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Rhinoscleroma

AuthorsVíctor Martins Isac1 Cássio Lemos Jovem2 Fabrício Guimarães Gonçalves3

Ramon Edgardo Figueroa4

1Department of Radiology – Brasilia University Hospital, Brasilia, DF, Brazil2Medical Doctor – Department of Radiology, Brasilia University Hospital, Brasilia, DF, Brazil3Department of Radiology, Brasilia University Hospital - Brasilia Institute of Diagnostic Imaging, Brasilia, DF, Brazil4Department of Radiology, Medical College of Georgia, GA, USA

Submitted on: 05/09/2009 Approved on: 03/12/2009

Correspondence to:

Fabrício Guimarães Gonçalves Hospital Universitário de Brasília- Departamento de RadiologiaSGAN 604/605 – Asa Norte Brasíla –DF –Brasil CEP. 70840-901Tel : 55 61 92183563 –FAX 55 61 3448 5388 E-mail: goncalves.neuroradio@gmail.com

The authors declare no confl ict of interest.

ABSTRACT

Rhinoscleroma is a chronic, infectious, granulomatous disease that can affect the lining mucosa of the entire respiratory tract. There are three stages of the disease: catarrhal/atrophic, hypertrophic/granulomatous, and the fi nal sclerotic phase. The diagnosis is possible by the histological fi ndings of Mikulicz cells and plasma cells degenerated into Russell bodies and by the fi nding of Klebsiella rhi-noscleromatis in the tissue biopsy. Treatment can be performed by specifi c antibiotics and by surgery in some situations. Imaging fi ndings are non-specifi c, but are helpful in staging and for treatment follow-up. We present a case report with Mikulicz cells, plasma cells degenerated into Russell bodies, and endoplasmic bacilli present in the biopsy material.

Keywords: rhinoscleroma, radiology, computed tomography.

[Braz J Infect Dis 2010; 14(1):1-22]©Elsevier Editora Ltda.

INTRODUCTION

Rhinoscleroma (scleroma) is a chronic granu-lomatous infection of slowly progressive course caused by Klebsiella rhinoscleromatis,1-6 which primarily affects the lining mucosa of the entire respiratory tract. The nose is affected in almost all the cases.2,4,6

Rhinoscleroma has a sporadic worldwide distribution, being considered endemic in situ-ations of poor hygiene, malnutrition, and in crowded environment, especially in regions of Central America, Africa, Middle East, China and India, and in rural areas of South America.1,2,4,5 Occasionally it is described in non-endemic re-gions as well.4

The disease has three stages: catarrhal/atrophic, with signs and symptoms resem-bling a common cold, with rhinorrhea (which can be fetid), nasal obstruction and crust for-mation in the affected mucosa; hypertrophic/granulomatous, with extensive granuloma-tous changes; and the fi nal sclerotic stage, with the tissue at the tip of the nose becom-ing infi ltrated, fi rm, and nodular.5,6 Histopa-thology plays an important role in diagnosing this entity. Treatment can be performed with antibiotics, while surgery is reserved for some cases of airway obstruction.

The authors report a case of rhinoscleroma in a young woman with nasal obstruction ac-companied by respiratory discomfort. The diagnosis was confi rmed by the presence of Mikulicz cells, plasma cells degenerated into Russell bodies, and endoplasmic bacilli in the biopsy material.

CASE REPORT

A 26-year-old female patient assisted at the Otorhinolaryngology Department of the Uni-versity Hospital of Brasília, presenting with a three-year history of bilateral nasal obstruc-tion. On physical examination, there was a granulomatous lesion obstructing the nasal vestibules, which extended forwards (Figure 1) with important bulging infi ltration of the nasal pyramid.

A multi-detector computed tomography (CT) of the paranasal sinuses showed a well-defi ned, widespread and homogeneous soft tissue lesion located in the anterior portion of the nasal fossae, infi ltrating the nasal pyramid and obstructing the nasal vestibules. There was mild enhancement and slight erosion of the na-sal process of the maxilla and of the foremost cartilaginous component of the nasal septum. At the same time, there was minimal bilateral

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Isac, Jovem, Gonçalves et al.

maxillary sinus mucosal thickening and a cystic lesion re-lated to one of the roots of the tooth number 12, consist-ent with a periradicular cyst (Figure 2). The fi nding of such cystic lesion in the present case could represent a radiologi-cal indication of low level of oral hygiene, since these lesions are associated with chronic dental infl ammatory processes leading to pulp necrosis. CT of the neck, performed from the base of the skull to the level of the carina, did not show additional similar-looking lesions at any other regions of the respiratory tract or enlarged cervical lymph nodes.

The lesion biopsy revealed vacuolated and distended his-tiocytes diffusely infi ltrating the submucosa, with round and

eccentrically located nuclei (Mikulicz cells), lymphocytic in-fl ammatory infi ltrate, some contained Russell bodies and mild fi brosis (Figure 3). The Mikulicz cells were positive for CD68 and negative for pan cytokeratin AE1/AE3, demon-strating the histiocytic nature of the injury. The Giemsa, Pe-riodic Acid Schiff, and Warthin-Starry stains demonstrated the presence of endoplasmic bacilli.

After the diagnosis of rhinoscleroma was established, the patient was treated with antibiotics with complete remis-sion, resulting in minimal residual nasal stenosis requiring no surgery due to signifi cant clinical improvement.

Figure 3: A/B/C.

Figure 2: A/B/C.

Figure 1:

Braz J Infect Dis 2010; 14(1):1-22-22112

Rhinoscleroma

DISCUSSION

Rhinoscleroma is caused by Klebsiella rhinoscleromatis, (En-terobacteriaceae group), an intracellular, gram-negative, non-motile, encapsulated, facultative and glucose-fermenting diplo-bacillus which causes diseases in humans.2-4

It is a chronic specifi c granulomatous infection, of indolent and progressive course, that predominantly affects the upper airway tract, affecting the nose in 95% to 100% of the cases. It affl icts young individuals (2nd to 3rd decades of life), without predilection for race, with a slight predominance in females and people of low socioeconomic status and of poor hygiene.5,6

Ferdinand von Hebra, an Austrian Dermatologyst, fi rst de-scribed the disease in 1870, initially described as a sarcoma.7 In 1876, Jan Mikulicz-Radecka described the histological features and the vacuolated cell, which was named after him.8 In 1882, Anton von Frisch discovered the causative agent.9 The fi rst case published in Brazil dates from 1890 by Adolpho Lutz.10

The disease is clinically and pathologically divided into three stages. First stage, catarrhal/atrophic in which the patient has nonspecifi c symptoms of a common cold or rhinitis, with persistent rhinorrhea, the lining mucosa may be atrophic. There is evidence of squamous metaplasia, subepithelial infi ltration of neutrophils and some granulation tissue on histology. In the second phase (hypertrophic/granulomatous), the patient may present with epistaxis, nasal deformity from enlargement of the nasal pyramid and erosion of the septal cartilage and/or the nasal bone. Histologically, there may be atrophy or hyper-plasia, the last being more common, known as epitheliomatous pseudo-hyperplasia, with infi ltrates of chronic infl ammatory cells, monocytes, lymphocytes, and histiocytes.6,11 The Mikulicz cells are histiocytes with numerous large vacuoles containing bacteria that may or may not be viable and can be found below the basal layer.2,5 The Russell bodies are eosinophilic structures within the cytoplasm of plasma cells.2,4,5 The fi nal or sclerotic phase is characterized by progression of stenosis and deformity of the nasal vestibule, with granulomatous areas surrounded by fi brotic tissue. At this stage, the Mikulicz cells and Russell bod-ies are hardly seen.12

The differential diagnosis of scleroma is extensive and may include other granulomatous lesions, fungal infections, and ne-oplasms, including tuberculosis, histoplasmosis, blastomycosis, Wegener’s granulomatosis, lethal mid-line granuloma, actino-mycosis, paracoccidioidomycosis, tertiary syphilis, leishmania-sis, vasculitis, lymphoma, basal cell carcinoma, and verrucous squamous cell carcinoma.2,4,11,14

By our knowledge there are a few large series of reports es-tablishing an imaging pattern based on CT scans. On the other hand, reports of single cases are more numerous, in which the CT scan shows mass unilateral or bilateral, with homogeneous density, specifi c limits, with standard variable-enhanced and destruction of bone and nasal septum.

Razek described the signal behavior of the scleroma on magnetic resonance imaging through an analysis of 15 patients.

By his series, in T1 weighted images, the lesions were slightly hyperintense related to the muscles and to the cerebrospinal fl uid, but less hyperintense than fat. In T2 weighted images, the lesions were homogeneously hyperintense varying to heteroge-neously hyperintese with hypointense foci.6

Diagnosing rhinoscleroma is challenging and it is not al-ways the fi rst diagnostic hypothesis by the time of clinical examination or by imaging studies. In the clinical setting of a young patient presenting with a long standing infi ltrating le-sion in nasal pyramid, involving and obstructing one or both nasal vestibules, with a personal positive history of poor hy-giene and coming from an endemic area, would lead to a sus-picious candidate for the disease, requiring for this reason the proper work out.

The computed tomography pattern observed in the present case was in agreement with current literature. These imaging fi ndings, although non-specifi c, are useful in determining the extent of the disease and in evaluating the therapeutic response. From the clinician standpoint, facing the suspicion of rhino-scleroma with signs, symptoms, and positive epidemiological data, the accurate diagnosis can be certainly made by fi nding the bacteria itself (which is not always possible), or by fi nding the specifi c histiocytes through specimen biopsy. The majority of the cases will exhibit a favorable response after a full course of specifi c antibiotic treatment.

ACKNOWLEDGEMENTS

Márcia Cabral A. T. de CabralPushikin Pires LealTânia Mara C. Rossi.

REFERENCES

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