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Rhinoscleroma in an Immigrant From Egypt: A Case Report

Edgardo Bonacina, MD,∗ Leonardo Chianura, MD, DTM&H,† Maurizio Sberna, MD,‡ Giuseppe Ortisi, MD,§ Giovanna Gelosa, MD,|| Alberto Citterio, MD,‡ Giovanni Gesu, MD,§ and Massimo Puoti, MD† Downloaded from https://academic.oup.com/jtm/article/19/6/387/1795562 by guest on 23 September 2021 Departments of ∗Pathological Anatomy; †Infectious Diseases; ‡Neuroradiology; §Microbiology, and; ||Otorinolaringoiatry, Niguarda Ca` Granda Hospital, Milano, Italy

DOI: 10.1111/j.1708-8305.2012.00659.x

Rhinoscleroma is a chronic indolent granulomatous infection of the nose and the upper 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.

hough rarely observed, rhinoscleroma has to be nasal fossae and ethmoid sinuses with complete bony T taken into consideration in travelers returning destruction of bilateral nasal turbinates (Figure 1). with ear, nose, and throat presentations, particularly Endoscopic biopsy was performed under local anesthe- after traveling to developing countries or regions where sia. Histopathologic examination revealed numerous this condition is endemic.1,2 foamy macrophages (Mikulicz cells) containing bacteria (Figure 2); no fungal hyphae were found.3 Staphylococcus Case Report aureus and Klebsiella rhinoscleromatis were isolated by culture of the tissue biopsy. A diagnosis of rhinoscle- A 51-year-old Egyptian male immigrant presented on roma was made. Staphylococcus aureus was sensitive May 14, 2010 at our hospital, with a 25-day history to all antibiotics tested. Klebsiella rhinoscleromatis was of light epistaxis from his left nostril. He had lived in resistant to amoxicillin but sensitive to the following Italy for 8 years and not traveled back to Egypt. Nasal antibiotics: co-amoxiclav, piperacillin-tazobactam, endoscopy revealed a spontaneously nodule meropenem, cefotaxime, ceftazidime, imipenem, occupying the left nasal fossa. gentamicin, amikacin, ciprofloxacin, levofloxacin, and Blood tests including full blood count, coagulation co-trimoxazole. Treatment was commenced with oral screen, glucose, bone profile, and renal and liver levofloxacin (500 mg once daily), rifampicin (600 mg function were all normal; inflammatory markers were once daily), and co-trimoxazole (sulfamethoxazole not requested for. Lymphocyte subset analysis revealed 1600 mg/trimethoprim 320 mg, three times a day) a CD4/CD8 ratio at the upper limit of normal (2.9; for 3 months, followed by levofloxacin (500 mg normal range 0.70–2.90); CD4 lymphocyte count once daily) and co-trimoxazole (sulfamethoxazole was 778 cells/μL. He tested positive for hepatitis C 800 mg/trimethoprim 160 mg, three times a day) for 9 (HCV-RNA 2 443 IU/mL; Abbott RealTime HCV months. His clinical course was followed up at monthly assay Abbott Molecular, Wiesbaden, Germany), HBsAg intervals in the outpatient department. was absent, and anti-HIV was negative. Repeat MRI scans at 8 and 11 months showed a Computed tomography (CT) scanning and magnetic decrease in the diameter of the implying resonance imaging (MRI) showed a mass in the favorable response to therapy (Figure 3).

Corresponding Author: Leonardo Chianura, MD, Discussion DTM&H, Department of Infectious Diseases, Niguarda Ca` Granda Hospital, Piazza Ospedale Maggiore, 3, I-20162 Rhinoscleroma is endemic to many countries but this Milano, Italy. E-mail: [email protected] chronic granulomatous disease occurs sporadically in

© 2012 International Society of Travel Medicine, 1195-1982 Journal of Travel Medicine 2012; Volume 19 (Issue 6): 387–390 388 Bonacina et al. Downloaded from https://academic.oup.com/jtm/article/19/6/387/1795562 by guest on 23 September 2021

A1 B1 B3

A2 B2 B4

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.

Western Europe usually in immigrant populations destruction are also features. The third sclerotic stage is arriving from countries where the disease is endemic. characterized by extensive fibrosis leading to extensive This disease is transmitted by air and humans are the scarring and possible nasal/laryngeal stenosis.2,5 only identified host. Our patient had lived in Italy The lack of awareness when disease presents in for 8 years without traveling back to Egypt; we had developed countries may lead to a delay in diagnosis hypothesized that he might have contracted the disease and can cause nasal deformities, airway obstruction, in Italy living in close contact with other immigrants and symptoms mimicking allergic or prolonged from Egypt. Moreover, we cannot exclude the possibility sinusitis. the patient might have acquired the infection in his Rhinoscleroma may mimic granulomatous, neoplas- country of origin with a delay in diagnosis because of tic or systemic infectious diseases including tuber- the slow progression of the disease. culosis, actinomycosis, , , histoplasmo- Rhinoscleroma usually involves the nasal cavity and sis, blastomycosis, paracoccidioidomycosis, sporotri- nasopharynx, but it may also affect the , , chosis, mucocutaneous leishmaniasis, lymphomas, bronchi, the middle ear, oral cavity, paranasal sinuses, verrucous carcinoma, sarcoidosis, and Wegener’s orbit, soft tissues of the lips, and nose. granulomatosis. 5 Rhinoscleroma is divided into three stages: catarrhal, Although radiological appearance is not pathog- granulomatous, and fibrotic.4,5 The catarrhal stage nomonic of rhinoscleroma, MRI should be performed causes symptoms of non-specific rhinitis that can because nasal masses can obstruct the ostiomeatal units last for weeks or months and often evolves into and secretions may be retained in the related sinuses. purulent and fetid with crusting. The second In the hypertrophic stage of rhinoscleroma, both T1- granulomatous stage is characterized by development and T2-weighted images show characteristic mild-to- of a bluish red nasal mucosa and intranasal rubbery marked high signal intensity.6 nodules or polyps, and manifests with epistaxis and nasal Nasal endoscopy may reveal signs of all three stages deformity; destruction of the nasal cartilage and bony of rhinoscleroma and aids accurate diagnosis based

J Travel Med 2012; 19: 387–390 Rhinoscleroma: A Case Report 389

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 Downloaded from https://academic.oup.com/jtm/article/19/6/387/1795562 by guest on 23 September 2021 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. Figure 2 Histological section (hematoxylin and eosin stain; The causative organism is resistant to most antibiotics ×400) showing foamy macrophages, so-called ‘‘Mikulicz cells’’ and, being intracellular, is not always exposed to (M) in a background of plasma cells (P) with occasional sufficient concentrations of the drug.8,10 Because K Russell bodies (R) in classical ‘‘Mott’’ cells. Insert shows rhinoscleromatis is an intracellular bacteria, prolonged argyrophilic bacterial forms (arrow) consistent with Klebsiella courses of rifampicin and fluoroquinolones would rhinoscleromatis, Warthin–Starry stain. theoretically be most effective, owing to their high concentration in macrophages.14 On the basis of the physical and radiological on histopathological examination and isolation of K examination, we adopted a conservative (non-surgical) rhinoscleromatis in culture.7 approach prior to biopsy results; when the diagnosis A positive culture in MacConkey agar is diagnostic of rhinoscleroma in granulomatous stage was made, of rhinoscleroma, but it is positive in only 50 to 60% surgical therapy was not considered, as there was no of patients. The diagnosis is confirmed by histology. nasal or pharyngeal obstruction.6 Classic histopathologic findings include plasma cells In our patient, considering the extension of the and large vacuolated Mikulicz cells with clear cytoplasm lesion with invasion into the ethmoid sinuses and its that contains bacilli and Russell bodies (which are potential extension to the central nervous system,10,15 transformed plasma cells). he was given an aggressive antibiotic treatment with Treatment of rhinoscleroma requires a combination levofloxacin and co-trimoxazole for 12 months, plus of appropriate antibiotics and surgical debridement if rifampicin in the first 3 months. Moreover, in this there is significant airway obstruction. The results of case superinfection by Saureuswas associated with current treatment are unsatisfactory and recurrence rhinoscleroma; the antibiotics combination he was given often occurs.8 Moreover, no randomized controlled is extensively used for .16 trials exist to compare various antibiotic treatment In our case, the detailed MRI follow-up performed choices and their efficacy.8,9 after 8 and 11 months had shown improvement based De Pontual and colleagues in their retrospective on both a decrease in the granuloma diameter and a series of 11 patients report a treatment duration of 3 to reduction of enhancement. 9 months with ciprofloxacin (7 patients), ceftriaxone We suggest that the antibiotic treatment of (2), (2), and clofazimine (2). Relapses rhinoscleroma in the granulomatous stage associated occurred in 3 of the 11 patients. They recommend with a bony destruction should be continued for at fluoroquinolones as the first drug of choice, because least 6 months; in an economically developed country of its good activity against Gram-negative bacilli, it should be maintained until repeated MRI scanning intracellular efficacy, and low toxicity profile.10 shows improvement. Gaafar and colleagues in their retrospective case series of 56 cases over 10 years report a medical Conclusion treatment duration of 3 months with a combination of co-trimoxazole and rifampicin. Since 2003, this was This report presents a case of nasal rhinoscleroma in replaced by ciprofloxacin for 3 months. Results were granulomatous stage in an urban non-endemic setting. disappointing, as a high incidence of recurrence was Rhinoscleroma is extremely rare in Italy. Consequently, found reaching up to 25% within 10 years.8 clinicians are infrequently confronted with this disease Fawaz and colleagues in their study of 88 cases report and the diagnosis may be missed. CT and MRI scans a treatment duration of 4 to 20 weeks with rifampicin (63 are useful in suggesting invasive space-occupying lesions patients), co-trimoxazole (11), and ciprofloxacin (14). with bony destruction of nasal turbinates. The diagnosis Relapses occurred in 24 out of 88 patients (27%).11 in this case was confirmed by histopathological findings

J Travel Med 2012; 19: 387–390 390 Bonacina et al.

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C2 C4 D2 D4

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). and by isolation of K rhinoscleromatis. Surgery was not 7. Gamea AM. Role of endoscopy in diagnosing scleroma in considered in this patient as there was no nasal or its uncommon sites. J Laryngol Otol 1990; 104:619–21. pharyngeal obstruction; he was treated with intensive 8. Gaafar HA, Gaafar AH, Nour YA. Rhinoscleroma: an antibiotic therapy until detailed clinical and imaging updated experience through the last 10 years. Acta follow-up showed benefits. Otolaryngol 2011; 131:440–6. 9. N’gattia KV, Kacouchia N, Koffi-N’guessan L, et al. Retrospective study of the rhinoscleroma about 14 cases Declaration of Interests in ENT departments of university hospitals. Eur Ann Otorhinolaryngol Head Neck Dis 2011; 128:7–10. The authors state they have no conflicts of interest to 10. de Pontual L, Ovetchkine P, Rodriguez D, et al. declare. Rhinoscleroma: a French national retrospective study of epidemiological and clinical features. Clin Infect Dis 2008; 47:1396–402. References 11. Fawaz S, Tiba M, Salman M, Othman H. Clinical, radiological and pathological study of 88 cases of typical 1. Miller RH, Shulman JB, Canalis RF, Ward PH. and complicated scleroma. Clin Respir J 2011; 5:112–121. Klebsiella rhinoscleromatis: a clinical and pathogenic enigma. 12. Zhong Q, Guo W, Chen X, et al. Rhinoscleroma: a Otolaryngol Head Neck Surg 1979; 87:212–221. retrospective study of pathologic and clinical features. 2. Suchanova PP, Mohyuddin NG, Rodriguez-Waitkus PM, J Otolaryngol Head Neck Surg 2011; 40:167–74. Eicher SA. Rhinoscleroma in an urban nonendemic 13. Tan SL, Neoh CY, Tan HH. Rhinoscleroma: a case series. setting. Otolaryngol Head Neck Surg 2011; 11:22. Singapore Med J 2012; 53:24–27. 3. Mikulicz J. Veber das rhinosclerom. Arch F Klin Chir 14. Maguina˜ C, Cortez-Escalante J, Osores-Plenge F, et al. 1877; 20:485. Rhinoscleroma: eight Peruvian cases. Rev Inst Med Trop 4. Hart CA, Rao SK. Rhinoscleroma. J Med Microbiol 2000; Sao˜ Paulo 2006; 48:295–9. 49:395–6. 15. Bahri HC, Bassi NK, Rohatgi MS. Scleroma with 5. Andraca R, Edson RS, Kern EB. Rhinoscleroma: a intracranial extension. Ann Otol Rhinol Laryngol 1972; growing concern in the United States? Mayo Clinic 81:856. experience. Mayo Clin Proc 1993; 68:1151–7. 16. Fraimow HS. Systemic antimicrobial therapy in 6. Razek AA, Elasfour AA. MR appearance of rhinoscleroma. osteomyelitis. Semin Plast Surg 2009; 23:90–99. Am J Neuroradiol 1999; 20:575–8.

J Travel Med 2012; 19: 387–390