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.

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.

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.

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.

Retrospective study of the rhinoscleroma about 14 cases in ENT departments of university hospitals (Côte d'Ivoire).

PURPOSE OF STUDY: To report the epidemiological, clinical, therapeutic and evolutionary aspects of rhinoscleroma in ENT departments of university hospitals (Côte d'Ivoire). MATERIAL AND METHOD: Retrospective study of rhinoscleroma conducted in the ENT and head and neck surgery departments in Côte d'Ivoire from January 1980 to December 2008 including the cases of confirmed rhinoscleroma and the treated cases. RESULTS: Fourteen cases of rhinoscleroma were found in 28 years. The early manifestations were not specific enough so the patients were seen with clinical status with obvious disorders or physical discomfort. Treatment was medical and surgical. Medical therapy was based on streptomycin, thiopenicol or ciprofloxacin administration. Surgery consisted in removing the fibrous adhesions to correct the functional and aesthetic disorders. CONCLUSION: Rhinoscleroma has become a more and more rare disease because of the sensitivity to the new molecules. Diagnosis can be difficult and delayed because of its clinical polymorphism.

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.

Clinicopathological study of non-neoplastic lesions of nasal cavity and paranasal sinuses.

An analysis of cases presenting as mass in nasal cavity (NC), paranasal sinuses (PNS), and nasopharynx (NP) was done over a period of 7 years in Jawaharlal Nehru Medical College, Aligarh. A provisional diagnosis was made after clinical assessment and radiological investigations, but final diagnosis was made after histopathological examination. The incidence of masses in NC, PNS, and NP was 34.3 cases per year, non-neoplastic lesions constituted 60% of these cases and their incidence was 20.7 cases per year. All the cases were carefully examined histopathologically and it was found that the region was affected by a variety of non-neoplastic lesions. Among 240 cases, 145 were non-neoplastic and 95 were neoplastic The lesions in the decreasing order of frequency were - nasal polyp, rhinoscleroma, tuberculosis, fungal infection, fibrous dysplasia, ossifying fibroma, cysts, nasal glioma, and cemento-ossifying fibroma. NP was involved by a range of neoplastic lesions; however, no non-neoplastic lesion was seen in this region.

[Dermatology in the tropics]. / Dermatologia en el tropico.

The Author reviews the cases diagnosed in the Republic of Panama of Mycetoma, Paracoccidioidosis, Lobo's disease, Chromomycosis, Histoplasmosis, Rhinosporidiosis, Sporotrichosis, Lepra, Rhinoscleroma, and cutaneous and mucocutaneous Leishmaniasis, and mentions the observed clinical manifestations in order to familiarize young physicians with the tropical dermatopathology which occurs in the rural areas of the country.

Dermatologia en el tropico / Dermatology in the tropics

The Author reviews the cases diagnosed in the Republic of Panama of Mycetoma, Paracoccidioidosis, Lobo's disease, Chromomycosis, Histoplasmosis, Rhinosporidiosis, Sporotrichosis, Lepra, Rhinoscleroma, and cutaneous and mucocutaneous Leishmaniasis, and mentions the observed clinical manifestations in order to familiarize young physicians with the tropical dermatopathology which occurs in the rural areas of the country

Rhinoscleroma: a growing concern in the United States? Mayo Clinic experience.

Rhinoscleroma is a chronic, progressive, granulomatous infection of the upper airways caused by the bacterium Klebsiella rhinoscleromatis. Although most cases occur in developing countries, recent immigration patterns have led to an increasing number of patients with rhinoscleroma in the United States. Rhinoscleroma may mimic various inflammatory and neoplastic processes, including leprosy, paracoccidioidomycosis, sarcoidosis, basal cell carcinoma, and Wegener's granulomatosis. Current therapy consists of a combination of surgical débridement and prolonged antimicrobial therapy. Rhinoscleroma should be added to the list of opportunistic infections that can occur in patients with human immunodeficiency virus.

Scleroma in Uganda: a review of 85 cases.

Biopsy and autopsy protocols revealed that between 1972 and 1981, 85 cases of scleroma were diagnosed in Uganda. The disease was found to affect females more than males, and was found to be more common in the middle aged. Scleroma occurred in the nose in 96% of cases and presented commonly at a late stage. The disease was found commonly among the tribes of south western Uganda and Itesot in the east. Possible factors associated with the distribution of this disease in Uganda are discussed.

[Scleroma and rhinoscleroma]. / Sclérome et rhinosclérome.

Scleroma is a specific granulomatous disease of bacterial origin, chronic evolution, with election in respiratory tracts; nose lesion is practically constant and so justifies the term Rhinoscleroma. Although they are many endemic foci throughout the world and in particular in Africa, it is an uncommon disease, often not recognized for polymorphic. The cases recorded out of already known foci concerned generally migrants; autochtonous cases in France are really infrequent. Within the evolutive lesions diagnosis is made out of the isolation of the responsible germ (Klebsiella rhinoscleromatis) and the presence of the specific Mikulicz' cell. To find out localizations in the same patient, we utilize X Rays and endoscopy. Antibiotic therapy is of long duration but treats the affection and prepares the way of surgery intervention to eliminate cicatricial deformities. Relapses are caused by shortness of therapy and some difficulty to monitor patients. Mortel in the past, scleroma should nowadays, be better recognized to be better treated and cured.