Rhinoscleroma in a 9-year old boy: Rare case report.

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.

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.

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.

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.

Rhinoscleroma: A case report

Rhinoscleroma, or scleroma, is a chronic, slowly progressing granulomatous disease involving the upper respiratory tract, especially the nasal vestibules, choanae, pharynx, and larynx. Almost certainly the causative agent is Klebsiella rhinoscleromatis. The disease occurs frequently in Eastern Europe, the Middle East, and parts of Latin America, but it is hard to find such case in Korea. We prisent a case of rhinoscleroma involving the nasal vestibule in a 18-year-old male who was admitted due to nasal obstruction for 5 years and epistaxis for 2 months. The resected specimen was an irregular polypoid mass with relatively firm consistency and measured 3 cm in the largest diameter. Microscopically, the lesion was characterized by extensive fibrosis and inflammatory cell infiltration. The infiltrates consisted of predominantly lumphocytes, plasma cells, foamy or granular histiocytes which were singly scattered or grouped in clusters. In the cytoplasm of the histiocytes, round slightly basophilic bodies were noted. Warthin-Starry satin showed short positive rods within the cytoplasm of the cell (Mikulicz cell). Electron microscopically, the cytoplasm of Mikulicz cells contained large, round or irregular shaped clear vacuoles in which numerous Klebsiella bacilli attached to the boundaries of the vaculoes were noted. With higher magnifications, the bacilli were seen as roung or rod-shaped organisms.