Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 313

Ahead of print publication  

Rhinoscleroma: Our experiences at a tertiary care teaching hospital of eastern India

1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar, Odisha, India
2 Department of Oral Pathology and Microbiology, IDS, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar, Odisha, India
3 Division of Microbiology and Toxicology, ICMR-National Institute of Occupational Health, Ahmedabad, Gujarat, India

Date of Submission14-Jan-2020
Date of Decision01-Mar-2020
Date of Acceptance24-Jun-2020

Correspondence Address:
Santosh Kumar Swain,
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University (Deemed to be), Bhubaneswar-751003, Odisha
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_15_20


Background: Rhinoscleroma (RS) is a chronic granulomatous disease due to infection of Klebsiella rhinoscleromatis. It often affects the respiratory mucosa, especially the nasal cavity and sometimes extends lower respiratory tract. RS is more common in certain geographical area than in others, but the pathogenesis and the risk factors of RS are still unclear. Materials and Methods: This was a retrospective study done between January 2014 and December 2019. The diagnosis of the RS was done on the basis of clinical presentations and histopathological findings. Details of clinical findings, investigations, and treatment were analyzed. Results: There were 13 cases of RS diagnosed after confirmation with histopathological examination. The female to male ratio was 1.6:1 with a mean age of 36 years. The most common clinical presentation was nasal obstruction. All were treated with ciprofloxacin. Relapse occurred in three cases which were confirmed by biopsy. Conclusion: RS is a rare clinical entity in Eastern India. Awareness of the clinical presentations of RS and early diagnosis will reduce the morbidity of this disease.

Keywords: Klebsiella rhinoscleromatis, nose, rhinoscleroma, upper respiratory tract

How to cite this URL:
Swain SK, Debta P, Sahu MC. Rhinoscleroma: Our experiences at a tertiary care teaching hospital of eastern India. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2021 Dec 6]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=321275

  Introduction Top

Rhinoscleroma (RS) is a chronic granulomatous infection of the nose and other parts of the upper respiratory tract. This infection is caused by a capsulate Gram-negative bacterium, Klebsiella rhinoscleromatis, which was first documented by Von Frisch in 1882.[1] RS is endemic Central and South America, Africa, South central and Eastern Europe, Middle East, and China.[2] It is commonly found in rural areas where socioeconomic condition is poor. The aggravating factors for RS are poor hygiene, crowding, and poor nutrition. Women are more commonly affected than men in the ratio of 13:1 and more in the second and third decades of life. Iron deficiency may predispose to the acquisition of RS.[3] RS is transmitted by air and the human beings are the only identified host. It usually affects the nasal cavity and nasopharynx but it sometimes affects the larynx, trachea, bronchi, paranasal sinuses, oral cavity, lips, orbit, and middle ear. The lack of the awareness of the RS in developing countries like India may lead to delay in diagnosis which causes deformity of the nose and airway obstruction. Here, our study is to assess the details of clinical presentations, diagnosis and treatment of the RS at a tertiary care teaching hospital of eastern India.

  Materials and Methods Top

This is a retrospective study conducted at a tertiary care teaching hospital of eastern India during January 2014 to December 2019. This study was approved by the Institutional Ethics Committee (IEC) with reference number IEC/IMS/SOAU/123/2013. All the patients of RS treated during this period were included in this study. No patients of this study were suffered from any systemic diseases such as diabetes mellitus, tuberculosis, leprosy and syphilis. Patients with clinical presentations (e.g., nasal obstruction, epistaxis, rhinorrhea and stridor), radiological features (e.g., homogenous masses in the nasal cavity or paranasal sinuses) and typical histopathological pictures including Mikulicz cells were included in this study. Inclusion of the patients in this study was based on the interpretation of the senior authors (otolaryngologists), radiologists and pathologists those were responsible for patients. We retrospectively analyzed the files of all the patients who had received a diagnosis of RS. Details of clinical findings, investigations and treatment were documented. Clinical parameters included in this study were age, gender, duration, diagnostic nasal endoscopic findings, radiological findings, histopathological pictures and treatment options with its outcome. The recurrence of the disease at follow up period was assessed. The diagnosis of the RS was based on the histopathological confirmation and bacteriological culture. We retained 13 patients with hospital records diagnosed as RS out of the 68275 consultations over this period of time which is 0.01% of the patients. The diagnosis of the RS was confirmed with histopathological examinations showing Mikulicz cells and Russel bodies [Figure 1]. All the patients of RS were treated with ciprofloxacin 500 mg one tablet twice daily. Surgical treatment was needed in patients with obstructive pathology. The prognosis of the disease was assessed by using endoscopic examination and imaging.
Figure 1: Photomicrograph shows Mikulicz cells which are large vaculated foamy cells with small nuclei and Russel bodies which are bright red degenerated plasma cells with background of several plasma cells (Hemotoxylin and Eosin, ×400)

Click here to view

  Results Top

In 5-year period of this study, 13 cases of RS were diagnosed which indicates rarity of this disease. There were 8 females (61.53%) and 5 males (38.46%) with age range from 14 years to 73 years. The mean age of the participating patients of this study was 36 years. Out of 13 patients, 7 (53.84%) were below poverty line card holders. Out of the 13 patients, seven patients (53.84%) were identified as the lesion confined to the one side nasal cavity, three patients (23.07%) with both sides nasal cavities. Four patients (30.76%) showed lesions confined to the nasal cavity and also at nasopharynx. Two patients (15.38%) showed involvement of the larynx. The diagnosis of the RS was done with help of histopathological examination showing Mikulicz cells and Russel bodies. Out of the 13 patients, 11 patients were presenting with nasal obstruction, two cases presenting dysphonia and four patients presenting with mouth breathing [Table 1]. Out of 13 patients with RS, two cases presented with epistaxis (15.38%). In 11 cases with nasal obstruction, 5 cases (38.46%) showed nasal septal involvement with the lesions. Four cases showed floor of the nasal cavity involvement along with nasopharyngeal involvement (30.76%). Two cases showed nasal cavity involvement along with oropharyngeal lesions (15.38%). Larynx was involved in two cases (15.38%). One case of laryngeal scleroma showed subglottic stenosis [Figure 2]. Another case of laryngeal scleroma showed an ulcer at the subglottic region. Physical examination of the nose and paranasal sinuses showed saddle nose [Figure 3] in 2 cases and 3 cases showed involvement of the pinched nose. Out of 13 cases, 2 cases showed involvement of the paranasal sinuses in imaging. Laryngeal endoscopy revealed involvement of two cases where as spreading of scleroma to the laryngeal lumen in one case. Computed tomography (CT) scan showed soft tissue mass in the nasal cavity in 11 cases with deformity of the nasal cavity in 2 cases due to bony erosion of the nose. All were treated with medical therapy such as antibiotics and corticosteroids. Ciprofloxacin was given for 3 months to all patients. Endoscopic surgical excision was done in 5 cases for removal of the lesions from the nasal cavity along with making patent nasal cavity. One laryngeal involvement case underwent tracheostomy for subglottic stenosis. All the patients were reviewed at the 6 months, 1 year and 2 years. Out of the 13 cases, three patients showed recurrence at the follow up of 1 year. The recurrence was confirmed by biopsy.
Table 1: Clinical profile of the rhinoscleroma patients

Click here to view
Figure 2: Patient of rhinoscleroma presenting with subglottic stenosis

Click here to view
Figure 3: Patient of rhinoscleroma presenting with saddle nose

Click here to view

  Discussion Top

RS is a chronic, insidious and infectious disease of the nose and other parts of the upper respiratory tract caused by K. rhinoscleromatis. The term “RS” was first coined by Von Hebra in 1870, who also described the nasal pathology of scleroma.[4] Mikulicz described the histopathological features of this disease in 1877 and he documented the disease in details and establishes its inflammatory and nonneoplastic nature.[5] RS is pathologically and clinically classified into 3 stages such as catarrhal (or atrophic) stage, granulomatous (or proliferative) stage and sclerotic (or fibrotic stage).[6] In catarrhal/atrophic stage, histological picture shows squamous metaplasia and nonspecific sub-epithelial infiltrations of polymorphonuclear white blood cells with granulation tissue. The diagnostic features are seen in the granulomatous stage of the RS where histological presentations include sub-epithelial presence of Mikulicz cells and large vaculated macrophages possessing rod shaped bacilli.[7] In sclerotic stage, patients usually show extensive fibrosis, which may cause stenosis and disfigurement. In fibrotic stage, the biopsy may be quite nonspecific. This infection is usually transmitted via airborne secretions. However, normal persons in contact with a RS patient for several years do not necessarily affected with such infection. The cellular immunity may be affected in RS patients; however, our patients in this study were immunocompetent.

RS can affect any parts of the upper respiratory tract. It often begins in the sub-epithelium of the nasal mucosa and spreads to other parts such as subepithelium of the pharynx which is affected in approximately 50% of the cases.[8] The clinical presentations of the RS are nonspecific such as rhinorrhea, nasal obstruction, epistaxis, cough and mouth breathing. In this study, the most common symptom was nasal obstruction. Anterior rhinoscopic examination shows friable mass which may bleed on touch. The most common symptom of RS is nasal obstruction followed by nasal deformity. After the nasal involvement, palate is involved.[9] The soft palate is more affected than hard palate. When the hard palate is affected, there is destruction of bone due to progressive nodular infiltrates. The nodular infiltration into soft palate may extend to the oropharynx and tonsillar fossa. Scarring of the soft palate leads to forward tilting of the uvula and fibrous stenosis. When the uvula is involved, a groove is formed at its base. The depth of this groove depends on the stage of the disease.[9] Anesthesia of the soft palate is important diagnostic finding in RS. Another clinical finding is palatal sign where V-shaped deformity of the soft palate which is found in lateral radiograph of the postnasal space. The palatal sign is a diagnostic feature in radiological picture in RS. Additional feature is ulcers at the base of the tongue and marked lymphadenopathy.[10] Larynx is sometimes involved by RS. Subglottis is the most common region to be affected by RS.[11] In this study, two cases were affected where one case revealed subglottic stenosis whereas another case showed an ulcer the subglottic region. It is thought that the lesion arises at the junction between the squamous epithelium of the vocal folds and the respiratory epithelium of the mucosa of the subglottis.[12] The differential diagnosis of RS at early stage is common rhinitis. At tumoral stage, it often mimic to other granulomatous and neoplastic or systemic infectious lesions such as tuberculosis, syphilis, leprosy, actinomycosis, histoplasmosis, blastomycosis, sporotrichosis, paracoccidioidomycosis, mucocutaneous leishmaniasis, lymphomas, sarcoidosis, verrucous carcinoma, and Wegner's granulomatosis.[13] When scar formation process occurs, ozaena, destructive mycosis, and mucocutaneous on scar tissue must be evoked.

The diagnosis of RS needs high index of suspicion, clinical and pathological correlation. The diagnosis of the RS is confirmed by histopathological examination or bacteriological identification from the nasal fluid. In case of nasal involvement, the septum and inferior turbinate are best locations for taking biopsy.[14] Biopsy should be taken from the areas where granuloma is seen because the histological changes are characteristic in this place. Immunoperoxidase monolayer assay technique is highly specific against capsular antigen of K. rhinoscleromatis which is reliable test for diagnosis of RS. K. rhinoscleromatis can be grown in blood agar or MacConky agar. The culture of the bacteria is important as it confirm the diagnosis and also help to select the antibiotic by sensitivities obtained in vitro. This organism can also be seen on routine HE staining and presence of Gram-negative diplobacilli confirm on Gram staining. Special silver staining such as Warthin-Starry and Giemsa are usually needed to show the classic histological picture. Immunohistochemical methods are also available with capsular antigen O2K3 being on the basis for Immunoperoxidase testing.[15] Although radiological tests are not diagnostic or pathognomonic of the RS, magnetic resonance imaging (MRI) can be done because of the nasal masses can block the osteomeatal complex and the secretions may be accumulated inside the paranasal sinuses. CT scans of the nose and paranasal sinuses (coronal Plane) are used performed to assess the nasal cavity and paranasal sinuses. Paranasal sinuses often show soft tissue attenuation material occupies the nasal cavity and sinuses. There is usually no bone destruction. CT scan will assess the site and extent of stenosis in case of laryngeal involvement. In MRI, both T1 and T2 weighted images reveal characteristic mild to marked high signal intensity in hypertrophic stage of the RS.[16] Diagnostic nasal endoscopy shows the extent of the lesions in all stages of the RS and aids for the accurate diagnosis which is based on the histopathological findings and isolation of K. rhinoscleromatis from culture.[17]

The treatment of the RS is often difficult because of the fact that the bacteria can remain dormant in an anaerobic spore form and it only be reactivated months to years later after initial treatment with antibiotics.[18] The treatment of RS includes combination of appropriate antibiotics and surgical debridement done if there is significant airway obstruction. The outcomes of current treatment are not satisfactory and often end in recurrence.[19] However, presently, there are no randomized controlled trials to compare different antibiotic treatment choices and their efficacy.[19] Antibiotics are the treatment of choice in RS. Treatment initially was choice of antibiotic because of low toxicity and acts at an intracellular level and effective against spore formation in anaerobic condition.[20] One study showed the outcome with 3–9 months treatment of antibiotics in RS and antibiotics used were ciprofloxacin, ceftriaxone, tetracycline and clofazimine where relapse occurred in 3 out of the 11 patients. They recommended fluoroquinolones as antibiotic of choice for the RS as it has good activity against Gram-negative bacilli, low toxicity and good intracellular efficacy.[19] Recently one study of three cases documented that treatment with long term antibiotics (3–6 months) with very few side effects (ciprofloxacin and co-trimoxazole) with or without surgical debridement is the mainstay of treatment.[21] One study by Tan and colleagues recommended a treatment regime with combination of ciprofloxacin and doxycycline for minimum six months.[22] Recently quinolones have been shown as adequate treatment. Ciprofloxacin is an antibiotic which has excellent tissue penetration and broad spectrum activity. It has comparatively less adverse effects. Its use is avoided in patients under the age of 12 years of age because of the chance of arthropathy. The advantage of ciprofloxacin is twice daily administration, which may improve patient compliance for longer period of treatment. In this study, ciprofloxacin was given to all patients. Quinolones are concentrated within the macrophage, which is another theoretical advantage of ciprofloxacin.[23] Ciprofloxacin with dose of 250 mg to 500 mg administered twice daily for 4 weeks was shown to have good efficacy in the region of the Mexico where the incidence of scleroma is endemic.[24] The exact duration of the antibiotic treatment is still not be established. Many authors suggested to continue treatment till negative tissue culture or histological examination for bacillus. Long term follow up needed to monitor the reactivation of disease after treatment of RS. In this study, surgical debridement was done in four patients. Prolonged treatment with antibiotics was done to all patients (treatment for 3–9 months). The antibiotics administered systemically were ciprofloxacin (9 patients), third generation cephalosporins (three patients) and tetracycline (one patient). Surgery is often needed for the disease particularly orolaryngotracheal forms where stenosis is found. These may require endoscopic laser or debridement of the stenotic part of the airway or may need open surgery preceded by tracheostomy.[25] Mortality due to RS is extremely rare, but may happen due to upper airway obstruction in case of undiagnosed disease or due to complication of the surgical procedure.

Ten of 13 patients did not experience relapse during the 1 year follow up. Relapse in 3 patients were confirmed by second biopsy. It is also a cheaper drug for long run treatment. The chance of recurrence in RS is still associated with any treatment regimen as the causative organism is often resistant to most of the antibiotics and being intracellular and not exposed to sufficient concentration of antibiotics. As K. rhinoscleromatis is an intracellular bacteria, long term antibiotics would theoretically effective, owing their high concentration inside the macrophages.[26] Treating RS is often challenging to the clinicians due to its rarity and its clinical polymorphism. Delay in diagnosis and treatment leads to local and regional propagation of the RS and lands in morbidity.

  Conclusion Top

RS is a chronic, infectious, slowly progressive disease of the nose and may involve the other parts of the upper airway. It commonly affects female and more in the middle age group. It should be considered as a differential diagnosis of the nasal mass or lesions. Histopathological examinations with abundant plasma cells with Russel bodies and Mikulicz cells, and in their cytoplasm there are Gram-negative bacilli confirm the diagnosis. Surgical excision alone cannot eradicate this disease which may recur particularly in fibrotic stage. It is rare in nonendemic countries but increased travel from endemic region leading to increased incidence of this disease. This clinical condition often mimic to other pathologies and if not treated lead to deformity of the nose and laryngeal stenosis. There is a need for awareness of clinical presentations and treatment options among clinicians.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Von Frisch A. On the etiology of rhinoscleroma. Wien Med Wochenschr 1882;32:969-82.  Back to cited text no. 1
Kim NR, Han J, Kown TY. Nasal rhinoscleroma in a nonendemic area: A case report. J Med Korean Sci 2003;18:455-8.  Back to cited text no. 2
Akhnoukh S, Saad EF. Iron deficiency in atrophic rhinitis and scleroma. Indian J Med Res 1987;85:576-9.  Back to cited text no. 3
Von Hebra F. About a peculiar new creation on the nose: Rhinoscleroma: together with the histological findings from Dr M Kohn. Wien Med Wochenschr 1870;20:1-5.  Back to cited text no. 4
Mikulicz J. About rhinoscleroma (Hebra). Langenbech's Arch Clin 1877;20:485-534.  Back to cited text no. 5
Hart CA, Rao SK. Rhinoscleroma. J Med Microbiol 2000;49:395-6.  Back to cited text no. 6
Kallel S, Ghorbel AM. Rhinoscleroma: A rare chronic infection of the nasal cavities. Pan Afr Med J 2018;31:247.  Back to cited text no. 7
Canalis RF, Zamboni L. An interpretation of the structural changes responsible for the chronicity of rhinoscleroma. Laryngoscope 2001;111:1020-6.  Back to cited text no. 8
Umphress B, Raparia K. Rhinoscleroma. Arch Pathol Lab Med 2018;142:1533-6.  Back to cited text no. 9
Lenis A, Ruff T, Diaz JA, Ghandour EG. Rhinoscleroma. South Med J 1988;81:1580-2.  Back to cited text no. 10
Sahil M, Hemmaoui B, Errami N, Benariba F. Laryngeal involvement of the rhinoscleroma. Eur Ann Otorhinolaryngol Head Neck Dis 2016;4:293-4.  Back to cited text no. 11
Abou-Seif SG, Baky FA, el-Ebrashy F, Gaafar HA. Scleroma of the upper respiratory passages: A CT study. J Laryngol Otol 1991;105:198-202.  Back to cited text no. 12
de Pontual L, Ovetchkine P, Rodriguez D, Grant A, Puel A, Bustamante J, et al. Rhinoscleroma: A French national retrospective study of epidemiological and clinical features. Clin Infect Dis 2008;47:1396-402.  Back to cited text no. 13
Chan TV, Spiegel JH. Klebsiella rhinoscleromatis of the membranous nasal septum. J Laryngol Otol 2007;121:998-1002.  Back to cited text no. 14
Andraca R, Edson RS, Kern EB. Rhinoscleroma: A growing concern in the United States? Mayo Clinic experience. Mayo Clin Proc 1993;68:1151-7.  Back to cited text no. 15
Razek AA, Elasfour AA. MR appearance of rhinoscleroma. AJNR Am J Neuroradiol 1999;20:575-8.  Back to cited text no. 16
N'gattia KV, Kacouchia N, Koffi-N'guessan L, Mobio NM, Kouassi-Ndjeundo J, Kouassi M, et al. Retrospective study of the rhinoscleroma about 14 cases in ENT departments of university hospitals (Côte d'Ivoire). Eur Ann Otorhinolaryngol Head Neck Dis 2011;128:7-10.  Back to cited text no. 17
Said-Al-Naief N, Edwards P, Carlos R, Sanchez-Romero C, De Almeida OP. Rhinoscleroma. A series of 16 cases. Oral Surg Oral Med Oral Pathol Oral Radiol 2017;124:228-9.  Back to cited text no. 18
Gaafar HA, Gaafar AH, Nour YA. Rhinoscleroma: An updated experience through the last 10 years. Acta Otolaryngol 2011;131:440-6.  Back to cited text no. 19
Holinger PH, Gelman HK, Wolfe CK Jr. Rhinoscleroma of the lower respiratory tract. Laryngoscope 1977;87:1-9.  Back to cited text no. 20
Suchanova PP, Mohyuddin NG, Rodriguez-Waitkus PM, Eicher SA. Rhinoscleroma in an urban nonendemic setting. Otolaryngol Head Neck Surg 2012;147:173-4.  Back to cited text no. 21
Tan SL, Neoh CY, Tan HH. Rhinoscleroma: A case series. Singapore Med J 2012;53:e24-7.  Back to cited text no. 22
Hooper DC, Wolfson JS. Fluoroquinolone antimicrobial agents. N Engl J Med 1991;324:384-94.  Back to cited text no. 23
Borgstein J, Sada E, Cortes R. Ciprofloxacin for rhinoscleroma and ozena. Lancet 1993;342:122.  Back to cited text no. 24
Swain SK, Sahu MC, Mohanty S, Samal R, Baisakh MR. Management of laryngotracheal stenosis-Still remains a challenge for successful outcome. Apollo Med 2016;13:102-7.  Back to cited text no. 25
Maguiña C, Cortez-Escalante J, Osores-Plenge F, Centeno J, Guerra H, Montoya M, et al. Rhinoscleroma: Eight peruvian cases. Rev Inst Med Trop Sao Paulo 2006;48:295-9.  Back to cited text no. 26


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


     Search Pubmed for
    -  Swain SK
    -  Debta P
    -  Sahu MC
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Materials and Me...
   Article Figures
   Article Tables

 Article Access Statistics
    PDF Downloaded11    

Recommend this journal