermedad que requiere un alto grado de sospecha y se debe tener en consideración actualmente ante la inmigración a nuestro medio desde regiones endémicas cercanas.

Klebsiella Rhinoscleromatis infection is a rare condition in our country, but is endemic in certain regions of the world. It is presented as a chronic entity, progressive and granulomatous aspect.Typically cause rhinoscleroma, but may also compromise other parts of the respiratory tract. Special attention requires the laryngeal and tracheal compromise therefore may pose a risk to the patient’s life. Its diagnosis is based on the clinical findings like nasal deformity or obstruction of the airway with dyspnea, dysphonia and stridor. It relies on imaging techniques such as computed tomography and magnetic resonance imaging, but the diagnosis is confirmed by histopathology. Its treatment includes antibiotics and surgery, which can be achieved by various techniques. In short, is a disease that requires a high degree of suspicion and should be taken into consideration now before the immigration to our country from nearly endemic regions.

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.

Rinoescleroma traqueobronquial calcificado / Calcified tracheobronquial rhinoescleroma

Se presenta un caso de rinoescleroma de la vía aérea superior e inferior, recidivante, de 20 años de volución, persistente a pesar de múltiples tratamientos. Las calcificaciones del árbol traqueobronquial que presenta plantearon un diagnóstico diferencial con traqueobroncopatía osteocondroplástica. En el artículo se revisan los hallazgos anatomopatológicos y radiológicos relevantes de estas patologías.

Rhinoscleroma: case report.

Rhinoscleroma is a chronic, granulomatous infection that most frequently affects the respiratory mucosa, especially the nasal cavity and eventually extending through the lower respiratory tract. The disease is endemic in some countries of Central America (El Salvador and Guatemala), Indonesia, India, Poland, Hungary, Russia and some African countries as well. It is a rare disease in South America. We report a 51-year-old male resident of a psychiatric institution in São Paulo presenting with progressive nasal obstruction, frontal headache, yellowish nasal discharge and a mass extruding through the right nasal vestibule. The present case report describes a Rhino-Sinus scleroma where histopathology was vital in the diagnosis. The patient was treated by surgical excision of the nasal mass followed by a course of ciprofloxacin. He has remained asymptomatic up to the last visit six months following treatment and has shown no evidence of recurrence.

Rhinoscleroma: eight Peruvian cases.

Rhinoscleroma is a rare infection in developed countries; although, it is reported with some frequency in poorer regions such as Central Africa, Central and South America, Eastern and Central Europe, Middle East, India and Indonesia. Nowadays, rhinoscleroma may be erroneously diagnosed as mucocutaneos leishmaniasis, leprosy, paracoccidioidomycosis, rhinosporidiasis, late syphilis, neoplasic diseases or other upper airway diseases. From 1996 to 2003, we diagnosed rhinoscleroma in eight patients attended in the Dermatologic and Transmitted Diseases service of "Cayetano Heredia" National Hospital, in Lima, Peru. The patients presented airway structural alterations producing nasopharyngeal, oropharyngeal and, in one patient, laryngeal stenosis. Biopsy samples revealed large vacuolated macrophages (Mikulicz cells) in all patients. Ciprofloxacin 500 mg bid for four to 12 weeks was used in seven patients and oxytetracycline 500 mg qid for six weeks in one patient. After follow-up for six to 12 months the patients did not show active infection or relapse, however, all of them presented some degree of upper airway stenosis. These cases are reported because of the difficulty diagnosing the disease and the success of antibiotic treatment.

Rhinoscleroma: eight Peruvian cases / Rinoscleroma: oito casos peruanos

Rhinoscleroma is a rare infection in developed countries; although, it is reported with some frequency in poorer regions such as Central Africa, Central and South America, Eastern and Central Europe, Middle East, India and Indonesia. Nowadays, rhinoscleroma may be erroneously diagnosed as mucocutaneos leishmaniasis, leprosy, paracoccidioidomycosis, rhinosporidiasis, late syphilis, neoplasic diseases or other upper airway diseases. From 1996 to 2003, we diagnosed rhinoscleroma in eight patients attended in the Dermatologic and Transmitted Diseases service of "Cayetano Heredia" National Hospital, in Lima, Peru. The patients presented airway structural alterations producing nasopharyngeal, oropharyngeal and, in one patient, laryngeal stenosis. Biopsy samples revealed large vacuolated macrophages (Mikulicz cells) in all patients. Ciprofloxacin 500 mg bid for four to 12 weeks was used in seven patients and oxytetracycline 500 mg qid for six weeks in one patient. After follow-up for six to 12 months the patients did not show active infection or relapse, however, all of them presented some degree of upper airway stenosis. These cases are reported because of the difficulty diagnosing the disease and the success of antibiotic treatment.

O rinoscleroma é uma infecção rara nos países desenvolvidos, no entanto, tem sido relatado com alguma freqüência nas regiões pobres da Africa Central, América Central e do Sul, Europa Central e Oriental, Oriente Médio, índia e Indonésia. A doença pode ser erroneamente diagnosticada como leishmaniose mucocutânea, hanseníase, paracoccidioidomicose, rinosporidiose, sífilis tardia, neoplasias ou outras doenças que afetam a via respiratória superior. No período de 1996 a 2003, foram diagnosticados oito casos de rinoscleroma no serviço de Doenças Dermatológicas e Infecciosas do Hospital Nacional "Cayetano Heredia", em Lima, Peru. Os pacientes apresentaram alterações estruturais das vias respiratórias, caracterizadas por estenose da nasofaringe e orofaringe, e em um paciente, a nível da laringe. As biópsias mostraram macrófagos com grandes vacúolos (células de Mikulicz). A ciprofloxacina 500 mg de 12/12 horas por quatro a 12 semanas foi usada em sete pacientes e oxitetraciclina 500 mg de 6/6 horas por seis semanas em um paciente. Durante o acompanhamento por seis a 12 meses todos os pacientes apresentaram cura clínica, sem recaída, embora exibissem algum grau de estenose na via respiratória superior. O motivo do relato deve-se ao fato desta doença constituir um grande desafio diagnóstico e pelo sucesso alcançado com o tratamento antibiótico.

Rinoscleroma: relato de caso / Rhinoscleroma: case report

Rinoscleroma é uma doença crônica granulomatosa, comprometendo principalmente a mucosa do trato respiratório, especialmente a cavidade nasal podendo eventualmente se estender através das vias aéreas inferiores. Esta doença é endêmica em alguns países da América Central (El Salvador e Guatemala), Indonésia, índia, Polônia, Hungria, Rússia e alguns países da Africa, porém é rara na América do Sul. Trata-se de um paciente do sexo masculino de 51 anos de idade residente de uma instituição psiquiátrica em São Paulo (capital). Apresentou queixa de obstrução nasal progressiva, cefaléia frontal, rinorréia amarelada e massa exteriorizando-se pelo vestíbulo da fossa nasal direita. Este relato de caso descreve um rinoscleroma, onde o exame anatomopatológico teve papel decisivo no diagnóstico. O paciente foi, portanto, submetido a procedimento cirúrgico, seguido de tratamento clínico com ciprofloxacina. O paciente evoluiu bem e manteve assintomático sem evidência clínica de recidiva até sua última visita em nosso departamento seis meses após o tratamento inicial.

Rhinoscleroma is a chronic, granulomatous infection that most frequently affects the respiratory mucosa, especially the nasal cavity and eventually extending through the lower respiratory tract. The disease is endemic in some countries of Central America (El Salvador and Guatemala), Indonesia, India, Poland, Hungary, Russia and some African countries as well. It is a rare disease in South America. We report a 51-year-old male resident of a psychiatric institution in São Paulo presenting with progressive nasal obstruction, frontal headache, yellowish nasal discharge and a mass extruding through the right nasal vestibule. The present case report describes a Rhino-Sinus scleroma where histopathology was vital in the diagnosis. The patient was treated by surgical excision of the nasal mass followed by a course of ciprofloxacin. He has remained asymptomatic up to the last visit six months following treatment and has shown no evidence of recurrence.

Rinoescleroma, una enfermedad rara en Chile: reporte de un caso clínico / Rhinoscleroma, a rare illness in Chile: report of a clinical case

Se presenta el primer caso publicado de rinoescleroma en un paciente chileno, con historia de tres años de evolución de obstrucción nasal izquierda que luego se hace bilateral. Es estudiado en el Policlínico de Otorrinolaringología del Hospital Barros Luco Trudeau, pesquisándose una masa en fosas nasales de aspecto polipoideo atípico. Mediante estudio histopatológico e infectológico se confirma el diagnóstico de rinoescleroma, con cultivo positivo para Klebsiella rhinoscleromatis. El paciente es tratado con moxifloxacino durante seis semanas previo a la cirugía endoscópica, efectuándose resección de la patología en ambas fosas nasales. Se trata con antibiótico durante seis semanas más, quedando, a los tres meses postcirugía, con leve obstrucción nasal a izquierda secundaria a una estenosis fibrosa de coana izquierda, residual a su patología.

Casos de rinoescleroma y sífilis nasal tratados con radioterapia (1935) / Rhinoescleroma and nasal syphilis cases treated with radiotherapy (1935)

Se trata de un grupo de pacientes, todas ellas mujeres jóvenes provenientes del área rural con rinoscleroma. La revisión plantea un problema que setenta años después sigue perfectamente vigente, haciendo referencia a la similitud de presentación clínca, complicaciones e imágenes tanto radiológicas como histológicas que puede haber entre tumores, enfermedades inflamatorias e infecciosas, donde el diagnóstico final dependerá de la perfección de la aplicación de múltiples disciplinas. El rinoscleroma refleja claramente el paso por distintas clasificaciones y orígnes etiólogicos, descrito por primera vez en la sociedad médica de Viena, en 1870, por Von Hebra y Kaposi, como una forma de sarcoma entre de origen glial y carcinoma, posteriormente la misma sociedad lo consideró una forma de sífilis. En 1840, Biercowsky y Kraskow habían demostrado la enfermedad en restos arqueológicos de Terracota y de la cultura maya, correspondientes a 300-600 años antes de Cristo. En 1872 y 1877, Mikuliez y Gerber describieron las primeras imágeneshistológicas; en 1882,Frisch aisló un germen que consideró el agente causal del rinoscleroma. Sin embargo, esto no puede demostrar hasta 1962, cien años después de la priemera descripción, cuando Steffen, Smith y Hoffman aislaron un germen que cumplía la totalidad de los postulados de Koch, llamado klebsiella rhinoscleromatis (1,5)reconocido, en su cápsulas tipo III y IV, por técnicas de fermetacio´n, inmunológicas, biología molecular, cultivos con subespecialización bioquímica e inmunoperoxidasa (2,3)

Rinoescleroma en edad escolar: a propósito de un caso / Rinoesclerosis in age school: a purpose of a case

El Rinoescleroma es una infección crónica granulomatosa progresiva causada por un bacilo Gram negativo Klebsiella rhinoescleromatis. Afecta usualmente la cavidad nasal y disemina rápidamente a estructuras vecinas es inespecifica: rinorrea purulenta, obstrucción nasal, epistaxis. El diagnóstico se basa en la demostración del microorganismo causal por biopsia o cultivo. En la biopsia del tejido afectado se aprecian células de MIKULICZ patognomónicas. El tratamiento consiste en la antibióticoterapia adecuada. Describimos un caso histológicamente de mostrado de Rinoescleroma, en un escolar masculino de 11 años de edad. Hacemos énfasis en la importancia de conocer esta patología debido a su poca frecuencia en nuestro medio, además de presentarse atípicamente en un escolar y de sexo masculino, ya que predomina entre la tercera y cuarta década de la vida, en el sexo femenino, y es el primer caso reportado en el Hospital Padre Justo de Rubio. Estado Táchira

Escleroma respiratoria en niños: experiencia en el Hospital de la Misericordia / Respiratory scleroma in child: experience in Hospital de la Misericordia

El escleroma respiratorio es una rara enfermedad granulomatosa en la población infantil causada por la Klebsiella rhinoscleromatis, de ubicación más frecuente en la nariz, aunque cualquier parte del tracto respiratorio superior o inferior puede estar involucrado. Diferentes factores pueden relacionarse con su ocurrencia, además la inmunidad celular parece estar alterada en los pacientes afectados. La enfermedad se caracteriza por presentar tres etapas con presentación clínica e histopatológica diferente para cada una de ellas. El diagnóstico se basa en la historia clínica y se confirma por anatomía patológica y de cultivo bacteriológico. Se realizó un estudio retrospectivo en el Hospital de La Misericordia de 1989 a 2001, ingresando tres pacientes con edades promedio de 12,6 años, con afectación nasal y laringotraqueal. El diagnóstico de escleroma fue confirmado por histopatología. Los pacientes recibieron manejo específico de su patología y antibióticos orales por seis meses con evolución satisfactoria. Debe tenerse un alto índice de sospecha de esta patología en pacientes provenientes de áreas endémicas