UNASSIGNED: Rhinoscleroma is classically described as a chronic granulomatous disease caused by Klebsiella rhinoscleromatis which primarily affects the nose and nasopharynx. When present, tracheal manifestations will be seen late in the disease course rather than on initial presentation. We describe a rare case of nonendemic rhinoscleroma that presented with tracheal lesions as an initial manifestation of disease.
UNASSIGNED: Case report and literature review.
UNASSIGNED: An 88-year-old male presented with longstanding dysphonia. Flexible laryngoscopy demonstrated a septal perforation and diffuse glottic lesions. CT neck demonstrated a nonobstructive polypoid tracheal lesion and mucosal thickening of the paranasal sinuses. Biopsy confirmed an atypical lympho-histiocytic proliferation and microorganisms within macrophages on Grocott methenamine silver and Steiner stains consistent with rhinoscleroma. He was referred for rheumatology and pulmonology consultation.
UNASSIGNED: Systemic diseases rarely affect the trachea, and even less frequently is a tracheal lesion identified as the initial manifestation of disease. The most common systemic diseases that affect the trachea include relapsing polychondritis, granulomatosis with polyangiitis, amyloidosis, and inflammatory bowel disease. The literature surrounding nasolaryngotracheal rhinoscleroma is limited, especially in nonendemic areas. It is necessary to include unusual etiologies of airway lesions in the differential diagnosis, which warrants comprehensive airway evaluation including biopsy.
UNASSIGNED: 4.

Rhinoscleroma is a rare benign granulomatous disease in children, the pathogen of which is Klebsiella rhinoscleromatis. Rhinoscleroma almost always affects the respiratory tract, the nasal cavities are the most common site, but can also be found over the entire extent of the respiratory tree, clinically the patient often presents with nasal obstruction with other non-specific signs, Histology is the basis of the diagnosis of rhinoscleroma. Early and appropriate medical treatment improves the prognosis and prevents progression to disabling sequelae. We present the case of a 9-year-old child who presented to the pediatric emergency department with a 2-month history of nasal obstruction unresponsive to medical treatment (Oxymetazoline). This was accompanied by mild nasal swelling and intermittent episodes of epistaxis. A CT scan of the paranasal sinuses was performed, ruling out a malignant tumor in the nasal cavity due to the absence of bone lysis. A nasal cavity biopsy with histopathological examination was conducted, confirming the diagnosis of rhinoscleroma. This was supported by the presence of an infiltrate rich in histiocytes and plasma cells, along with the identification of Russell bodies during PAS (Periodic Acid Schiff) staining. The patient underwent a medical treatment regimen involving a sulfamide-based antibiotic therapy (trimethoprim/sulfamethoxazole) for 8 weeks, along with a short-term corticosteroid therapy at a dose of 1 mg/kg/24 hours. At the first follow-up after 1 month, there was a noticeable reduction in nasal swelling and an improvement in nasal obstruction. A second follow-up 3 months later showed favorable results, with complete disappearance of both the swelling and nasal obstruction. Considering the possibility of recurrence, an annual follow-up was decided upon.

暂无摘要。

Rhinoscleroma is an infectious granulomatous disease. It is important to identify pathognomonic Mikulicz cells on microscopy, as these can be rare and the chronic inflammatory infiltrate can appear otherwise nonspecific on biopsies.

Rhinoscleroma is an uncommon chronic infection caused by Klebsiella rhinoscleromatis, a gram-negative bacillus that affects the upper respiratory tract. Although the nasal cavity is the most affected area, pulmonary involvement has not been previously documented. This case report describes a 61-year-old man followed for nasal rhinoscleroma who was admitted to the intensive care unit with acute respiratory distress and septic shock due to bilateral infectious bronchopneumonia. Despite immediate intubation and intravenous antibacterial treatment, the patient died 10 days later. Klebsiella rhinoscleromatis was identified in a distal pulmonary swab and blood culture. While cases of rhinoscleroma and bacteremia caused by K rhinoscleromatis infection have been documented, the case we present here is the first to report on acute respiratory distress syndrome caused by fulminant pneumonia along with septic shock.

Extensive involvement of the rhinoscleroma to the multiple paranasal sinuses, nasopharynx, and oropharynx is infrequent in clinical practice. Although pathology is diagnostic, other similar pathology should be ruled out in each case due to the nonspecific clinical symptoms of the patients. Radiology is always the key investigation in patients with sinonasal rhinoscleroma to find out the involvement of the disease and for proper surgical planning. Although majorities of the patients get benefited from prolonged medical treatment, surgery is warranted in cases of obstruction of the upper airway and significant nasal deformities. Preservation of the bony- cartilaginous framework is really a challenge in extensive sinonasal rhinoscleroma, especially in revision cases. A close follow-up is required in each case of sinonasal rhinoscleroma due to the high chance of its recurrence. In the present case, we have presented an extensive sinonasal rhinoscleroma which was successfully managed by surgery.

Granulomatous formation in the nose and paranasal sinuses still presents an unmet clinical challenge as it affects both the physical health and personality of patients, and the lack of a systematic diagnostic and disease management approach has further complicated the scenario. Occurrence of granulomatous nasal disorder in the rural Indian population is associated with several factors such as lack of proper medical care, lack of hygienic and clean working and living conditions, and limited financial abilities to access the already overburdened primary healthcare system. This study aims to understand the correlation disease incidence, manifestation of signs and symptoms and associated socio-epidemiological parameters for 104,000 patients over a period of 23 months in Odisha. Primarily the study used socio-epidemiological surveys collected, annotated, and curated independently for granulomatous nasal disorder patients and compared this with their clinical records for signs & symptoms and performed several statistical analyses to better understand the disease incidence correlation with age, socio-economic and gender based parameters. Overall results suggest that males from low socio-economic background living in rural areas are the most vulnerable population and commonly affected with granulomatous nasal disorder (commonly Rhinosporidiosis sub-type) with common origin of the disease reported at the nasal septum and floor. We also highlight that change in the mass of nose followed by bleeds on touch, nasal discharge and hanging mass in throat are the most common manifestations presented by patients with granulomatous nasal disorders. Together, this research work identifies and characterise clinical and socio-economic factors that lead to and exacerbate the incidence of granulomatous nasal disorders.

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.

暂无摘要。

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.
Склерома представляет собой хроническое инфекционное заболевание, характеризующееся формированием воспалительных гранулем, преимущественно верхних дыхательных путей, с последующим рубцеванием. Причины развития склеромы остаются невыясненными, но принято считать, что грамотрицательная кокковая бактерия Klebsiella rhinoscleromatis (палочка Волковича—Фриша) является возбудителем этого заболевания. Эндемичными по распространению склеромы считаются тропические и умеренные зоны, такие как Африка, Азия, Восточная Европа, Южная Америка и Центральная Америка. Особенности течения склеромы в представленном случае и сложности в установлении диагноза обусловлены нетипичным, достаточно быстрым развитием болезни, большим распространением процесса (в том числе в орбиту), с переходом на мягкие ткани щеки и кость верхней челюсти, что не характерно для склеромы. Процесс первично локализовался в левой верхнечелюстной пазухе и имитировал синусит. Наличие радикулярных кист и затемнение левой верхнечелюстной пазухи на рентгенограммах с учетом клиники болезни интерпретированы неверно, что привело к установлению неправильного диагноза и последующим хирургическим вмешательствам. Более прицельное и последовательное исследование гистологического материала, полученного после трех хирургических вмешательств, позволило выявить характерные признаки склеромы, установить правильный диагноз и назначить эффективное лечение. В нашем случае мы применили Ципрофлоксацин курсом на 3 мес в максимальной суточной дозе 2000 мг в сочетании с пробиотиками. На фоне лечения отмечено значительное улучшение самочувствия пациентки, лицевые боли стали менее интенсивными, отмечено уменьшение объема тканей левой щеки и улучшение носового дыхания.