First report of rare persistence granulomatous extra respiratory Rhinoscleroma of oral pathology.

Rhinoscleroma (RS) is a rare chronic specific progressive granulomatous disease of the upper airway and affect the nasal cavity, larynx, nasopharynx and may spread to the lower respiratory tract. Extra-respiratory involvement has rarely been described. A case report of extra-respiratory RS with oral manifestation in Egyptian female patient has been presented as a tumour extruded from mucosal lining of upper lip. She was living in crowded conditions with malnutrition and poor hygiene. On the first look, the lesion appeared to be carcinoma without any indication of infectious disease, and then patient was prepared for incisional biopsy. Upon clinicopathological evaluation, the diagnosis was made as RS in the granulomatous stage based on the presence of dense plasma cell infiltration with Mikulicz cells and Russell bodies. Long term oral ciprofloxacin 500 mg twice/day was started as a single treatment. By the end of six weeks antibiotic therapy, the large granulomatous mass reached the cicatricial stage, became very stiff fibrotic mass with sclerotic scar, markedly indurated & significantly decreased in size. This case shows the significant of through clinical examination and lab investigations to achieve correct diagnosis.

[Widespread of Scleroma. A rare clinical case]. / Skleroma, rasprostranennaya forma. Redkoe klinicheskoe nablyudenie.

Scleroma is a chronic infectious disease characterized by developing inflammation nye granulomas, mainly of the upper respiratory tract, with subsequent scarring. The reasons for the development of scleroma remain unclear, but it is generally accepted that the gram-negative coccal bacterium Klebsiella rhinoscleromatis (pas-Volkovich's loch - Frisch) is the causative agent of this disease. Endemic in the spread of scleroma is considered There are tropical and temperate zones such as Africa, Asia, Eastern Europe, South America and Central America. Features of development scleroma in the present case and the complexity of the diagnosis caused by atypical, the rapid development of the disease, the greater prevalence of the process (including the orbit), with the transition to the soft tissues of the cheek bone and upper jaw, which is not typical for scleroma. The process was primarily localized in the left maxillary sinus and mimicked sinusitis. The presence of radicular cysts and darkening of the left maxillary sinus on radiographs, taking into account the clinical picture of the disease, were interpreted incorrectly, which led to the establishment of an incorrect diagnosis and subsequent surgical interventions. More targeted and consistent examination of histo-the logical material obtained after three surgical interventions made it possible to identify the characteristic signs scleroma, establish the correct diagnosis and prescribe effective treatment. In our case, we applied Ciprofloxacin course for 3 months at a maximum daily dose of 2000 mg in combination with probiotics. During treatment, it was noted a significant improvement in the patient's well-being, facial pains became less intense, a decrease in the volume of ma tissues of the left cheek and improved nasal breathing.

Tracheal rhinoscleroma.

INTRODUCTION: Tracheal rhinoscleroma is an infectious granulomatosis of the tracheobronchial tract caused by a Gram-negative bacillus. Exclusively tracheal involvement has been rarely reported in the literature. The purpose of this study was to report a case of subglottic stenosis secondary to rhinoscleroma. SUMMARY: A 46-year-old North African woman with no medical or surgical history presented with inspiratory dyspnoea that had been present for several years. Endoscopic examination under general anaesthesia revealed tracheal stenosis. Histological examination of mucosal biopsies demonstrated Mikulicz cells and culture of bacteriological samples taken during a second biopsy confirmed the diagnosis of rhinoscleroma. CO2 laser subglottic obstruction relief was performed and treatment with ofloxacin was initiated. No recurrence of tracheal stenosis was observed with a follow-up of 6 months. DISCUSSION: The diagnosis of rhinoscleroma is based on histological and bacteriological examination. Cultures are positive in 60% of cases, but negative cultures do not exclude the diagnosis of rhinoscleroma. Specific treatment consists of long-term antibiotic therapy, while surgery may be indicated for symptomatic treatment.

Topical mitomycin C as an adjunct to surgical debulking and medical treatment in rhinoscleroma.

BACKGROUND: Rhinoscleroma is a chronic granuloma that is endemic in Egypt and is caused by gram-negative bacilli (Klebsiella rhinoscleromatis). The nasal mucosa is affected in almost all cases, which causes nasal obstruction, anosmia, and epistaxis. The disease usually passes through an atrophic stage (atrophic rhinitis) and a granulomatous stage before ending in the fibrotic stage, with possible bone destruction. OBJECTIVE: This study assessed the effect of topical mitomycin C (MMC) on reducing the recurrence of granulation tissue and intranasal adhesions after endoscopic debulking and continuous medical treatment of nasal rhinoscleroma. MATERIALS AND METHODS: This double-blind randomized clinical trial was performed with 30 patients who had rhinoscleroma (granulomatous and fibrotic stages). At the end of endoscopic debulking, meshes impregnated with MMC was placed in one nasal cavity for 5 minutes, while another meshes impregnated with saline solution were placed in the opposite side. Repeated meshes with MMC were placed in the same side during the follow-up period. Patients were followed up for 1 year. The recurrence of granulation tissue and the degree of intranasal adhesions were recorded and compared according to the Lund-Kennedy score. RESULTS: At 12 months' follow-up, among the total 30 patients, 30 MMC sides were compared with 30 control sides: 69% of MMC sides versus 32% of control sides had no recurrence of granulation tissue (p = 0.01) and 65% of MMC sides versus 24% of control sides had no recurrence of intranasal adhesions (p = 0.03). The Lund-Kennedy score decreased from 2.1 ± 0.64 to 0.8 ± 0.41 and from 2.3 ± 0.33 to 1.9 ± 0.20 in the MMC sides and control sides, respectively, with a nonstatistically significant lower incidence of intranasal adhesions in the MMC sides. CONCLUSION: Topical MMC may reduce granulation tissue and intranasal adhesion formation in patients with rhinoscleroma. Further studies with a larger number of samples and longer follow-up periods are recommended.

[Rhinoscleroma disease accompanied with laryngotracheal stenosis: a case report].

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.

Rhinoscleroma: a case series report and review of the literature.

Rhinoscleroma is a rare chronic granulomatous infectious disease and important differential diagnosis in developing countries and emigrants from these regions. In this survey we present seven cases from University Hospital Kigali, Rwanda, and one more from Germany. All cases are discussed on the background of the current literature.

[Giant rhinoscleroma]. / Rhinosclérome géant.

UNLABELLED: Rhinoscleroma is a chronic granulomatous respiratory tract disease. The initial lesion site is often intra-nasal. Giant tumor presentations are rare. The authors report a case of extensive nasal rhinoscleroma. OBSERVATION: A 35-year-old African male patient consulted for a large tumor of the nose. The tumor had appeared 25 years before; the initial lesion site was intra-nasal. It presented as a small non-obstructive growth. The tumor was removed 20 years before, and recurred in an exophytic presentation. The main tumor was associated to peripheral, peri-nasal, and upper lip nodules. There was a central granuloma, bleeding on contact. Nasal obstruction was complete. The histological and bacteriological examination of a biopsy specimen confirmed the diagnosis of rhinoscleroma. Trimethoprim-sulfamethoxazole was effective on the infection. DISCUSSION: Rhinoscleroma is frequently located on the nasal mucosa, but extra-nasal giant tumor presentations are rare. It can involve the whole respiratory tract. It is endemic in developing countries. Sporadic cases have been described in non-endemic areas, among migrants. The diagnosis is proved by histology. Specific and early antibiotic therapy is effective. It avoids surgical mutilation, sequels, and recurrence.

[Rhinoscleroma: two cases in Bobo-Dioulasso in Burkina Faso]. / Le rhinosclérome: deux observations à Bobo-Dioulasso au Burkina Faso.

INTRODUCTION: Rhinoscleroma is a chronic granulomatous infection of the respiratory tract due to Klebsiella rhinoscleromatis (K. rhinoscleromatis). PATIENTS AND METHOD: We observed two cases of rhinoscleroma in the Souro-Sanou university hospital of Bobo-Dioulasso, Burkina Faso, over a 9-year period (2009 to 2010). RESULTS: A 19-year-old man and a 38-year-old woman both consulted with obstructive granuloma of the nasal fossae; one also had a velar localization. The treatment consisted of surgical debridement and antibiotic therapy (ciprofloxacin). Good results followed the surgery. On follow-up after two and one and a half years, we observed no relapse. CONCLUSION: The diagnosis of rhinoscleroma should be considered for any obstructive tumor of the nasal fossae. Histopathological examination contributes to the diagnosis.

Rhinoscleroma: a retrospective study of pathologic and clinical features.

OBJECTIVE: Rhinoscleroma, a chronic granulomatous bacterial disease of the nasal mucosa that often extends through the lower respiratory tract, is caused by infection with the gram-negative bacillus Klebsiella rhinoscleromatis (KR). We report the clinicopathology and histology associated with KR infection-induced rhinoscleroma in patients admitted to the Beijing Tongren Hospital over a 30-year period. METHODS: The clinical and pathologic features of 40 cases of upper aerodigestive tract infections were retrospectively studied. Histochemical examination of biopsy samples was performed, including periodic acid-Schiff, modified Warthin-Starry, and acid-fast stains. In addition, immunohistochemical staining for CD43, CD20, CD68, and lysozymes was performed in 11 specimens, and 8 specimens were analyzed by transmission electron microscopy. RESULTS: KR infection was confirmed in each of the 40 samples. Twenty-seven patients remained relapse free 1 to 10 years following treatment with antibiotic supplemented in some cases with surgery or radiotherapy, and all 13 cases of relapse were successfully eradicated by the end of treatment. KR infection was localized to phagosomes within Mikulicz cells, as determined by immunohistochemistry and electron microscopy. Significant tissue injury was observed in most cases. CONCLUSION: Long-term antibiotic therapy successfully eradicated KR infection in all cases. Although late diagnosis was common in this cohort, retrospective examination of biopsy samples suggests that diagnosis can be improved by combining clinical findings with histologic analysis.

Retrospective study of the rhinoscleroma about 14 cases in ENT departments of university hospitals (Côte d'Ivoire).

PURPOSE OF STUDY: To report the epidemiological, clinical, therapeutic and evolutionary aspects of rhinoscleroma in ENT departments of university hospitals (Côte d'Ivoire). MATERIAL AND METHOD: Retrospective study of rhinoscleroma conducted in the ENT and head and neck surgery departments in Côte d'Ivoire from January 1980 to December 2008 including the cases of confirmed rhinoscleroma and the treated cases. RESULTS: Fourteen cases of rhinoscleroma were found in 28 years. The early manifestations were not specific enough so the patients were seen with clinical status with obvious disorders or physical discomfort. Treatment was medical and surgical. Medical therapy was based on streptomycin, thiopenicol or ciprofloxacin administration. Surgery consisted in removing the fibrous adhesions to correct the functional and aesthetic disorders. CONCLUSION: Rhinoscleroma has become a more and more rare disease because of the sensitivity to the new molecules. Diagnosis can be difficult and delayed because of its clinical polymorphism.

Rhinoscleroma: an updated experience through the last 10 years.

CONCLUSION: Rhinoscleroma is a chronic, specific, granuloma of the nose and other parts of the respiratory system. The disease is endemic in Egypt and many other countries. The causative organism is Klebsiella rhinoscleromatis bacillus, proved by fulfilling Koch's postulates. The mode of infection is not known and its worldwide irregular geographical distribution is not understood. Lines of treatment are unsatisfactory and a tendency for recurrence is the rule. OBJECTIVES: Our aim was to study the clinical presentation, microbiology, pathological staging, follow-up, and lines of treatment of new rhinoscleroma patients admitted or seen at Alexandria Main University Hospital from January 1999 until January 2009. METHODS: Demographic data and the results of clinical, bacteriological, and histological examinations were reviewed. Medical and surgical treatments were evaluated. Follow-up as regards the results of treatment and incidence of recurrence was assessed. RESULTS: Fifty-six patients were included in the study. There were 26 males and 30 females, and 85% of patients presented in the third and fourth decades of life. The nose was affected in 100% of patients. Other regions affected were the nasopharynx in 13 patients, palate in 7 patients, skin in 2 patients, larynx in 3 patients, trachea in 17 patients, nasolacrimal duct in 2 patients, and premaxilla in 1 patient. No lymph node affection was reported. Klebsiella rhinoscleromatis strain III was isolated from 100% of patients. Antibiotics used were a combination of trimethoprim-sulfamethoxazole 400 mg and rifampicin 300 mg twice daily for 3 months. Since 2003, this was replaced by ciprofloxacin 500 mg twice daily for 3 months. Surgical procedures performed were removal of nasal granulations, bronchoscopic dilatation, bipolar coagulation of skin lesions, tracheostomy, and repair of pharyngeal stenosis. Results were disappointing, as a large number of patients did not attend for follow-up. A high incidence of recurrence was found, reaching up to 25% within 10 years.

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.

[Rare concurrence of airway scleroma and nonspecific aortoarteritis (Takayasu's disease)].

A case of a rare concomitance of airway scleroma and nonspecific aortoarteritis was studied in a 14-year-old girl, a native from the scleroma endemic Rovno Region. She had been treated for 4 years for this disease verified by a histological study of a laryngeal biopsy specimen. The fact that scleroma was a predictor of Takayasu's disease in this case cannot be ruled out; suggesting a pathogenetic association between both diseases. Concentric narrowing of the lumen of the aorta and its branching vessels contributed to the development of hypoxia and CNS ischemia, which worsened the severity of a pathological process in the airway and caused a fatal outcome during an episode of asphyxia.

Rhinoscleroma of the sinuses.

INTRODUCTION: Rhinoscleroma is a rare chronic, granulomatous disease of the respiratory tract. OBJECTIVE: The aim of this paper was to report 2 unusual cases of rhinoscleroma and to review the literature. MATERIAL: We present two cases of sinus rhinoscleroma diagnosed and treated at the ENT Department of a French University hospital. RESULTS AND CONCLUSION: Rhinoscleroma primarily affects the nasal cavity but the nasopharynx (18%-43%), larynx (15%-40%), trachea (12%) and bronchi (2% to 7%) can also be involved. However, the paranasal sinuses are usually free of disease. Rhinological signs are generally the first reported by patients. CT scan and MRI are useful for diagnosis. Diagnosis of rhinoscleroma was based on histological characteristics and presence of Klebsiella rhinoscleromatis on biopsy cultures. In most cases treatment involves prolonged antibiotic therapy with aesthetic surgical reconstruction when necessary. However, rhinoscleroma is difficult to eradicate and its recurrence rate is high.

Rhinoscleroma in three siblings.

Rhinoscleroma is a chronic, granulomatous infectious disease that responds poorly to treatment. In recent years an increasing number of cases have been reported in nonendemic areas, explained largely by major migratory movements. We describe rhinoscleroma in three siblings. They had ulcerated but painless lesions, which bled spontaneously, and hemorrhagic scabs or crusts in their noses. In one child, the lesions had destroyed the entire left nasal ala and alar cartilage and most of the right. Dermatopathologic study identified the Mikulicz macrophages that contained organisms. It is possible that disposing factors could have been the neutropenia common to the three children and their poor living conditions. They were treated with a combination of trimethoprim-sulfamethoxazole and cefalexin, for a period of 3 months. We present this unusual case history of three siblings affected by a process that is relatively infrequent in our area of practice and is not considered very contagious. It is important to recognize the clinical signs characteristic of this disease, the diagnosis of which is not easy. Improvements in living conditions, hygiene, and health standards are essential prerequisites for its control and prevention.