Rhinoscleroma in an Immigrant From Egypt: A Case Report

Authors


Corresponding Author: Leonardo Chianura, MD, DTM&H, Department of Infectious Diseases, Niguarda Cà Granda Hospital, Piazza Ospedale Maggiore, 3, I-20162 Milano, Italy. E-mail: leochianura@libero.it

Abstract

Rhinoscleroma is a chronic indolent granulomatous infection of the nose and the upper respiratory tract caused by Klebsiella rhinoscleromatis; this condition is endemic to many regions of the world including North Africa. We present a case of rhinoscleroma in a 51-year-old Egyptian immigrant with 1-month history of epistaxis. We would postulate that with increased travel from areas where rhinoscleroma is endemic to other non-endemic areas, diagnosis of this condition will become more common.

Though rarely observed, rhinoscleroma has to be taken into consideration in travelers returning with ear, nose, and throat presentations, particularly after traveling to developing countries or regions where this condition is endemic.[1, 2]

Case Report

A 51-year-old Egyptian male immigrant presented on May 14, 2010 at our hospital, with a 25-day history of light epistaxis from his left nostril. He had lived in Italy for 8 years and not traveled back to Egypt. Nasal endoscopy revealed a spontaneously bleeding nodule occupying the left nasal fossa.

Blood tests including full blood count, coagulation screen, glucose, bone profile, and renal and liver function were all normal; inflammatory markers were not requested for. Lymphocyte subset analysis revealed a CD4/CD8 ratio at the upper limit of normal (2.9; normal range 0.70–2.90); CD4 lymphocyte count was 778 cells/μL. He tested positive for hepatitis C (HCV-RNA 2 443 IU/mL; Abbott RealTime HCV assay Abbott Molecular, Wiesbaden, Germany), HBsAg was absent, and anti-HIV was negative.

Computed tomography (CT) scanning and magnetic resonance imaging (MRI) showed a mass in the nasal fossae and ethmoid sinuses with complete bony destruction of bilateral nasal turbinates (Figure 1). Endoscopic biopsy was performed under local anesthesia. Histopathologic examination revealed numerous foamy macrophages (Mikulicz cells) containing bacteria (Figure 2); no fungal hyphae were found.[3] Staphylococcus aureus and Klebsiella rhinoscleromatis were isolated by culture of the tissue biopsy. A diagnosis of rhinoscleroma was made. Staphylococcus aureus was sensitive to all antibiotics tested. Klebsiella rhinoscleromatis was resistant to amoxicillin but sensitive to the following antibiotics: co-amoxiclav, piperacillin-tazobactam, meropenem, cefotaxime, ceftazidime, imipenem, gentamicin, amikacin, ciprofloxacin, levofloxacin, and co-trimoxazole. Treatment was commenced with oral levofloxacin (500 mg once daily), rifampicin (600 mg once daily), and co-trimoxazole (sulfamethoxazole 1600 mg/trimethoprim 320 mg, three times a day) for 3 months, followed by levofloxacin (500 mg once daily) and co-trimoxazole (sulfamethoxazole 800 mg/trimethoprim 160 mg, three times a day) for 9 months. His clinical course was followed up at monthly intervals in the outpatient department.

Figure 1.

Axial TC (A1), coronal TC (A2), T2 axial MRI (magnetic resonance imaging) (B1), T2 coronal MRI (B2), T1 axial MRI (B3), and T1 coronal contrast-enhanced MRI (B4) show soft tissue mass over the nasal fossae and ethmoid sinuses with complete bony destruction of bilateral nasal turbinates, and partial loss of the nasal septum with loss of the ethmoidal floor. Maxillary sinuses appear opaque and partially opaqued the right frontal sinus. Post-gadolinium with fat suppression (B4) shows heterogeneous enhancing soft tissue over nasal fossae and ethmoid sinuses with bony destruction of bilateral nasal turbinates.

Figure 2.

Histological section (hematoxylin and eosin stain; ×400) showing foamy macrophages, so-called “Mikulicz cells” (M) in a background of plasma cells (P) with occasional Russell bodies (R) in classical “Mott” cells. Insert shows argyrophilic bacterial forms (arrow) consistent with Klebsiella rhinoscleromatis, Warthin–Starry stain.

Repeat MRI scans at 8 and 11 months showed a decrease in the diameter of the granuloma implying favorable response to therapy (Figure 3).

Figure 3.

The magnetic resonance imaging (MRI) studies performed as follow-up have documented an improvement based on a decrease in the granuloma diameter. MRI follow-up after 8 months of therapy: T2 axial MRI (C1), T2 coronal MRI (C2), T1 axial MRI (C3), and T1 coronal contrast-enhanced MRI (C4). MRI follow-up after 11 months of therapy: T2 axial MRI (D1), T2 coronal MRI (D2), T1 axial MRI (D3), and T1 coronal contrast-enhanced MRI (D4).

Discussion

Rhinoscleroma is endemic to many countries but this chronic granulomatous disease occurs sporadically in Western Europe usually in immigrant populations arriving from countries where the disease is endemic. This disease is transmitted by air and humans are the only identified host. Our patient had lived in Italy for 8 years without traveling back to Egypt; we had hypothesized that he might have contracted the disease in Italy living in close contact with other immigrants from Egypt. Moreover, we cannot exclude the possibility the patient might have acquired the infection in his country of origin with a delay in diagnosis because of the slow progression of the disease.

Rhinoscleroma usually involves the nasal cavity and nasopharynx, but it may also affect the larynx, trachea, bronchi, the middle ear, oral cavity, paranasal sinuses, orbit, soft tissues of the lips, and nose.

Rhinoscleroma is divided into three stages: catarrhal, granulomatous, and fibrotic.[4, 5] The catarrhal stage causes symptoms of non-specific rhinitis that can last for weeks or months and often evolves into purulent and fetid rhinorrhea with crusting. The second granulomatous stage is characterized by development of a bluish red nasal mucosa and intranasal rubbery nodules or polyps, and manifests with epistaxis and nasal deformity; destruction of the nasal cartilage and bony destruction are also features. The third sclerotic stage is characterized by extensive fibrosis leading to extensive scarring and possible nasal/laryngeal stenosis.[2, 5]

The lack of awareness when disease presents in developed countries may lead to a delay in diagnosis and can cause nasal deformities, airway obstruction, and symptoms mimicking allergic rhinitis or prolonged sinusitis.

Rhinoscleroma may mimic granulomatous, neoplastic or systemic infectious diseases including tuberculosis, actinomycosis, syphilis, leprosy, histoplasmosis, blastomycosis, paracoccidioidomycosis, sporotrichosis, mucocutaneous leishmaniasis, lymphomas, verrucous carcinoma, sarcoidosis, and Wegener's granulomatosis. [5]

Although radiological appearance is not pathognomonic of rhinoscleroma, MRI should be performed because nasal masses can obstruct the ostiomeatal units and secretions may be retained in the related sinuses. In the hypertrophic stage of rhinoscleroma, both T1- and T2-weighted images show characteristic mild-to-marked high signal intensity.[6]

Nasal endoscopy may reveal signs of all three stages of rhinoscleroma and aids accurate diagnosis based on histopathological examination and isolation of K rhinoscleromatis in culture.[7]

A positive culture in MacConkey agar is diagnostic of rhinoscleroma, but it is positive in only 50 to 60% of patients. The diagnosis is confirmed by histology. Classic histopathologic findings include plasma cells and large vacuolated Mikulicz cells with clear cytoplasm that contains bacilli and Russell bodies (which are transformed plasma cells).

Treatment of rhinoscleroma requires a combination of appropriate antibiotics and surgical debridement if there is significant airway obstruction. The results of current treatment are unsatisfactory and recurrence often occurs.[8] Moreover, no randomized controlled trials exist to compare various antibiotic treatment choices and their efficacy.[8, 9]

De Pontual and colleagues in their retrospective series of 11 patients report a treatment duration of 3 to 9 months with ciprofloxacin (7 patients), ceftriaxone (2), tetracycline (2), and clofazimine (2). Relapses occurred in 3 of the 11 patients. They recommend fluoroquinolones as the first drug of choice, because of its good activity against Gram-negative bacilli, intracellular efficacy, and low toxicity profile.[10]

Gaafar and colleagues in their retrospective case series of 56 cases over 10 years report a medical treatment duration of 3 months with a combination of co-trimoxazole and rifampicin. Since 2003, this was replaced by ciprofloxacin for 3 months. Results were disappointing, as a high incidence of recurrence was found reaching up to 25% within 10 years.[8]

Fawaz and colleagues in their study of 88 cases report a treatment duration of 4 to 20 weeks with rifampicin (63 patients), co-trimoxazole (11), and ciprofloxacin (14). Relapses occurred in 24 out of 88 patients (27%).[11]

Recently, Suchanova and colleagues in their study of three cases suggest that management with long-term antibiotics (3–6 months) with the fewest side effects (ciprofloxacin and co-trimoxazole) plus or minus surgical debridement is the mainstay of therapy.[2]

Zhong and colleagues in their retrospective case series of 40 patients over 30 years report that 27 patients remained relapse-free 1 to 10 years following treatment with antibiotics supplemented in some cases with surgery or radiotherapy.[12]

Tan and colleagues in their study of four cases recommend a treatment regime consisting of a combination of ciprofloxacin and doxycycline for at least 6 months.[13]

The cases of recurrences reported in the literature are not associated with any particular treatment regimen. The causative organism is resistant to most antibiotics and, being intracellular, is not always exposed to sufficient concentrations of the drug.[8, 10] Because K rhinoscleromatis is an intracellular bacteria, prolonged courses of rifampicin and fluoroquinolones would theoretically be most effective, owing to their high concentration in macrophages.[14]

On the basis of the physical and radiological examination, we adopted a conservative (non-surgical) approach prior to biopsy results; when the diagnosis of rhinoscleroma in granulomatous stage was made, surgical therapy was not considered, as there was no nasal or pharyngeal obstruction.[6]

In our patient, considering the extension of the lesion with invasion into the ethmoid sinuses and its potential extension to the central nervous system,[10, 15] he was given an aggressive antibiotic treatment with levofloxacin and co-trimoxazole for 12 months, plus rifampicin in the first 3 months. Moreover, in this case superinfection by S aureus was associated with rhinoscleroma; the antibiotics combination he was given is extensively used for staphylococcal infection.[16]

In our case, the detailed MRI follow-up performed after 8 and 11 months had shown improvement based on both a decrease in the granuloma diameter and a reduction of enhancement.

We suggest that the antibiotic treatment of rhinoscleroma in the granulomatous stage associated with a bony destruction should be continued for at least 6 months; in an economically developed country it should be maintained until repeated MRI scanning shows improvement.

Conclusion

This report presents a case of nasal rhinoscleroma in granulomatous stage in an urban non-endemic setting. Rhinoscleroma is extremely rare in Italy. Consequently, clinicians are infrequently confronted with this disease and the diagnosis may be missed. CT and MRI scans are useful in suggesting invasive space-occupying lesions with bony destruction of nasal turbinates. The diagnosis in this case was confirmed by histopathological findings and by isolation of K rhinoscleromatis. Surgery was not considered in this patient as there was no nasal or pharyngeal obstruction; he was treated with intensive antibiotic therapy until detailed clinical and imaging follow-up showed benefits.

Declaration of Interests

The authors state they have no conflicts of interest to declare.

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