Rhinoscleroma Presenting as a Mass Lesion – An Unusual Presentation in the Era of Antibiotics
Year: 2025
Type: article
Study design: Case Report
Abstract: INTRODUCTION Rhinoscleroma is a specific type of chronic granulomatous inflammation, caused by a Gram-negative bacilli Klebsiella rhinoscleromatis (KR) which is a subspecies of Klebsiella pneumoniae (KP).[1] The disease involves the upper respiratory tract. Commonly, it involves the nasopharynx, however, it may also involve paranasal sinuses, oropharynx, larynx, tracheobronchial tree, rarely middle ear and cervical lymph node.[2] In rare cases, intracranial extension has been described.[1] The term ‘rhinoscleroma’ was first used by Von Hebra in 1870.[3] The causative agent was first described by Von Frisch in 1882, as a member of the Enterobacteriaceae family, KR.[4] Rhinoscleroma is endemic in low-income areas with poor hygiene conditions and regions of Central Africa, Egypt, the Middle East, India, Indonesia, the Middle East and Central America.[2,5] In view of reduced incidence in the current times epidemiologic data remains limited.[2,5] CASE REPORT A 48-year-old male presented with a mass over the nasal bridge, slowly growing over 6 months. On clinical examination, there was a painless, subcutaneous nodular mass lesion measuring 2 cm × 2 cm × 1 cm [Figure 1]. A complete excision of the lesion was done and the sample was sent for histopathology. On gross examination, it was a skin-covered mass with grey white cut surface. The tissue was processed entirely and stained with haematoxylin and eosin. On microscopy, sections revealed the epidermis with hyperkeratosis and flattening of rete ridges [Figure 2a]. Dense inflammation was seen in the dermis. There were sheets and clusters of histiocytes with dense lymphoplasmacytic infiltrate [Figure 2a-c]. Foamy macrophages, Russell bodies [Figure 2c] and occasional giant cells were seen. Foamy histiocytes were filled with bacilli (Mikulicz cells) [Figures 2d and 3a]. There were few proliferating capillaries with mild congestion. There was no evidence of malignancy in the material studied. In view of the presence of chronic granulomatous inflammation in the dermis the Fite-Faraco stain was done to rule out leprosy, it was negative [Figure 3c]. Other special stains periodic acid-Schiff (PAS) [Figure 3b] and Giemsa [Figure 3d] showed weak-to-moderate positive staining for the bacilli. These bacilli were negative for acid-fast stain (Zeihl–Neelson) stain, thus ruling out tuberculosis. The final diagnosis was rhinoscleroma. Postoperatively the patient was given a 3-month course of ciprofloxacin. Subsequently the patient recovered in one and half months and did not show any recurrence on a 6-month follow-up.Figure 1: Elevated nodular lesion over the nasal bridgeFigure 2: (a) Epidermis with flattening of rete ridges and dense inflammation in the dermis (H and E, ×100), (b) Sheets of Mikulicz cells (H and E, ×400), (c) Lymphoplasmacytic infiltrate with occasional Russell bodies (H and E, ×400), (d) Mikulicz cells with intermixed inflammation (H and E, ×400)Figure 3: (a) Sheets of foamy macrophages (H and E, ×400), (b) Periodic acid-Schiff stain showing few intracytoplasmic bacilli (oil lens ×1000 PAS stain), (c) Fite-Faraco stain (oil lens ×1000 special stain), (d) Giemsa showing few faint intracytoplasmic bacilli (oil lens ×1000 special stain)DISCUSSION KR is a subspecies of KP occasionally, Klebsiella ozaenae and K. pneumoniae have been reported.[1,2] The mode of transmission is postulated to be airborne contaminated particles or fomites and it is noted in areas of low levels of hygiene.[1] KR mainly affects humans, however, rare reports of its identification in cockroaches and chickens in poor hygiene settings have been noted.[6] In a review by Umphress and Raparia, the age of the patients ranged from 5 to 69 years with a female preponderance.[2] Clinically, the infection has three distinct stages with clinical and pathological overlap.[6] It begins as a catarrhal phase, presenting as non-specific rhinitis that can last for months and then it is followed by purulent rhinorrhoea with crusting. The granulomatous stage shows congested nodular lesion.[2] The last stage is the cicatricial phase with extensive scarring. Other clinical features that can be noted are the destruction of nasal cartilage, epistaxis and nasal deformities.[1] The present case had a nodular lesion, the patient did not complain of any other symptoms. Histopathology findings and their inflammatory and nonneoplastic behavior were provided in detail by Mikulicz in 1877.[5] The catarrhal phase shows granulation tissue with neutrophils, granulomatous stage shows dense infiltration of plasma cells, lymphocytes, Russell bodies and foamy macrophages with bacilli called the Mikulicz cells.[7] The catarrhal and sclerotic stages usually do not have these Mikulicz cells and sclerotic stages show predominantly fibrosis.[7] The exact pathway of Mikulicz cell formation is uncertain, however, KR has a mucopolysaccharide capsule that interferes with the deposition of complement and phagocytosis, thus aiding in its intracellular survival.[1,2] It has been postulated that there is a defect in cellular immunity, a reduced number of CD4 cells as compared to CD8 cells and this may result in improper function of macrophages.[2] The strain of KP causing rhinoscleroma is capsular type 3 (K3). KR has certain typical biochemical features such as positive test results for methyl red, negative for urease and negative for citrate reductase.[8] The current sample was received in formalin for histopathology, hence above tests were not done and the diagnosis was made based on clinical features and histopathology. Corelli et al. described that Mikulicz cell formation and interleukin-10 production are not dependent on KR capsular polysaccharide, they inactivated capsule export machinery in KR and studied its virulence in murine models.[6] Although rhinoscleroma has a chronic course there is a failure to form well-formed granuloma and poor T-cell response.[1] On histopathology, rhinoscleroma may mimic numerous nonneoplastic and neoplastic lesions. Ahmed et al. have described that in paucity or absence of Mikulicz major criteria such as predominance of plasma cells, absence of eosinophils and minor criteria such as young female patient, bilateral nasal involvement, squamous metaplasia, nasal crustation, neutrophils and Russell bodies may be considered for diagnosis.[7] Mikulicz cell predominance leads to confusion with other histiocytic proliferation [Table 1], such as Rosai-Dorfman disease, clinically there is painless cervical lymphadenopathy, massive splenomegaly with extranodal disease in the central nervous system and skin.[2] The present case showed a dermal lesion; however, other features such as splenomegaly and emperipolesis were not prominent. Leprosy shows histiocytic proliferation and macrophages filled with lepra bacilli, these bacilli are positive for Fite-Faraco stain.[4] Other conditions to be considered are atypical mycobacterial infection, fungal infections, syphilitic gumma, granulomatosis with polyangiitis and lymphoproliferative disorders in cases of predominant lymphocytes and plasma cells.[2] Histiocytic proliferations such as juvenile xanthogranuloma and Erdheim–Chester disease (which shows systemic histiocytosis) should also be considered.[2]Table 1: Differential diagnosis of granulomatous stage of rhinoscleromaNon-necrotising granulomas of Sarcoidosis may create a diagnostic dilemma, however, the disease also has lung and widespread skin involvement.[3] On MacConkey agar lactose fermenting mucoid colonies are noted in 50%–60% of cases and bacilli are Gram-negative, PAS and Giemsa-positive.[4] Most treatment protocols comprise excision of the mass lesion and long-term antibiotic treatment (trimethoprim and sulfamethoxazole or quinolones, cephalosporins and doxycycline/tetracycline, alone or in combination).[9] The present case was treated with ciprofloxacin for 3 months then he recovered. CONCLUSION Rhinoscleroma presenting as a subcutaneous mass lesion is rare in current times owing to better hygiene and early access to antibiotics. Most of the patients take treatment in the initial catarrhal stage. This report highlights the significance of meticulous histopathological examination of such cases and to rule out other similar neoplastic and non-neoplastic proliferations of histiocytes. The disease responds to antibiotics, however, in view of recurrences follow-up is advised. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.
Source: Journal of Medical Evidence
Authors: Rakesh Rajiv Patkar, Shilpa Mishra
Language: English
Subfield: Small Animals
Field: Veterinary
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W4412013924