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.

Correlation Between Nasal and Laryngeal Lesions of Rhinoscleroma in Patients of Upper Egypt.

INTRODUCTION: Rhinoscleroma (RS) is a chronic granulomatous disease of URT caused by Klebsiella Rinoescleromatis. RS is considered endemic in Egypt. The nasal mucosa represents the primary region of occurrence. The disease can potentially spread to involve the larynx and trachea causing dysphonia, stridor, and airway obstruction. OBJECTIVES: To describe various nasal and laryngeal presentations of RS in our endemic area, to correlate between these findings and to alert physicians to suspect RS in any case of unexplained nasal or laryngeal lesion. METHODS: The study included 100 patients admitted in our otorhinolaryngolgy department (Minia University, Minia, Egypt). Patients presented with various manifestations of Rhino-pharyngo-laryngo scleroma. Diagnosis based on clinical, bacteriological, and pathological examination. RESULTS: Patients had typical nasal stages of RS, however; only 12 % of patients had the typical described laryngeal presentations (ie, subglottic narrowing and subglottic membrane). Other patients presented with atypical laryngeal presentations (eg, unhealthy vocal folds, ventricular fold hypertrophy, and suproglottic sticky greenish discharge). There was significant correlation between nasal stages and laryngeal lesions. CONCLUSION: RS can present with atypical laryngeal presentations in endemic areas which should be kept in mind to avoid misdiagnosis. Possible laryngeal lesions of RS can be predicted from observing associated nasal lesions.

[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.

Extranodal natural killer/T cell lymphoma with involvement of the bilateral submandibular lymph node mimicking a rhinoscleroma: a diagnostic challenge.

Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) is a rare and aggressive tumor that can mimic inflammatory conditions and other tumors, and present a diagnostic challenge. We report a 57-year-old Chinese man previously misdiagnosed with rhinoscleroma. The patient had undergone multiple biopsies when the histopathological diagnosis of ENKL was made. Because the diagnosis may be obscured by extensive necrosis, multiple biopsies are necessary to make a certain diagnosis. According to the diagnosis process of this patient, the need to consider a neoplastic cause should be highlighted when faced with an aggressive nasal disease not responsive to maximal treatment.

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 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: 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.

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.

Airway scleromas and their extensions.

BACKGROUND: Scleroma is a rare, chronic, granulomatous infectious disease of the respiratory tract mucosa which begins in the nose and spreads to the respiratory tract and adjoining structures. We report on the extensions and the management of 134 cases of scleroma in the Highlands region of Papua New Guinea. METHODS: The charts and treatment records of 134 scleroma cases were retrospectively reviewed from 1995 to 2013. The staging, extensions, treatment and results of treatment were reviewed and analysed. RESULTS: Of the 134 cases, 72 (53.7%) were females and the age ranged from 6 to 65 years. The disease was confined to the nose and nasopharynx in 71 (53.0%) cases. Extension of the disease from the nose to the Eustachian tube occurred in nine (6.7%) cases and into the middle ear in four (3.0%) cases. Further extensions to the larynx and trachea were seen in 17 (12.7%) cases each and the bronchus in three (2.2%) cases. Primary laryngoscleroma without involvement of the nose and nasopharyngeal stenosis without laryngeal involvement were managed in 11 (8.2%) and nine (6.7%) cases respectively. Others cases treated were ethmoid scleroma with proptosis and scleroma involving the upper lip in one (0.75%) case each. Ninety-nine (74.9%) patients were treated medically while 35 (26.1%) patients required surgery. Cure were achieved in 83 (61.9%) cases treated medically and 26 (19.4%) had successful surgical outcome. CONCLUSION: Scleroma begins in the nose and when not arrested, extends causing obstruction to the airway and cosmetic deformity which requires surgical intervention.

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.

Rhinoschleroma.

A 39 year-old man came to our institution because of a five-year history of a progressive painful growing mass on his left nostril, which cause airway obstruction with ulceration. Because of a suspicion of malignancy, surgery (mass resection and subtotal nasal reconstruction) was performed. Histologic samples ruled out malignancy, and tissue cultures for fungus and mycobacteria were negative, but regular aerobic cultures were positive for Klebsiella rhinoscleromatis. The patient was given a six-month regimen of ciprofloxacin, and a dramatic improvement was observed.

[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.