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Table of Contents
Year : 2020  |  Volume : 17  |  Issue : 2  |  Page : 117-119

Rhinoscleroma in a pediatric patient

1 Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India
2 Department of Oral Pathology and Microbiology, IDS, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India
3 Department of ENT, Apollo Hospital, Bhubaneswar, Odisha, India

Date of Submission16-Jan-2020
Date of Acceptance25-Apr-2020
Date of Web Publication18-Jun-2020

Correspondence Address:
Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_3_20

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Rhinoscleroma (RS) is a chronic granulomatous disease caused by Klebsiella rhinoscleromatis. The nose is affected in all cases but sometimes extend to other parts of the airways. Diagnosis of this disease is done on the basis of the histopathological examination (HPE) or direct evidence of the bacteria in nasal exudates. The differential diagnoses of the RS are syphilis, malignancy, and midline granuloma. Streptomycin is a drug of choice although fluoroquinolones and cephalosporins also provide good results. Surgery is opted in case of obstruction or deformity. Here, we report a case of an 11-year-old girl diagnosed as RS on the basis of the HPE. The patient was treated by endoscopic excision of the nasal mass followed a course of ciprofloxacin. She was remained asymptomatic at the last visit of 1 year after treatment and has no evidence of recurrence.

Keywords: Mikulicz cells, nasal cavity, pediatric patient, rhinoscleroma, Russell bodies

How to cite this article:
Swain SK, Debta P, Pradhan S. Rhinoscleroma in a pediatric patient. Apollo Med 2020;17:117-9

How to cite this URL:
Swain SK, Debta P, Pradhan S. Rhinoscleroma in a pediatric patient. Apollo Med [serial online] 2020 [cited 2020 Jul 11];17:117-9. Available from: http://www.apollomedicine.org/text.asp?2020/17/2/117/287089

  Introduction Top

Rhinoscleroma (RS) is an uncommon granulomatous disease often found in the respiratory tract mucosa and caused by the Klebsiella rhinoscleromatis, a rod-shaped Gram-negative bacterium.[1] The cases of RS have been reported from several countries, such as tropical Africa, Middle East, India, Southeast Asia, South and Central America; however, in recent years, some cases are also detected in nonendemic areas because of increased migration of the population from different parts of the world.[2] After nasal cavity, the other common sites affected by RS are nasopharynx (18%–43%), paranasal sinuses (22%), and larynx (15%–40%).[3] RS is common in rural areas where socioeconomic conditions are very poor. This disease is facilitated in crowding area, poor nutrition, and poor hygiene. It is more in females than in males (13:1) and tends to found in the second and third decades of life.[4] It is thought that iron deficiency may lead to disease acquisition.[4] Here, we report a case of RS in an 11-year-old child.

  Case Report Top

An 11-year-old girl attended the outpatient department of otorhinolaryngology with complaints of rhinorrhea, nasal bleeding, and nasal block in the right nostril for the past 3 months. Anterior rhinoscopy showed a friable mass in the right nostril. Examinations of the throat and ear were within normal limits. Diagnostic nasal endoscopy demonstrated the same appearance of the nasal mass, with the presence of granulomatous lesions inside the nasal cavity attaching to the floor of the nose [Figure 1]. Routine blood tests and serological tests for HIV were within normal limits. Chest X-ray was normal. Computed tomography (CT) scan revealed opacity/mass in the right nostril [Figure 2]. There was no evidence of bony erosion in the nasal wall. Biopsy was taken from the right nasal cavity and sent for histopathological examination (HPE). HPE revealed extensive inflammatory infiltrates along with lymphoplasmacytic and histiocytic predominance. The plasma cells showed Russell bodies [Figure 3]. Intrahistiocytic bacilli correspond to K. rhinoscleromatis which was also recognized. After confirmation of the diagnosis, the treatment was done with ciprofloxacin (250 mg twice daily) for 3 months with satisfactory follow-up. The endoscopic examination revealed no evidence of recurrence after 1 year of treatment.
Figure 1: Diagnostic nasal endoscopy revealing granulomatous mass in the right nasal cavity

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Figure 2: Computed tomography scan of the paranasal sinus showing radiopaque soft tissue mass in the right side nasal cavity

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Figure 3: Histopathological picture showing Mikulicz cells which are large vacuolated foamy cells with small nuclei and Russell bodies which are bright red degenerated plasma cells with background of several plasma cells (H and E, ×400)

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  Discussion Top

RS is a chronic granulomatous disease caused by K. rhinoscleromatis, an encapsulated Gram-negative bacillus which belongs to the family of Enterobacteriaceae. It forms spores in anaerobic condition which can excrete exotoxin that leads to scleroma formation in the absence of the presence of bacilli.[5] It is often associated with poor socioeconomic condition, malnutrition, poor hygiene, HIV infection, and overcrowding. RS is common among females and often affects the middle-aged people. Bilateral nasal cavities are usually affected. In our case, the lesion is confined to one side nasal cavity. Sometimes, it affects the nasopharynx and larynx. Involvement of the larynx may lead to severe stridor. Pediatric patients are rarely affected; however, very few cases are reported in the medical literature. In this patient, the cellular immunity may be impaired; however, this patient was immunocompetent.

There are three stages of the disease: catarrhal, granulomatous, and fibrotic stages. Thefirst stage is nasal type where the examination and biopsy may not be specific. In the granulomatous stage of RS, granulomatous lesions are seen which can block the nostrils. The biopsy is diagnostic in granulomatous stage.[6] This stage may be associated with extranasal extension where the laryngeal involvement is the most common (60%–80%) and may be associated with stridor.[7] In the fibrotic stage, there is extensive fibrosis which leads to obstruction rather than exophytic masses. The diagnosis of the fibrotic stage is difficult as the organism is usually not visible.

The clinical symptoms of the patients with RS are nonspecific and often present with mucopurulent nasal discharge, cough, breathing difficulty due to nasal block, dyspnea, nasal bleeding, and headache.[8] It is a slowly progressive disease and characterized by periods of remission and relapse. Patients often require medical advice only when the obstructive granulomatous stage leads to obstruction at the nasal cavity.

The investigations of RS include diagnostic nasal endoscopy, imaging, and HPE. Diagnostic nasal endoscopy helps assess the lesion in the nasal cavity and its extension to other parts of the upper airway. CT scan of the nose and paranasal sinuses reveals the lesions in the nasal cavity and paranasal sinuses. HPE confirms the diagnosis. One study documented RS in children where the lesions appear as amyloid-like protein in the HPE, which may be due to an autoimmune reaction.[9] One study revealed RS in three siblings staining at the nonendemic area and thought to be due to neutropenia acting as a predisposing factor.[5] The differential diagnosis of the RS includes tuberculosis, actinomycosis, leprosy syphilis, histoplasmosis, paracoccidioidomycosis, sporotrichosis, and parasitic infections, such as mucocutaneous leishmaniasis.[10] The RS should also be differentiated from Wegener's granulomatosis, carcinoma, and lymphomas. The specific diagnosis of the RS is based on the HPE and rod-shaped bacillus which is positive to periodic acid–Schiff and Warthin–Starry stain. Immunoperoxidase staining with anticapsular antisera is also useful for the identification of causative bacteria. Bacterial culture with MacConkey agar or blood is also helpful for the identification of the bacilli in approximately 50% of the cases.[11] The histological picture of RS is characterized by the presence of Mikulicz cells, inflammatory cells consisting of multiple plasma cells along with occasional eosinophils and Russell bodies. These Russell bodies are thought to be originated from plasma cells and liberated after disintegration of the cells. The Mikulicz cells are clear cytoplasm vacuolated histiocytes possessing the bacillus. Factors responsible for transforming the histiocytes into Mikulicz cells are unknown.[12] Untreated patients of RS often progress to involve other parts of the respiratory tract. If trachea is involved, the disease may lead to the progressive airway obstruction, which is a life-threatening complication.[13] The sclerotic stage of the RS shows extensive fibrosis, which may cause stenosis and disfigurement. Biopsy from fibrotic stage is often nonspecific. In our case, the patient was in granulomatous stage as multiple numbers of Mikulicz cells were found. Immunoperoxidase monolayer assay method against capsular antigen of K. rhinoscleromatis is very specific and reliable for the diagnosis of the RS. The differential diagnosis is usually other granulomatous diseases of the noses, nasal polyps, and sarcoidosis. Presence of extranasal sites must be carefully investigated by endoscopic examination or radiological investigations to progression of the advanced forms, which may need long-term treatment.[3]

The drug of choice is streptomycin although good results have been seen with cephalosporins, ciprofloxacin, and fluoroquinolones. Ciprofloxacin has good antibacterial spectrum and excellent tissue penetration with minimal side effects. Surgery is useful in severe cases of obstruction or nasal deformity. The treatment period is around 3–6 months which targets intracellular bacteria. Currently, the protocol comprises the antibiotic fluoroquinolone therapy with demonstrable efficacy, but the clinical and endoscopic examination must be necessary until negative biopsies for eradication of the disease.[13] Surgery is often reserved for the disease, particularly orolaryngotracheal forms where stenosis is detected.[13] These may require endoscopic laser or debridement of the stenotic part of the airway or may need open surgery preceded by tracheostomy.[14] 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.

  Conclusion Top

RS is considered as a differential diagnosis of the nasal masses in the pediatric patient. Surgical excision cannot eradicate the disease and may recur in the fibrotic stage. The clinical presentations are usually nonspecific, and granulomatous stage of this disease often evokes the possibility of the RS. HPE confirms the diagnosis where Mikulicz cells are pathognomonic for K. rhinoscleromatis. The diagnosis of the RS is often difficult and delayed because of its clinical polymorphism. However, early diagnosis and prolonged treatment are important criteria for avoiding recurrence and late squeal.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hart CA, Rao SK. Rhinoscleroma. J Med Microbiol 2000;49:395-6.  Back to cited text no. 1
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. 2
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). Europ Ann Otorhinolaryngol Head Neck Dis 2011;128:7-10.  Back to cited text no. 3
Abdel-Aziz M. Rhinoscleroma in a child. Int J Pediatric Otorhinolaryngol Extra 2009;4:72-4.  Back to cited text no. 4
Botelho-Nevers E, Gouriet F, Lepidi H, Couvret A, Amphoux B, Dessi P, et al. Chronic nasal infection caused by Klebsiella rhinoscleromatis or Klebsiella ozaenae: Two forgotten infectious diseases. Int J Infect Dis 2007;11:423-9.  Back to cited text no. 5
Dharan M, Nactigal D, Rosen G. Intraoperative demonstration of Mikulicz cells in nasal scleroma. A case report. Acta Cytol 1993;37:732-4.  Back to cited text no. 6
Ramachandran P, Piquette C, Poole JA. Rhinoscleroma in a young adult with chronic rhinitis and dyspnea. Ann Allergy Asthma Immunol 2008;101:335-6.  Back to cited text no. 7
Abalkhail A, Satti MB, Uthman MA, Al Hilli F, Darwish A, Satir A. Rhinoscleroma: A clinicopathological study from the Gulf region. Singapore Med J 2007;48:148-51.  Back to cited text no. 8
Karchev T, Kabakchiev P. Amyloid-like protein in children with rhinoscleroma. Rhinology 1989;27:27-36.  Back to cited text no. 9
Fuchs HA, Tanner SB. Granulomatous disorders of the nose and paranasal sinuses. Curr Opin Otolaryngol Head Neck Surg 2009;17:23-7.  Back to cited text no. 10
Meyer PR, Shum TK, Becker TS, Taylor CR. Scleroma (Rhinoscleroma). A histologic immunohistochemical study with bacteriologic correlates. Arch Pathol Lab Med 1983;107:377-83.  Back to cited text no. 11
Kim NR, Han J, Kwon TY. Nasal rhinoscleroma in a nonendemic area: A case report. J Korean Med Sci 2003;18:455-8.  Back to cited text no. 12
Yigla M, Ben-Izhak O, Oren I, Hashman N, Lejbkowicz F. Laryngotracheobronchial involvement in a patient with nonendemic rhinoscleroma. Chest 2000;117:1795-8.  Back to cited text no. 13
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. 14


  [Figure 1], [Figure 2], [Figure 3]


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