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Rev Bras Otorrinolaringol CASE REPORT
Rhinoscleroma: Case Report
Mônica Elisabeth Simons1, Lidio Granato2, Roberto
Claudio Batista Oliveira3, Mônica Porto Alves
Keywords: klebsiella rhinoscleromatis, microscopy, Rhinoscleroma is a chronic, granulomatous infection that most frequently affects the respiratory mucosa, especially the nasal cavity and eventually extending through the lower respiratory tract. The disease is endemic in some countries of Central America (El Salvador and Guatemala), Indonesia, India, Poland, Hungary, Russia and some African countries as well. It is a rare disease in South America. We report a 51-year-old male resident of a psychiatric institution in São Paulo presenting with progressive nasal obstruction, frontal headache, yellowish nasal discharge and a mass extruding through the right nasal vestibule. The present case report describes a Rhino-Sinus scleroma where histopathology was vital in the diagnosis. The patient was treated by surgical excision of the nasal mass followed by a course of ciprofloxacin. He has remained asymptomatic up to the last visit six months following treatment and has shown no evidence of recurrence.
1 MD, Otorhinolaryngology Intern - Santa Casa de São Paulo.
2 Assistant Professor - Department of Otrhinolaryngology - Santa Casa de Misericórdia de São Paulo.
3 MD, Otorhinolaryngologist.
4 MD, 3rd Year Resident - Santa Casa de Misericórdia de São Paulo.
Mailing Address: Mônica Elisabeth Simons - Rua Dom Constantino Barradas 88 apto. 42 São Paulo SP 04134-110.
Tel/Fax: (0xx11) 5062-6448 - E-mail: [email protected] Paper submitted to the ABORL-CCF SGP (Management Publications System) on March 7th, 2005 and accepted for publication on September 8th, 2005.
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limits. Psychiatrically the patient was confused and agitated due to mental deficiency.
Rhinoscleroma is a chronic granulomatous, slowly Computed tomography of the paranasal sinuses progressive infection that affects the nose and other res- revealed a lesion with soft tissue attenuation material in piratory tract structures1. It was first described in 1870 by the nasal fossae, the right maxillary sinus, the anterior the dermatologist Ferdinando Von Hebra2 and posteriorly ethmoid cells, in some posterior ethmoid cells, and in named respiratory scleroma. The Greek name "skleroma," part of the frontal sinus to the right. There was minor soft meaning hard tumefaction, was adopted in 1932 at the tissue attenuation material in the left maxillary sinus. The International Clinical Otorhinolaryngology Conference (in sphenoid sinus and the rhinopharynx were unaltered and Madrid), emphasizing involvement of upper and lower no bone destruction was observed. (Figures 1 and 2) airways1,3. It occurs frequently in the nasal fossae, even- Treatment included surgery followed by antibiotics. tually extending itself to the larynx, the rhinopharynx, the Surgical removal of the tumor from the nasal fossae was mouth and the paranasal sinuses; the lips, trachea and a sinusectomy through the transmaxillary approach with bronchi may also be affected to a lesser degree4,5. Extra- a degloving access route. The specimen was referred to respiratory involvement has rarely been described1.
pathology for examination. At surgery a 3 cm diameter Scleroma is endemic in some Central American hard whitish-gray mass was found inserted in the right countries (El Salvador and Republic of Guatemala), Indo- maxillary sinus and the right nasal fossa, extending itself nesia, India, Poland, Hungary, Russia and some African into the ethmoid sinus; it had a smooth surface with irre- countries1,6. In South America the frequency is low1,7.
gular contours and contained pus. Bone structures were The first observation of scleroma published in Brazil preserved and the cartilage septum was deviated to the was made by Adolpho Lutz in 18908. Few cases have been left by tumoral compression.
reported in Brazilian medical literature, thus the reason for Pathology disclosed nasal mucosa containing a di- publishing this case, which includes the histopathology ffuse lymphoplasmacytic inflammatory process with large and treatment.
vacuolated macrophages typical of Mikulicz cells and many plasmocytes transformed into Russel bodies. (Figure 3) CLINICAL CASE PRESENTATION
Ciprofloxacin was used (500 mg. 12/12h for 14 days) based on literature data.
J.P.S is a 51-year-old brown unmarried male patient The patient returned to the Otorhinolaryngology born in Minas Gerais, coming from Sao Paulo (SP state), unit in good physical condition and no signs of recurrence and living in a psychiatric support institution. He was in the nasal mucosa.
brought to the Otorhinolaryngology unit at the Sao Paulo Santa Casa de Misericordia Hospital by his sister, and presented a reddish mass in his right nare, with frequent bleeding episodes, for the past five months. He also had a long history of sneezing, nasal pruritus, thickened and yellowish rhinorrhea, bilateral nasal obstruction, and daily intense frontal headaches.
On further investigation, he had lost some weight (three kilograms) during those five months, and was an ex-smoker and ex-drinker of alcoholic beverages, having given up these habits four years ago. He had worked as a polisher, in contact with chemical products during 10 years, and had been retired due to a psychiatric condition (mental deficiency). He was taking biperiden, chlorpro-mazine and haloperidol, and lived in a psychiatric support institution.
The otorhinolaryngological exam revealed an enlar- ged nose due to a violet-colored mass with small ulcers, a hardened consistency, a smooth surface, and irregular borders, which extruded from the right nare. The left nasal fossa was obstructed by a complete deviation of the nasal septum. It was not possible to introduce a nasopharyngos- Figure 1. Computed tomography of the paranasal sinuses - coronal
cope. On examination with the Glatzel mirror, both nasal plane. Soft tissue attenuation material occupies the nasal fossae, the fossae were not pervious.
right maxil ary sinus, and some ethmoid cel s. The cartilaginous septum The remaining physical exam was within normal is deviated to the left. There is no bone destruction.
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truction. This stage can last for weeks or months. (2) the hypertrophic stage: this stage includes granulation tissue with deformity by widening of the nasal pyramid, and nasal septum cartilage destruction. Epistaxis, anosmia, and anesthesia of the soft palate, among other symptoms, may occur. (3) the sclerotic stage: this stage is characterized by extensive scarring and laryngeal, and nasal vestibular stenosis in severe cases10,11.
Scleroma is an opportunistic disease in immunosup- pressed patients and has many differential diagnoses, such as specific granulomatous diseases caused by bacteria (tu-berculosis, actinomycosis, syphilis and leprosy), by fungi (histoplasmosis, blastomicosis, paracoccidioidomycosis), by other parasites (mucocutaneous leishmaniasis), systemic diseases (sarcoidosis and Wegener's granulomatosis), and neoplasms (verrucous carcinoma)12.
The patient in our case had a tumor that extruded from the right nare. It was typically a hypertrophic stage lesion. The first diagnosis, however, was of a neoplasm, al-though granulomatous diseases had not been excluded.
Given the emotional situation of the patient and Figure 2. Computed tomography of the paranasal sinuses - axial plane.
based on the physical exam and radiographic data, we Soft tissue at enuation material occupies the right nasal fossa, the right decided for surgery even without a preoperative biopsy. maxil ary sinus, and part of the left maxil ary sinus. The cartilaginous The complete specimen was sent to pathology. Frequently septum is deviated to the left. There is no bone destruction.
in these cases biopsies do not provide a final diagnosis, due to secondary contamination and insufficient material.
Histopathology defines the diagnosis based on fin- ding Mikulicz cells and degenerated plasmocytes in Russel bodies, described in 1877 by Johann Von Mikulicz13. The etiological agent is Klebsiella rhinoscleromatis, a Gram-negative facultative intracellular encapsulated bacillus, a member of the Enterobacteriaceae family identified by Von Frisch in 188214.
Mikulicz cells are clear cytoplasm vacuolated his- tiocytes containing the bacillus. Factors involved in trans-forming histiocytes into Mikulicz cells are unknown9.
Antibiotic treatment is used as a single treatment to eradicate the infection mostly in the catarrhal stage, or as ancillary treatment in other stages of the disease to reduce mortality and avoid complications. Drug treatment may be combined with surgery in cases with granulomatous lesions Figure 3. Microscopy exam of the nasal mucosa showing a lympho-
or scarring stenosis. Many antibiotics have been used to plasmacytic infiltrate with Mikulicz cel s, Russel bodies and congested treat rhinoscleroma. Streptomycin has severe side effects, and dilated blood vessels.
especially on the vestibular system; also resistance to this drug has developed in a number of countries. Tetracycline requires a prolonged course of treatment and also has sig-nificant adverse effects. Rifampicin provides good results Scleroma is a specific granulomatous infection that in the treatment of rhinoscleroma, but requires effective affects the nose and, less frequently, other structures of monitoring of toxicity. Trimethoprim-sulphamethoxazo- the respiratory tract. It usually affects people with lower le may be effective and is widely used in Third World social and economic status, and is associated with lack of countries. Recent studies have shown that the quinolones, hygiene and prolonged contact with infected patients3,9.
ciprofloxacin and fluoroquinolone readily penetrate the The disease progresses in three stages: (1) the ca- tissues and are clinically effective3.
tarrhal stage: patients have non-specific rhinitis symptoms that progress to fetid rhinorrhea, crusting and nasal obs- BRAZILIAN JOURNAL OF OTORHINOLARYNGOLOGY 72 (4) JULY/AUGUST 2006 http://www.rborl.org.br / e-mail: [email protected] Documento descargado de http://bjorl.elsevier.es el 07/10/2016. Copia para uso personal, se prohíbe la transmisión de este documento por cualquier medio o formato.
Scleroma is a better name for this clinical entity, 1. Granato L, Jorge JJ, Souza DG, França LCM. Rinoscleroma - Conside- as it affects both the nasal cavity and the remaining res- rações sobre um caso. RBM - Otorrinolaringologia 1977;43(1):1-11.
2. Hebra FV. Vever ein eigent huemliches neugebilde ander nase. Wien piratory tract. It is a rare condition in our country and frequently is difficult to diagnose. This is due to the dise- 3. Badia L, Lund VJ. A case of rhinoscleroma treated with ciprofloxacin. ase stages, where patients may present with non-specific J Laryngology & Otology 2001;115:220-2.
rhinitis symptoms, signs of granulomatous proliferation 4. Wilson WR, Montgomery WW. Infectious diseases of the paranasal sinuses. In: Paparella MM, Shumrick DA, Gluckman JL, Meyerhoff or scarring.
WL, eds. Otolaryngology, III: Head and Neck. 3rd ed. Philadelphia: When the disease progresses with proliferation, it Saunders 1991; 1843-60.
may simulate a tumor, as in our patient. At a later stage, 5. Rifai M. Laryngotracheal resection for post scleromatous laryngeal scarring may produce retraction in any part of the respi- stenosis. J Laryngol Otol 1989;103:935-58.
6. Shaw HJ. Martin H. Rhinoscleroma. A clinical perspective. J Laryngol ratory tract.
Otol 1961;1011-39.
Material should be adequate for histopathology. 7. Granato L, Mimica I, Mimica LMJ, et al. Rinoscleroma: apresentação A cell infiltrate containing lymphocytes and plasmocytes de um caso. Acta Awho 1985;4(2):47-50.
including Mikulicz cells and Russel bodies is highly sugges- 8. Lutz, A. Zur Kosmistik dês Rhinoskleroms, Monastsh F. prakt. Dermat tive of scleroma. The differential diagnosis should be made 9. Kim NR, Han J, Kwon TY. Nasal rhinoscleroma in a nonendemic with granulomatous diseases, leprosy and tuberculosis, all area: a case report. J Korem Med Sci 2003;18:455-8.
of which may be identified with specific histopathological 10. Al Razek AKA, Al Elasfour A. MR appearance of rhinoscleroma: article. Am J Neuroradiology 1999;20:575-8.
11. Becker TS, Shum TK, Waller TS, et al. Radiological aspects of rhinos- Isolating Klebsilela Rhinoescleromatis using special cleroma. Radiology 1981;141:433-8.
culture media is not always possible in these cases.
12. Al Serhani AM, Al Qahtani AS, Arafa M. Association of rhinoscleroma The correct diagnosis is given based on histopatho- with rhinosporidiosis. Rhinology 1998;36(1):43-5.
logical findings and the clinical history, which includes 13. Mikulicz J. Veber das rhinosclerom. Arch F Klin Chir 1877;20:485.
14. Von Frisch A. Zur aetiologie des rhinoskerons. Eien Méd Wschr information on living conditions and the work environ- BRAZILIAN JOURNAL OF OTORHINOLARYNGOLOGY 72 (4) JULY/AUGUST 2006 http://www.rborl.org.br / e-mail: [email protected]

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