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.

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.

[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.

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: a case report.

Rhinoscleroma is a chronic inflammatory disease in which granulation tissue with a typical cell content is found. The paper presents the case of a 77-year-old woman with clinically diagnosed nodule in the nasal cavity. The histopathological examination revealed granulation tissue with plasma cells and Mikulicz's cells. The clinical and morphological picture of the case in question is a rare opportunity to bring to mind a disease that used to be common in Poland and which clinically can imitate malignant tumour.

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.

[Pathologic diagnosis of specific infections in upper aerodigestive tract].

OBJECTIVE: To find a fast and simple method for detection of specific pathogens in upper aerodigestive tract. METHODS: Sixty-one cases of specific infections in upper aerodigestive tract encountered during a 10-year period in Beijing Tongren Hospital were retrospectively studied. Six histochemical stains, including PAS, Giemsa, Gram, methylene blue, modified Warthin-Starry and acid-fast stains were applied. The morphology of different pathogens was studied and the staining patterns were compared. RESULTS: There were 23 cases of pharyngeal treponemal infection, 10 cases of short treponemal infection, 4 cases of mycobacterial infection, 4 cases of infection by rhinoscleroma bacilli, 1 case of sinonasal fungal infection, 1 case of combined infection of bacteria and Oidium albicans, 2 cases of tonsillar Actinomycetes and 16 cases of non-specific bacterial infections. Both pharyngeal treponemal infection and infection by rhinoscleroma bacilli could be detected by modified Warthin-Starry stain. As for sinonasal fungal infection, PAS, Giemsa and modified Warthin-Starry stains were useful in differentiating different types of fungi. Mycobacteria were best demonstrated by conventional acid-fast stain. CONCLUSIONS: Special histochemical stains performed on histologic sections are useful for diagnosing specific infections in upper aerodigestive tract.

[The specific features of the pathological morphology and pathogenesis of scleroma: morphological, histochemical, and electron microscopic studies].

The specific features of the pathological histology of scleroma have been continuously studied at the Pathology Department of the Lvov Medical University since 1945. A total of 1025 biopsy specimens from 656 patients with scleroma were examined. Along with diverse dystrophic and metaplastic changes in the epithelial coverings, which were affected by scleroma, there were its original inflammatory enlargements that were likely to play an active part in the inflammatory process. Abundant formation of fibrous structures in the granulation tissue undoubtedly reflects the protective functioning of an organism. Impaired innervation led not only to ageusia and anosmia in patients with scleroma; loss of nervous communications with the organism is very likely to affect the course of the pathological process to either degree. Electron microscopy provided more evidence for the presence of endobiosis in scleroma and the inability of drugs to affect directly the Frish bacillus due to its secreted mucopolysaccharide the composition of which is little studied.

Rhinoscleroma: a French national retrospective study of epidemiological and clinical features.

BACKGROUND: Rhinoscleroma (RS) is a rare, chronic, granulomatous disease of the upper respiratory tract that is associated with infection with Klebsiella rhinoscleromatis. RS is more common in certain geographic regions than in others, but other risk factors and the pathogenesis of RS remain unclear. METHODS: We sent a standardized questionnaire to all pathologists and otolaryngology specialists in French University Hospitals and asked whether they had seen patients with RS in the previous 16 years (1990-2005). We then retrospectively reviewed the files of all patients identified. RESULTS: We collected 11 cases of RS, with a median patient age at diagnosis of 35.7 years (range, 5-72 years). The 3 patients with a familial history of RS presented with early-onset forms of RS; 1 had an uncommon aggressive presentation of the disease with ischemic stroke. Two unrelated consanguineous families were identified, 1 of which included 2 affected siblings. Two patients with sporadic disease were positive for HIV infection. All patients were living in France, but most were immigrants from areas where RS is endemic (North Africa, 3 of the 11 patients; West Africa, 4 patients; and Turkey, 1 patient). The probable duration of exposure to K. rhinoscleromatis in endemic areas varied widely: 0-28 years. Clinical features and outcome also varied considerably among cases. Biopsies had been performed for all patients and revealed granulomas containing Mikulicz cells. Cultures of biopsy tissue were positive for K. rhinoscleromatis in 5 of the 11 cases. Prolonged antibiotic treatment was administered to all patients, as follows: ciprofloxacin (7 patients), third-generation cephalosporins (2), tetracycline (2), and clofazimine (2). Eight of the 11 patients did not experience relapse during extended periods of follow-up (1.3-12 years). Relapses in 3 patients were confirmed by a second biopsy. CONCLUSIONS: The occurrence of early-onset RS in multiplex and/or consanguineous families suggests that genetic control of the host response to K. rhinoscleromatis may be involved in the pathogenesis of RS in endemic areas.

Rosai-Dorfman disease presenting as laryngeal masses.

Extranodal lesions may be the sole manifestation of Rosai-Dorfman disease (RDD). Although the head and neck region is one of the most common extranodal sites, laryngeal involvement is very rare. A 44-year-old woman presented with a complaint of progressive dyspnea. She had a three-year history of treatment for asthma and a history of operation for a nasal mass that afflicted her for 15 years and was diagnosed as rhinoscleroma. On physical examination, she had three subcutaneous lesions, in the left lower eyelid, right epicanthal area, and left forearm, respectively. No lymphadenopathy was present. Laryngoscopic examination revealed three solid, polypoid masses in the subglottic region, 1 cm in diameter. With a two-staged operation, the laryngeal masses were excised totally together with the subcutaneous lesions. Histological examination of all the specimens showed proliferation of histiocytes. Immunohistochemical staining revealed typical S-100 protein-positive histiocytes and emperipolesis. Both laryngeal and subcutaneous lesions were diagnosed as RDD. A re-evaluation of sections from the previous operation specimen of the nasal mass showed the same morphological features. The patient was healthy without recurrence, nine months following surgery.

[Rare concurrence of airway scleroma and nonspecific aortoarteritis (Takayasu's disease)].

A case of a rare concomitance of airway scleroma and nonspecific aortoarteritis was studied in a 14-year-old girl, a native from the scleroma endemic Rovno Region. She had been treated for 4 years for this disease verified by a histological study of a laryngeal biopsy specimen. The fact that scleroma was a predictor of Takayasu's disease in this case cannot be ruled out; suggesting a pathogenetic association between both diseases. Concentric narrowing of the lumen of the aorta and its branching vessels contributed to the development of hypoxia and CNS ischemia, which worsened the severity of a pathological process in the airway and caused a fatal outcome during an episode of asphyxia.

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.

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.