Indian Journal of Pathology and Microbiology

: 2011  |  Volume : 54  |  Issue : 4  |  Page : 806--808

Cytohistological features of rhinoscleroma

Neelam Sood1, Sanjeev Sood2, Sunaina Arora1, Deepika1,  
1 Department of Pathology and Lab Medicine, Deen Dayal Upadhya Hospital, Hari Nagar, New Delhi, India
2 Consultant ENT, MC Hospital, Janak Puri, New Delhi, India

Correspondence Address:
Neelam Sood
Department of Pathology and Lab Medicine, Deen Dayal Upadhya Hospital, Hari Nagar, New Delhi


Rhinoscleroma, also known as Mikulicz disease, is a chronic progressive disease caused by Klebsiella rhinoscleromatis. The disease is uncommon in Delhi. There are no cytological references of this disease. We describe the cytological features which should alert the pathologist to rule out this entity, which needs to be treated early. Immunohistochemistry on histopathological material is a useful adjunct to the diagnosis.

How to cite this article:
Sood N, Sood S, Arora S, Deepika. Cytohistological features of rhinoscleroma.Indian J Pathol Microbiol 2011;54:806-808

How to cite this URL:
Sood N, Sood S, Arora S, Deepika. Cytohistological features of rhinoscleroma. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 Oct 7 ];54:806-808
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Full Text


Rhinoscleroma is a chronic progressive specific granulomatous infectious disease affecting the upper respiratory tract. [1],[2] It is endemic in to Central Africa, Central and South America, Central and Eastern Europe, Middle East, Parts of India, and Indonesia. [3] Three overlapping clinicopathological stages have been described: Catarrhal-atrophic, granulomatous, and sclerotic. [2],[3] Early treatment is necessary to avoid the spread of disease. [3],[4] There have been a few reports from India, [1],[5],[6] but we have not come across any cytological reference to this entity.

We present a case that was being evaluated for nasal mass and confused with malignancy and subsequently, on thorough clinicopathological evaluation, was diagnosed as rhinoscleroma. The cytological features have also been described.

 Case Report

A 56-year-old healthy female, a resident of Delhi for last 15 years, presented with right-sided nasal obstruction for last 1 month with recurrent history of similar complaint for past 6 months. She also complained of persistent headache, swelling on cheek and eye, and bleeding on blowing nose. Past history of purulent persistent nasal discharge was also elicited. General physical examination was unremarkable. Local examination showed presence of mild right eye proptosis with deviation to right and diffuse cheek swelling. Anterior rhinoscopy showed a firm, polypoidal vascular mass in right nasal cavity arising from the lateral wall, filling anterior naris and pushing septum to the left side, which bled on touch. Posterior rhinoscopy was unremarkable. Hematological and biochemical investigations were within normal limits. Erythrocyte sedimentation rate (ESR) was 25 mm in first hour.

Radiological findings were as follows: Computed tomography (CT) scan showed a right nasal mass with ethmoidal sinus involvement and was impinging the right maxillary sinus [Figure 1]. Patient was referred for fine needle aspiration (FNA) to rule out malignancy. Smears showed presence of foamy macrophages in a background of lymphocytes and plasma cells. These cells had central to eccentric nuclei with indistinct nucleoli [Figure 2]. Stains for acid fast bacilli (AFB), Grocott's silver methenamine, were negative. Gram's stain showed an occasional gram negative bacillus within the macrophage. The histiocytes were periodic acid Schiff (PAS) positive. No evidence of emperipolesis was noted. Acute onchronic inflammatory lesion with suggestion for excision and histological examination to rule out rhinoscleroma/underlying malignancy was signed out.{Figure 1}{Figure 2}

Histopathological examination of multiple firm to friable bits submitted showed pseudoepitheliomatous hyperplasia of the overlying epithelium. Underlying zone was densely infiltrated by chronic inflammatory cells comprising of lymphocytes, plasma cells, and groups of large vacuolated histiocytes with single or multiple cytoplasmic vacuoles and small centrally or peripherally located nuclei (Mikulicz cells). Nuclear chromatin was fine with indistinct nucleoli [Figure 3]. An occasional cell showed intracellular gram negative bacilli. Russell bodies were also noted. An occasional ill-formed noncaseating granuloma comprising of epitheliod cells and lymphocytes were seen. Special stain (PAS) and immunostain CD 68 (mouse monoclonal PG-M1 clone, 1:40 ABC Technique, DBS, Lyon, France) [Figure 4] were helpful in aiding the diagnosis. Microbiological cultures established the exact causative microorganism (Klebsiella rhinoscleromatis).{Figure 3}{Figure 4}

Patient was put on the antibiotic for 1 month (rifampicin 600 mg OD and ciprofolxecin 500 mg BD), but the failure of above regime led to surgical resection of the medial turbinate mass followed by course of antibiotic comprising rifampicin and levofloxacin for 3 months. The patient is asymptomatic 20 months following treatment.


Rhinoscleroma almost always affects the respiratory tract. Nose is the most common site (95-100%) as seen in our case. [2] Bilateral, asymmetrical and other organs including lymph nodes nearby may also be involved. [1],[2] This patient was older (56 years) unlike the reported peak age of presentation of 2 nd and 3 rd decade. [5] Female sex predilection has been reported. [2],[5],[6] This entity is seen in north India as reported by some workers. [2],[5],[6] However, this was the first case seen in last 20 years from this part of Delhi in our department.

Along with the infection, an altered immune response with impaired cellular immunity leads to the development of the disease. [3] However, an alteration in CD4:CD8 population in blood has been postulated as a cause of chronicity of this disease. [7] The altered proportion of CD4+ and CD8+ lymphocytes in the lesion may produce disabled macrophages, allowing bacterial multiplication inside them and an ineffectual delayed type hypersensitivity response. [3] The disease in early catarrhal stage is characterized by subepithelial infiltration by bacilli followed by capillary proliferation. The plasma cells infiltrate in chronic stage and granuloma form in the last stage. [3] Histiocytes are collected aggregated and show phagocytosis of bacteria, neutrophils. Subsequently, their phagosomes are distended and become Mikulicz cell. [3] In the presence of granulomas, leprosy, tuberculosis, leishmaniasis, and syphilis have to be ruled out and was done in this case too. [1],[4]

The cytological features are though not pathognomonic, but the lymphoplasmacytic inflammatory infiltrate admixed with classical Mikulicz cells should alert the pathologist to have good clinicoradiological correlation in excluding rhinoscleroma as the diagnosis. Mistaken diagnosis of metastatic carcinoma has also been rendered in absence of awareness of this entity. [1] It is also important to look for emperipolesis to rule out Rosai Dorfman disease (RDD). [8] CD68 is helpful to distinguish these cells from histiocytes of RDD. [8] All stains including PAS, AFB, Gram's, Groccott's silver methenamine, Warthin Starry, and particularly, CD68 are helpful for the diagnosis. [8]


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