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.

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.

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.

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.

Rhinoscleroma: a chronic infectious disease of poor areas with characteristic histological features - report of a series of six cases.

Rhinoscleroma is a chronic infectious disease that is endemic in certain poor areas of the world. The diagnosis often relies on pathology, the outcome is disappointing and then marked by recurrence. We present a retrospective series of six patients histologically diagnosed with rhinoscleroma. Some clinical and histological features of the disease are discussed below, as its adequate management implies a multidisciplinary approach.

Rinoscleroma. Una entidad a considerar en la rutina diaria del patólogo / Rhinoscleroma: an entity to consider in the daily routine of the pathologist

El rinoscleroma es una enfermedad infrecuente en España, pero endémica en ciertas regiones del mundo. Se presenta como una enfermedad inflamatoria crónica granulomatosa progresiva que típicamente compromete la mucosa nasal, pero puede presentarse en otras localizaciones del tracto aereodigestivo superior. Su diagnóstico se apoya en estudios de imagen y en hallazgos clínicos como la deformidad nasal o la obstrucción de la vía aérea con disnea, disfonía y estridor respiratorio. Sin embargo, usualmente requiere confirmación histopatológica, aunque la detección de la bacteria Klebsiella rhinoscleromatis en etapas iniciales sería definitivo. Esta es una enfermedad que precisa un grado alto de sospecha y que debe tenerse en cuenta en la actualidad ante el incremento de la inmigración a nuestro medio desde regiones endémicas (AU)

The rhinoscleroma is a rare pathology in Spain, but endemic in certain regions of the world. It presents as a chronic, progressive and granulamatous entity, typically compromises the nasal mucosa, but it can occur in other locations of the upper aerodigestive tract. The diagnosis is based on clinical findings as nasal deformity or obstruction of the airways with dyspnoea, dysphonia and stridor and it relies on imaging techniques. However, ususally requires confirmatory histopathological diagnosis although detecting the bacterium Klebsiella rhinoscleromatis not in advance stages would be definitive. This is a disease that accurate a high degree of suspicion and should be taken into consideration currently due to the increase of immigration to our country from endemic regions (AU)

Rhinoscleroma: a detailed histopathological diagnostic insight.

Rhinoscleroma (RS) is a chronic specific disease of nose and upper respiratory passages caused by Klebsiella rhinoscleromatis bacilli. It is endemic in Egypt and in sporadic areas worldwide. Diagnosis of RS depends on identification of the pathognomonic Mickulicz cells (MCs) which is most prominent during granulomatous phase but spares or absent during catarrhal or sclerotic phases of the disease. This study aimed to identify the potential diagnostic features of nasal RS when MCs are absent. Nasal biopsies from 125 patients complaining of chronic nasal symptoms were retrieved for this study; including 72 chronic non specific inflammatory lesions and 53 RS diagnosed by PAS and Geimsa stains. The detailed histological differences among the two groups were measured statistically. RS was frequently a bilateral disease (P < 0.05) of young age (P < 0.001) with a female predominance (P < 0.05) and usually associated with nasal crustations (P < 0.001). Five strong histological indicators of RS were specified by univariate binary logistic regression analyses including squamous metaplasia (OR 27.2, P < 0.0001), dominance of plasma cells (OR 12.75, P < 0.0001), Russell bodies (OR 8.83, P < 0.0001), neutrophiles (OR 3.7, P < 0.001) and absence of oesinophiles (OR 12.0, P < 0.0001). According to Multivariate analysis, the diagnostic features of RS in absence of MCs can be classified into major criteria including dominance of plasma cells infiltration and absence of oesinophiles and minor criteria including young age, female gender, bilateral nasal involvement, nasal crustation, squamous metaplasia, Russell bodies, and neutrophiles. The diagnostic model using the two major criteria confirmed or excluded RS in 84.3% of the investigated cases.

Rhinoscleroma in a 5-year-old Portuguese Child.

Rhinoscleroma is a chronic granulomatous infectious disease that is rare in Western Europe. We report the case of a 5-year-old Portuguese boy diagnosed with rhinoscleroma in the context of recurrent epistaxis. He had a 6-month course of antibiotic (amoxicillin plus clavulanate) therapy with full recovery.

[Expression and significance of IgG4 in inflammatory disease of nasal cavity and paranasal sinuses].

OBJECTIVE: To study the prevalence of IgG4-positive plasma cells in inflammatory disease of nasal cavity and paranasal sinuses and its association with IgG4-related sclerosing disease (IgG4-SD). METHODS: The expression of IgG4 and IgG in plasma cells of 103 cases diagnosed as inflammatory disease of nasal cavity and paranasal sinuses with dense lymphoplasmacytic infiltrate was studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system. RESULTS: Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (>50 per high-power field) in 28 cases, moderate infiltration (30 to 50 per high-power field) in 23 cases, mild (10 to 29 per high-power field) in 30 cases and negative (<10 per high-power field) in 22 cases (P < 0.05). Twenty-two cases studied fulfilled the diagnostic criteria of IgG4-SD (>50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including 3 cases of chronic sinusitis (3/20), 3 cases of nasal polyps (3/18), 3 cases of inflammatory pseudotumor (3/17), 4 cases of fungal sinusitis (4/20), 1 case of rhinoscleroma (1/12), 7 cases of Wegener's granulomatosis (7/11) and 1 case of Rosai-Dorfman disease (1/2). CONCLUSION: Inflammatory disease of nasal cavity and paranasal sinuses fulfilling the diagnostic criteria IgG4-SD is not uncommon. Definitive diagnosis of IgG4-SD requires correlation with other clinical and laboratory findings. Some cases of unexplained inflammatory disease of nasal cavity and paranasal sinus may represent a member of the IgG4-SD spectrum. IgG4 carries diagnostic value in differential diagnosis of inflammatory disease occurring in nasal cavity and paranasal sinuses.

[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.

Coexistence of rhinoscleroma with Rosai-Dorfman disease: is rhinoscleroma a cause of this disease?

INTRODUCTION: Rhinoscleroma and Rosai-Dorfman disease have been reported to coexist in the same patient at different sites. Rosai-Dorfman disease may have an aetiological relationship with rhinoscleroma, although this has not yet been proved. CASE REPORT: A case of a 42-year-old woman with recurrent nasal masses is presented, with histopathological proof of both conditions coexisting in the same nasal lesion. DISCUSSION: The aetiopathology, clinical features and treatment of both diseases are discussed and a literature survey is reported. Histologically, the presence of Mikulicz cells with entrapped, rod-like, Gram-negative bacilli and Russell bodies suggests rhinoscleroma. Emperipolesis and S-100-positive histiocytes confirm the diagnosis of Rosai-Dorfman disease. The presence of both in the same slides from affected tissues has never been demonstrated before. In the light of this evidence, the author believes that rhinoscleroma must be considered in the aetiology of Rosai-Dorfman disease.

Rhinoscleroma: a case series.

Rhinoscleroma is a chronic, slowly progressive, inflammatory disease of the upper respiratory tract. It is associated with Klebsiella rhinoscleromatis infection. We present the clinical and pathological features of four patients diagnosed with rhinoscleroma at the National Skin Centre, Singapore between 1997 and 2010. All four patients presented with only cutaneous involvement, and the diagnosis was clinched via histological examination. The patients were treated with a combination of antibiotics. Two patients who were on follow-up at the time of this writing responded positively to the antibiotic treatment, while two were lost to follow-up. Rhinoscleroma is a diagnostic challenge, as it is an uncommon disease in Singapore and Malaysia. We highlight this condition to raise awareness of the disease in order to aid in early diagnosis of patients. Without treatment, this condition can result in significant complications, including involvement of the lower airways. Early diagnosis and appropriate treatment help to reduce morbidity.