[Rhinoscleroma of the cavum with expression in cervical lymph nodes: about a case]. / Rhinosclerome du cavum avec expression ganglionnaire cervicale: à propos d'un cas.

Rhinoscleroma is a specific granulomatous and chronic disorder with insidious evolution. It is causes by pathogen Klebsiella rhinoscleromatis. It mainly occurs in the nasal cavities and positive diagnosis is sometimes problematic. We report the case of a 19 year old female patient presenting with rhinoscleroma considered atypical due to its rare nasopharyngeal localization and its exceptional association with cervical lymphadenopathy in the right submandibular angle region. Anatomopathological exam revealed Mikulicz's cells, thus enabling the diagnosis. The patient underwent antibiotic therapy with ciprofloxacin for 16 weeks associated with washing of nasal cavities with physiological saline solution. Patient's outcome was favorable during the 14-month follow-up period.

Rhinoscleroma.

CONTEXT.­: Rhinoscleroma is a rare, chronic, infectious granulomatous process involving the upper respiratory tract caused by gram-negative bacilli, Klebsiella rhinoscleromatis. The site most commonly affected is the nasopharynx; however, lesions in various other locations have been described. OBJECTIVE.­: To review the literature for all the reported cases of rhinoscleroma in the past 5 years. DATA SOURCES.­: Published cases of rhinoscleroma from a PubMed (National Center for Biotechnology Information, Bethesda, Maryland) search were reviewed. CONCLUSIONS.­: Rhinoscleroma in nonendemic regions is extremely rare; however, with increased travel, immigration, and globalization, it is imperative to recognize this entity because the symptoms can be devastating and in some cases fatal. Although nasopharynx is the common site of involvement, unusual sites such as the trachea can be involved in rare cases. Rhinoscleroma can be managed effectively with a combination of antibiotics and surgical debridement and repair; however, recurrence rates do remain high.

Rhinoscleroma: a chronic infectious disease of poor areas with characteristic histological features - report of a series of six cases.

Rhinoscleroma is a chronic infectious disease that is endemic in certain poor areas of the world. The diagnosis often relies on pathology, the outcome is disappointing and then marked by recurrence. We present a retrospective series of six patients histologically diagnosed with rhinoscleroma. Some clinical and histological features of the disease are discussed below, as its adequate management implies a multidisciplinary approach.

[Rhinoscleroma: a rare chronic infection of the nasal cavities]. / Le rhinosclérome une infection chronique rare des fosses nasales.

We report the case of a 80-year old hypertensive female patient, presenting with bilateral nasal obstruction not improved by symptomatic treatment. The examination of the nasal cavity showed pseudotumoral hypervascularized granulomatous bleeding lesion on both sides of the anterior portion of the nasal septum (A). The remainder of the septal mucosa showed granulomatous appearance. CT scan of the facial bones showed tissue lesion of the nasal cavities on both sides of the septum which was moderately enhancing after injection of iodinated contrast medium (ICM), without bone lysis (B). Biopsy showed rhinoscleroma. The patient received combination therapy including cyclin associated with cotrimoxazole without disappearance of the nasal lesion. Endonasal excision of the septal mass was performed. Histological examination confirmed the diagnosis of rhinoscleroma. The patient underwent ciprofloxacin therapy for 1 month. Patient evolution was good with nasal clearance at 1-year follow up. Rhinoscleroma is a granulomatous infection of the nasal cavities due to enteric bacterium belonging to the Klebsiella family (Klebsiella rhinoscleromatis). Treatment is essentially medical. Surgery is used to remove obstructive pseudotumoral lesions resistant to medical treatment.

Rinoscleroma. Una entidad a considerar en la rutina diaria del patólogo / Rhinoscleroma: an entity to consider in the daily routine of the pathologist

El rinoscleroma es una enfermedad infrecuente en España, pero endémica en ciertas regiones del mundo. Se presenta como una enfermedad inflamatoria crónica granulomatosa progresiva que típicamente compromete la mucosa nasal, pero puede presentarse en otras localizaciones del tracto aereodigestivo superior. Su diagnóstico se apoya en estudios de imagen y en hallazgos clínicos como la deformidad nasal o la obstrucción de la vía aérea con disnea, disfonía y estridor respiratorio. Sin embargo, usualmente requiere confirmación histopatológica, aunque la detección de la bacteria Klebsiella rhinoscleromatis en etapas iniciales sería definitivo. Esta es una enfermedad que precisa un grado alto de sospecha y que debe tenerse en cuenta en la actualidad ante el incremento de la inmigración a nuestro medio desde regiones endémicas (AU)

The rhinoscleroma is a rare pathology in Spain, but endemic in certain regions of the world. It presents as a chronic, progressive and granulamatous entity, typically compromises the nasal mucosa, but it can occur in other locations of the upper aerodigestive tract. The diagnosis is based on clinical findings as nasal deformity or obstruction of the airways with dyspnoea, dysphonia and stridor and it relies on imaging techniques. However, ususally requires confirmatory histopathological diagnosis although detecting the bacterium Klebsiella rhinoscleromatis not in advance stages would be definitive. This is a disease that accurate a high degree of suspicion and should be taken into consideration currently due to the increase of immigration to our country from endemic regions (AU)

Rhinoscleroma of nose extruding into oral cavity.

Rhinoscleroma (RS) is a rare chronic granulomatous disease of the upper airways affecting nasal cavity, nasopharynx, and paranasal sinuses. Klebsiella rhinoscleromatis is the causative agent of this infection and Mikulicz cells are specific to this lesion. RS is commonly seen in poorer regions such as Central Africa, South America, Middle East, India and Indonesia. It is predominantly found in rural areas and people with poor socio-economic conditions. Most patients present with chronic rhinitis, sneezing, headache and deviated nasal septum similar to current case. An association with oral cavity has not been reported previously, as per authors' knowledge. This report describes a rare case of RS of nasal cavity extending into the oral cavity.

Rhinoscleroma in a 5-year-old Portuguese Child.

Rhinoscleroma is a chronic granulomatous infectious disease that is rare in Western Europe. We report the case of a 5-year-old Portuguese boy diagnosed with rhinoscleroma in the context of recurrent epistaxis. He had a 6-month course of antibiotic (amoxicillin plus clavulanate) therapy with full recovery.

A rare case of primary otoscleroma of the middle ear.

OBJECTIVE: We report an extremely rare case of primary otoscleroma. METHOD: We present a case report and a review of the world literature concerning otoscleroma. RESULTS: An adult woman presented with chronic suppurative otitis media with tubotympanic disease and conductive hearing loss. On mastoid exploration, dark granulations were seen, which were identified as otoscleroma on histopathological examination. The patient responded well to streptomycin. CONCLUSION: To the best of our knowledge, this is the first report of primary otoscleroma in the world literature. This case indicates that Frisch's bacillus can also spread to the middle ear.

Rhinoscleroma in an immigrant from Egypt: a case report.

Rhinoscleroma is a chronic indolent granulomatous infection of the nose and the upper respiratory tract caused by Klebsiella rhinoscleromatis; this condition is endemic to many regions of the world including North Africa. We present a case of rhinoscleroma in a 51-year-old Egyptian immigrant with 1-month history of epistaxis. We would postulate that with increased travel from areas where rhinoscleroma is endemic to other non-endemic areas, diagnosis of this condition will become more common.

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.

Grave afectación laringotraqueal secundaria a rinoescleroma por Klebsiella pneumoniae ssp. ozaenae / No disponible

No disponible

Rhinoscleroma: a French national retrospective study of epidemiological and clinical features.

BACKGROUND: Rhinoscleroma (RS) is a rare, chronic, granulomatous disease of the upper respiratory tract that is associated with infection with Klebsiella rhinoscleromatis. RS is more common in certain geographic regions than in others, but other risk factors and the pathogenesis of RS remain unclear. METHODS: We sent a standardized questionnaire to all pathologists and otolaryngology specialists in French University Hospitals and asked whether they had seen patients with RS in the previous 16 years (1990-2005). We then retrospectively reviewed the files of all patients identified. RESULTS: We collected 11 cases of RS, with a median patient age at diagnosis of 35.7 years (range, 5-72 years). The 3 patients with a familial history of RS presented with early-onset forms of RS; 1 had an uncommon aggressive presentation of the disease with ischemic stroke. Two unrelated consanguineous families were identified, 1 of which included 2 affected siblings. Two patients with sporadic disease were positive for HIV infection. All patients were living in France, but most were immigrants from areas where RS is endemic (North Africa, 3 of the 11 patients; West Africa, 4 patients; and Turkey, 1 patient). The probable duration of exposure to K. rhinoscleromatis in endemic areas varied widely: 0-28 years. Clinical features and outcome also varied considerably among cases. Biopsies had been performed for all patients and revealed granulomas containing Mikulicz cells. Cultures of biopsy tissue were positive for K. rhinoscleromatis in 5 of the 11 cases. Prolonged antibiotic treatment was administered to all patients, as follows: ciprofloxacin (7 patients), third-generation cephalosporins (2), tetracycline (2), and clofazimine (2). Eight of the 11 patients did not experience relapse during extended periods of follow-up (1.3-12 years). Relapses in 3 patients were confirmed by a second biopsy. CONCLUSIONS: The occurrence of early-onset RS in multiplex and/or consanguineous families suggests that genetic control of the host response to K. rhinoscleromatis may be involved in the pathogenesis of RS in endemic areas.

Klebsiella rhinoscleromatis of the membranous nasal septum.

Rhinoscleroma is a chronic, infectious, granulomatous disease that may present with mass lesions in the respiratory tract anywhere from the nose to the trachea. The nose is involved in 95-100 per cent of cases. There are three stages of the disease: catarrhal-atrophic, granulomatous (also known as hypertrophic) and sclerotic. The diagnosis is made either by positive Klebsiella rhinoscleromatis culture or from the classic histological findings of Mikulicz cells and transformed plasma cells with Russell bodies. Rhinoscleroma is endemic to areas of Africa, South-East Asia, Mexico, Central and South America, and Central and Eastern Europe, and it has been associated with low socioeconomic status. In the past, rhinoscleroma was infrequent in the US population but, with current trends in migration, the incidence of rhinoscleroma may be on the rise. There is often a delay in diagnosis in non-endemic areas such as the US due to unfamiliarity with the disease, the stage-dependent clinical and histological manifestations of disease, and the fact that only 50-60 per cent of cultures are positive for K rhinoscleromatis. Such late diagnosis leads to increased morbidity in the form of nasal and airway obstruction and nasal deformity from erosive processes. Rhinoscleroma is difficult to cure and prone to recur. Currently, the recommended treatment consists of a combination of surgical debridement and long-term antibiotic therapy. We present a case report of a culture-positive diagnosis of rhinoscleroma, and we review the existing literature.

Chronic nasal infection caused by Klebsiella rhinoscleromatis or Klebsiella ozaenae: two forgotten infectious diseases.

OBJECTIVES: Klebsiella rhinoscleromatis and Klebsiella ozaenae are associated with chronic diseases of the upper airways: rhinoscleroma and ozena, respectively. These have become uncommon in developed countries. We report herein one case of each disease in patients living in Marseilles, France, and include a review of the literature. METHODS: Diagnosis was made by direct evidence of bacteria (specific cultures and autoimmunohistochemistry on nasal biopsy) and using an indirect method (serology). In addition, the literature review showed that the majority of publications were old, confirming the fact that these diseases have been long forgotten. RESULTS: The specific and original methods used have allowed us to confirm the pathogenic role of K. ozaenae in ozena and confirmed rhinoscleroma in a granulomatous lesion. In the literature, K. rhinoscleromatis is only associated with rhinoscleroma whereas K. ozaenae is also associated with clinical diseases other than chronic rhinitis. CONCLUSIONS: In cases of chronic rhinitis, ozena and rhinoscleroma should be kept in mind, even in developed countries, and systematically screened for, especially as there are specific diagnostic tools and effective treatments available.

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.