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.

Rhinoscleroma disease accompanied with laryngotracheal stenosis: a case report / 临床耳鼻咽喉头颈外科杂志

Rhinoscleroma disease accompanied with laryngotracheal stenosis as a main clinical manifestation is seldom. Here one case is reported. At the beginning, the patient had pharyngalgia and his swallowing was not smooth. After that, he had difficulty in breathing. Surgical therapy, antibiotics treatment and nutritional support were performed. The patient was cured.

Expression and significance of IgG4 in inflammatory disease of nasal cavity and paranasal sinuses / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD).</p><p><b>METHODS</b>The expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.</p><p><b>RESULTS</b>Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2).</p><p><b>CONCLUSION</b>Inflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.</p>

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.

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.

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.

Pathologic diagnosis of specific infections in upper aerodigestive tract / 中华病理学杂志

<p><b>OBJECTIVE</b>To find a fast and simple method for detection of specific pathogens in upper aerodigestive tract.</p><p><b>METHODS</b>Sixty-one cases of specific infections in upper aerodigestive tract encountered during a 10-year period in Beijing Tongren Hospital were retrospectively studied. Six histochemical stains, including PAS, Giemsa, Gram, methylene blue, modified Warthin-Starry and acid-fast stains were applied. The morphology of different pathogens was studied and the staining patterns were compared.</p><p><b>RESULTS</b>There were 23 cases of pharyngeal treponemal infection, 10 cases of short treponemal infection, 4 cases of mycobacterial infection, 4 cases of infection by rhinoscleroma bacilli, 1 case of sinonasal fungal infection, 1 case of combined infection of bacteria and Oidium albicans, 2 cases of tonsillar Actinomycetes and 16 cases of non-specific bacterial infections. Both pharyngeal treponemal infection and infection by rhinoscleroma bacilli could be detected by modified Warthin-Starry stain. As for sinonasal fungal infection, PAS, Giemsa and modified Warthin-Starry stains were useful in differentiating different types of fungi. Mycobacteria were best demonstrated by conventional acid-fast stain.</p><p><b>CONCLUSIONS</b>Special histochemical stains performed on histologic sections are useful for diagnosing specific infections in upper aerodigestive tract.</p>

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.

Clinicopathological study of non-neoplastic lesions of nasal cavity and paranasal sinuses.

An analysis of cases presenting as mass in nasal cavity (NC), paranasal sinuses (PNS), and nasopharynx (NP) was done over a period of 7 years in Jawaharlal Nehru Medical College, Aligarh. A provisional diagnosis was made after clinical assessment and radiological investigations, but final diagnosis was made after histopathological examination. The incidence of masses in NC, PNS, and NP was 34.3 cases per year, non-neoplastic lesions constituted 60% of these cases and their incidence was 20.7 cases per year. All the cases were carefully examined histopathologically and it was found that the region was affected by a variety of non-neoplastic lesions. Among 240 cases, 145 were non-neoplastic and 95 were neoplastic The lesions in the decreasing order of frequency were - nasal polyp, rhinoscleroma, tuberculosis, fungal infection, fibrous dysplasia, ossifying fibroma, cysts, nasal glioma, and cemento-ossifying fibroma. NP was involved by a range of neoplastic lesions; however, no non-neoplastic lesion was seen in this region.

Relapsing Polychondritis with Subglottic Stenosis Diagnosed after Tonsillectomy: A case report / 대한마취과학회지

Relapsing polychondritis (RP) is a rare autoimmune connective tissue disease of unclear pathogenesis and may present with the multisystem involvements and varied presentations, courses and responses to the therapy. The respiratory complaints or history such as dyspnea or snoring deserve to be in consideration, especially in the cases of no specific causes and must be ruled out the rare autoimmune airway diseases such as relapsing polychondritis, Wegener's granulomatosis, rhinoscleroma, or amyloidosis resulting in serious airway obstruction without adequate management. In this report we described a case of 15-year-old young male with the delayed diagnosis of relapsing polychondritis with intractable subglottic stenosis during the course of the management for several months despite of tonsillectomy for the relief of the airway obstruction.

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

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.

Scleroma of the Larynx: A Case Report

Scleroma of the larynx is a rare specific granulomatous disease and is usually presented as alaryngeal extension of a primary rhinoscleroma. We report an unusual case of laryngeal scleromain a 64-year-old female patient whose presented with progressive upper respiratory obstructivesymptoms lasted for 2 years. Symptoms were acutely exaggerated during the course of one month.On physical examination, the nasal mucosa was atrophic and crusted, and septal perforationwas noted. The fiberoptic laryngoscopy revealed a yellowish granulated epiglottic mass with adirty surface mimicking laryngeal cancer. Histological sectioning showed the characteristic featuresof scleroma including Mikulicz cells with scattered plasma cells and some neutrophils.Warthin-starry stains and electron microscopy examination showed numerous short rods insidethe cytoplasm of the Mikulicz cells. After the diagnosis, the patient underwent an emergency tracheostomyfollowed by long-term antibiotic treatment, and her symptoms have slowly improved.