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.

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.

Patología infecciosa del vestíbulo nasal / Infectious diseases of the nasal vestibule

El vestíbulo nasal corresponde a la primera porción de la fosa nasal, éste se encuentra delimitado lateralmente por los cartílagos alares y medialmente por el borde caudal del septum nasal y la columela. Las enfermedades infecciosas del vestíbulo nasal son patologías frecuentes en la práctica clínica; su diagnóstico se realiza en base a sospecha clínica y examen físico, requiriendo habitualmente solo manejo médico ambulatorio. Desde el punto de vista etiológico pueden ser virales, bacterianas y fúngicas. Las principales especies bacterianas involucradas corresponden a Staphylococcus coagulasa negativa, S. epidermidis, S. hominis y S. haemolyticus, difteroides spp y S. aureus. Su manejo es esencialmente médico con casos excepcionales requiriendo manejo quirúrgico. En la actualidad existe escasa información epidemiológica al respecto, lo que dificultad la clasificación de los dichos cuadros clínicos. Se realizó una revisión de la literatura sobre cuadros infecciosos que afectan el vestíbulo nasal para lograr sistematizar y clarificar las distintas patologías y sus tratamientos.

The nasal vestibule corresponds to the first portion of the nasal passage, limited laterally by the lateral crus and medially by the caudal edge of the nasal septum and columella. Infectious diseases of the nasal vestibule are frequent in clinical practice, diagnosis is made based on clinical suspicion and physical examination, usually requiring only ambulatory medical management. In terms of etiology, they can be viral, bacterial and fungal. The main bacterial species involved correspond: Coagulase-negative Staphylococcus, S. epidermidis, S. hominis and S. haemolyticus, difteroides spp and S. aureus. Management is essentially medical and only exceptionally requires surgery. Currently, there is a lack of epidemiological information in this regard, which makes it difficult to classify these clinical conditions. A review of the literature on infectious conditions that affect the nasal vestibule was performed, to systematize and clarify the different pathologies and their management.

A Myriad of Scleroma Presentations: The Usual and Unusual.

To prospectively study a series of scleroma patients with a focus on socioeconomic status, clinical presentation, pathology, microbiology, and treatment. A total of 52 patients diagnosed with scleroma were included in the study. Demographic data of the patients were collected and clinical, radiographic, and histopathologic findings were analyzed. Biopsies from atypical patients were sent for electron microscopic examination. Patients were treated both medically and surgically with clinical follow-up to determine outcomes and the incidence of recurrence. Fifty-two patients were included in the study. There were 23 males (44.2%) and 29 females (55.8%). The highest incidence of scleroma was identified in individuals from poor, rural areas in the third to fourth decades of life. The nose was affected in all cases. Other affected sites included the nasopharynx, soft and hard palate, larynx and trachea, lacrimal passages, skin, and gingiva. Some patients had atypical presentations. Complete follow-up of all patients was hindered by non-compliance with clinic visits and the long duration of medical treatment. Recurrence or relapse of the granulomatous lesions after initial improvement occurred in 11 patients (21.2%) within 1 to 3 years. Scleroma is a stubborn, chronic, granulomatous bacterial disease. The diverse presentations may mimic neoplasms and other granulomatous conditions. We present cases with unusual presentations and demonstrate the transmission electron microscopic features of these lesions. Furthermore, we confirm the importance of achieving full eradication of the etiologic bacilli to prevent recurrent disease.

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.

Local immunity status of oropharynx in patients with scleroma

Introduction: Scleroma is a rare chronic granulomatous disease of the upper respiratory tract caused by Klebsiella pneumoniae subsp. rhinoscleromatis. To date its pathogenesis is as yet little understood. At the same time, scleroma is associated with a number of immune system disturbances. The aim: To study local immunity status of oropharynx in patients with scleroma, and to compare its parameters in various clinical forms of the disease. Material and methods: 20 apparently healthy subjects and 92 patients with scleroma (33 males, 59 females) underwent clinical immunologic evaluation. There were 31 patients with dominating infiltrative form of scleroma, 30 ­ with dominating atrophic form, 31 ­ with dominating scarring form. Concentration of secretory and monomeric immunoglobulin A, immunoglobulin G, α-interferon, interleukin 1ß in oropharyngeal secretion was determined by enzyme immunoassay. Resluts: Patients with scleroma were found to have altered local immunity of oropharyngeal secretion. There was a strong tendency for decreased concentration of secretory immunoglobulin A - 1.3-2.0 times, and decreased immunoglobulin G level ­ 1.5-2.3 times (р < 0.05) as compared to the values in healthy subjects. Specific features of local immunity in oropharyngeal secretion in various forms of scleromatous inflammatory process in upper respiratory tract were found: the most significant decrease of α-interferon concentration in atrophic and scarring forms of the disease, and the largest increase of anti-inflammatory interleukin 1ß and immune complex concentration in infiltrative form of scleroma. Conslusions: The study revealed deficiency of local immunity factors in oropharynx, being indicative of immunopathogenetic role of diagnosed disturbances in development and persistence of chronic inflammation in scleroma, and emphasizing the necessity of immunocorrection in complex therapy of the disease.

[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.

Long-term follow-up of repair of external nasal deformities in rhinoscleroma patients.

INTRODUCTION: Rhinoscleroma (RS) is a chronic, progressive, granulomatous infectious disease endemic in Egypt and the Middle East, affecting the nose in 95-100% of cases, and resulting in functional and esthetic sequelae. The aim of the study was to investigate the feasibility of performing reconstructive procedures to improve external deformities in RS patients, with long-term follow up. PATIENTS & METHODS: This prospective, controlled cohort study included 25 patients who were seeking rhinoplasty to improve their esthetic appearance, with evident clinical and histopathological history of RS. Another 25 patients seeking esthetic augmentation rhinoplasty in the same period formed the control group. All the participants were evaluated objectively by two independent rhinoplasty surgeons, and subjectively with ROE 2 years postoperatively. RESULTS: All the participants showed significant improvement in esthetic facial evaluation postoperatively, with a correlative rise in ROE score. There was an insignificant difference between the studied groups. Inconsequential complications were encountered throughout the follow-up period, but with insignificant incidence in both groups. CONCLUSION: Rhinoplasty for correction of RS external deformities is both safe and beneficial, with no risks of flaring up or increased complications. LEVEL OF EVIDENCE: Level 2b.

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.

Social geography of Rhinoscleroma and qualitatively and quantitatively abnormal cell-mediated immunity.

Rhinoscleroma is a progressive chronic granulomatous disease of the upper respiratory tract that may extend to the tracheobronchial tract. It is common belief that the pathology is determined by Klebsiella Rhinoscleromatis. In the authors' opinion, the infection with Klebsiella Rhinoscleromatis may not represent the only etiopathogenic factor of the disease. Rhinoscleroma is reported in many countries, but has a peculiar social and geographic distribution, in that it assumes an endemic character only in some regions of the Middle East, West Russia, North Africa, Indonesia, Central and South America. In Europe, most of the cases are reported in Poland, Hungary and Romania. In Italy, Rhinoscleroma is almost exclusively located in the southern and island regions. Rhinoscleroma is predominantly reported in rural areas, in the presence of poor socio-economic conditions, which according to many authors would be a co-factor triggering the disease. In this article, the authors review some inconsistencies in etiology, histology and epidemiology of Rhinoscleroma. Based on the overall picture, they propose that intrinsic factors, possibly of genetic origin, may give rise to the disease, and suggest possible lines of research to distinguish between extrinsic and intrinsic factors as determinants for Rhinoscleroma.

Rhinoscleroma pathogenesis: The type K3 capsule of Klebsiella rhinoscleromatis is a virulence factor not involved in Mikulicz cells formation.

Rhinoscleroma is a human specific chronic granulomatous infection of the nose and upper airways caused by the Gram-negative bacterium Klebsiella pneumoniae subsp. rhinoscleromatis. Although considered a rare disease, it is endemic in low-income countries where hygienic conditions are poor. A hallmark of this pathology is the appearance of atypical foamy monocytes called Mikulicz cells. However, the pathogenesis of rhinoscleroma remains poorly investigated. Capsule polysaccharide (CPS) is a prominent virulence factor in bacteria. All K. rhinoscleromatis strains are of K3 serotype, suggesting that CPS can be an important driver of rhinoscleroma disease. In this study, we describe the creation of the first mutant of K. rhinoscleromatis, inactivated in its capsule export machinery. Using a murine model recapitulating the formation of Mikulicz cells in lungs, we observed that a K. rhinoscleromatis CPS mutant (KR cps-) is strongly attenuated and that mice infected with a high dose of KR cps- are still able to induce Mikulicz cells formation, unlike a K. pneumoniae capsule mutant, and to partially recapitulate the characteristic strong production of IL-10. Altogether, the results of this study show that CPS is a virulence factor of K. rhinoscleromatis not involved in the specific appearance of Mikulicz cells.