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Rhinoscleroma
Igor Teixeira Raymundo1, Sharlene Castanheira Pádua1, Thaís Gonçalves Pinheiro1, Ana Emília Borges de Azevedo2,
Márcio Nakanishi3, Carlos Augusto Costa Pires de Oliveira4.
1) Resident Physician in Otolaryngology at University Hospital of Brasilia2) Resident Physician in Pathology at University Hospital of Brasilia3) Doctor in Otolaryngology. Otolaryngologist at University Hospital of Brasilia4) Doctorate in Medicine by University of Minnesota. Professor at University of Brasilia and Head of Otorhinolaryngology Department of University Hospital of Brasilia University Hospital of Brasilia.
Brasilia/DF - Brazil Mailling address: Igor Teixeira Raymundo - SHIN QI 10 conj. 10 CS 08 Lago Norte - Brasília / DF - Brazil - Zip Code: 71525-100.
Article received on September 3, 2009. Article accepted on October 3, 2009.
Introduction: Rhinoscleroma is a chronic granulomatous Introdução: Rinoscleroma é uma doença infecciosa crônica infectious disease caused by the bacterium Klebsiella do tipo granulomatosa causada pela bactéria Klebsiella rhinoscleromatis. It affects the respiratory tract mucosa, most rhinoscleromatis. Acomete a mucosa do trato respiratório, mais frequently in the nose. It is considered endemic to certain frequentemente o nariz. É considerada endêmica em determi- countries of Africa and Central America, but is rare in Brazil.
nadas regiões com África e América Central, porém é rara no Nasal involvement occurs in 3 phases: catarrhal, Brasil. O acometimento nasal ocorre em 3 fases: catarral, granulomatous, and sclerotic. Throughout its course, the disease granulomatosa e cicatricial. Em todo o seu curso a doença presents nonspecific symptoms, making it difficult to diagnose.
apresenta sintomatologia inespecífica, daí a dificuldade em Diagnosis is established by culture or by anatomopathological ser diagnosticada. Seu diagnóstico é estabelecido através de observation of Mikulicz cells or Russell corpuscles. Treatment cultura ou pelo encontro de células de Mikulicz ou corpús- consists of long-term antibiotic therapy and, occasionally, culo de Russel no estudo anatomopatológico. O tratamento consiste em antibioticoterapia por longo período, associada Objective: We report a case of rhinoscleroma in a young woman who complained of obstruction in both nostrils and persistent Objetivo: Este relato tem por objetivo ilustrar um caso de headache. Our intent is to enable otorhinolaryngologists to rinoscleroma em uma paciente jovem com queixa de obstru- diagnose this rare disease, which presents with nonspecific ção nasal bilateral de longa data e cefaleia. O intuito é alertar symptoms that resemble numerous pathologies of the nasal os otorrinolaringologistas para o diagnóstico desta doença rara, que se apresenta com sintomas inespecíficos e semelhantes Keywords: rhinoscleroma, klebsiella infections, nasal acquired a inúmeras patologias que acometem a região nasal.
Palavras-chave: rinoscleroma, infecções por klebsiella,deformidades adquiridas nasais.
Intl. Arch. Otorhinolaryngol., São Paulo - Brasil, v.15, n.4, p. 526-528, Oct/Nov/December - 2011.
Rhinoscleroma is a chronic granulomatous infectious disease that compromises the respiratory tract mucosa(most frequently in the nose) and may eventually extendto the lower airways (the larynx, trachea, and bronchi).
Recently, practitioners have adopted the term scleroma(1,2). It was first described by Ferdinando Von Hebra in1870 (3).
Rhinoscleroma is an infectious disease caused by the bacterium Klebsiella rhinoscleromati, an encapsulatedgram-negative member of Enterobacteriaceae that can beisolated by culture medium. It is considered endemic tosome countries of Africa, Central America, and South America, but is rare in Brazil (4). It is associated with somepredisposing factors such as low socioeconomic status,poor hygiene, immunodepression, and contact with infectedpatients (5).
The disease develops insidiously from the nasal mucosa, and progression occurs in 3 phases: catarrhal(characterized by rhinorrhea, crusting, and nasal obstruction,often confused with simple rhinitis); granulomatous (wherenodes are found in the submucosa and infiltrating lesions);and sclerotic (marked by gross scar tissue, which may occurin the vestibule and/or in larynx stenosis) (1). The differential diagnoses include neoplasms and other inflammatoryconditions such as leprosy, paracoccidioidomycosis,sarcoidosis, and Wegener granulomatosis (6).
Diagnosis can be confirmed by culture (with 50% to 60% positive specificity) or by histopathology. Treatment On examination, she presented with significant consists of antibiotic therapy and, in some cases, surgery nasal pyramid bulging. Previous rhinoscopy showed a lesion with a granulomatosis aspect, occupying both nasalcavities near the vestibule (Picture 1). Laryngoscopy wasnormal.
Computed tomography of the paranasal sinuses showed soft tissue material occupying the lower Otorhinolaryngology Service of Brasilia’s University Hospi- portion of the nasal cavities without maxillary sinus tal complained of obstruction in both nostrils since the past involvement. There were no signs of bone destruction 3 years, significant loss of sleep, and frequent headaches in the frontal region. She denied vocal alterations or dyspnea.
She reported that 2 years ago she underwent unsuccessful The patient underwent biopsy of the lesion under nasal surgery for synechia resection. At that time, no biopsy local anesthesia, and pathology revealed diffuse infiltration of distended and vacuolated histiocytes with roundednuclei located eccentrically (Mikulicz cells) (Picture 3).
She denied drug use, nasal trauma, immunological Giemsa, PAS, and Warthin-Starry staining revealed deficiency, or family history of similar symptoms. She has intracytoplasmic bacilli. These findings established the never been a smoker or an alcoholic.
Intl. Arch. Otorhinolaryngol., São Paulo - Brasil, v.15, n.4, p. 526-528, Oct/Nov/December - 2011.
Several antibiotics can be used to treat rhinoscleroma.
Tetracycline or streptomycin is typically used for a minimumperiod of 4 weeks. Quinolones have also been proveneffective, with the advantage of fewer side effects (2). Wechose gemifloxacin in our case because it is the onlyrespiratory quinolone available freely to the patients in thisambulatory clinic.
In addition to its rarity in Brazil, the diagnosis of rhinoscleroma can be especially difficult due to severalfactors such as differential diagnosis, limited sensitivity ofdiagnostic methods, and varying form of presentation The treatment was tetracycline therapy (500 mg There was partial reduction in the lesion size. We 1. Canalis FR, Zamboni L. An Interpretation of the Structural added concomitant gemifloxacin (320 mg/day for 2 weeks).
Changes Responsible for the Chronicity of Rhinoscleroma.
After completing this antibiotic cycle, there was complete lesion remission, although slight cicatricial stenosis of thenasal cavities remained. Given the significant clinical 2. Simons ME, Granato L, Oliveira RC, Alcantara MP.
improvement, the patient chose not to undergo further Rinoscleroma: relato de caso. Rev Bras Otorrinolaringol. 2006, 3. Von Frisch A. The etiology of rhinoscleroma. Wien Med The patient presented with the classical symptoms 4. Hart CA, Rao SK. Editorial: Rhinoscleroma. J Med Microbiol.
of rhinoscleroma, restricted to the nasal mucosa.
Nevertheless, the disease can affect other respiratory tractregions, such as the larynx (15-40%), nasopharynx (18– 5. Chan TV, Spiegel JH. Klebsiella rhinoscleromatis of the 43%), paranasal sinuses (26%), trachea (12%), and bronchi membranous nasal septum. J Laryngol Otol. 2007, 121:998- Histopathological analysis validated the diagnosis 6. Andraca R, Edson R, Kern E. Rhinoscleroma: a growing by revealing the presence of classical Mikulicz cells concern in the United States? Mayo Clinic experience. Mayo (histiocytes containing bacillus) or Russell corpuscles (plas- ma cells with hyaline degeneration). These findings areeasily recognized when the disease is in the granulomatous 7. Badia L, Lund VJ. A case of rhinoscleroma treat with stage. The diagnosis can also be defined by culture medium, ciprofloxacin. J Laryngol Otol. 2001, 115:220-2.
which has 50% to 60% specificity (3).
Intl. Arch. Otorhinolaryngol., São Paulo - Brasil, v.15, n.4, p. 526-528, Oct/Nov/December - 2011.

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