Laryngeal Presentations Of Rhinoscleroma
Author 1: Osama G. Abdel-Naby Awad, MD.
Otolaryngology, Head and Neck department. Minia University,Minia, Egypt.
Author 2: Abdel-Rahiem A. Abdel-Keriem, MD.
Otolaryngology, Head and Neck department. Minia University,Minia, Egypt.
Author 3: Effat A. Zaki, MD.
Speech and phoniatrics unit, Otolaryngology, Head and Neck
department.
Minia University,Minia, Egypt.
Name of author responsible for correspondence and proofs:
Osama G. Abdel-Naby Awad, MD.
Otolaryngology, Head and Neck department. Minia University,Minia, Egypt.
122 Kornish El-Neel Street, Minia City, Minia, Egypt.
Zip code: 61111, Fax: 02-086232505.
e-mail : omarsmsm2014@yahoo.com
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Abstract
Background: Rhinoscleroma is a chronic granulomatous disease of the upper
respiratory tract caused by Klebesiella rhinoscleromatis. . It is considered endemic in
Egypt. The nasal mucosa represents the primary region of occurrence. The disease can
potentially spread to involve the larynx and trachea causing dysphonia, stridor and
airway obstruction.
Objective: To describe the various laryngeal presentations of Rhinoscleroma in our
endemic area.
Methods: The study included 50 patients with positive histopathological and
bacteriological examination for Rhinoscleroma presented with dysphonia and the
other manifestations of Rhinoscleroma.
Results: 12% only of the patients had the typical subglottic narrowing with sticky
greenish disharge and subglottic membrane. Other patients presented with atypical
laryngeal presentations e.g. (unhealthy vocal folds, ventricular fold hypertrophy,
suproglottic sticky greenish discharge).
Conclusion: Rhinoscleroma can present with atypical laryngeal presentations which
should be kept in mind to avoid misdiagnosis.
Keywords: Rhinoscleroma, Histopathology; Larynx; Hoarseness.
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Introduction
Rhinoscleroma is a chronic, progressive, granulomatous infectious disease of
the upper respiratory tract. It was 1st reported in Europe, but it is now rarely diagnosed
on that continent. The term RS was 1st coined in 1870 by Von Hebra 1, who described
a nasal lesion that they classified as a form of sarcoma. In 1877, Mikulicz 2 described
the histological features of this disease in detail and established its non neoplastic
inflammatory nature. Von Frisch3 identified the causal agent of this lesion in 1882 as a
gram negative coccobacillus, now known as klebesiella rhinoscleromatis.
Rhinoscleroma is found predominantly in rural areas and is commoner where
socio-economic conditions are poor. Acquisition of the disease is facilitated by
crowding, poor hygiene and poor nutrition. Females are more frequently affected than
males and disease tends to present in the second and third decades of life. There is
also suggestion of iron deficiency may predispose to the disease acquisition.4
Rhinoscleroma affects most areas of the respiratory tract. The nose is affected
in (95%-100%) of cases and the pharynx in up to half, other affected areas include the
Eustachian tubes,5 sinuses,6 mouth,7 orbit,8 larynx,9 trachea and bronchi.10 The skin in
proximity to the affected mucosa e.g. upper lip or nose may be involved and there is a
report of back lesions.11 Spread by extension to the brain is also possible.12 In two
patients with Acquired Immunodeficiency Syndrome and Rhinoscleroma, there was
no evidence of dissemination despite low CD4 lymphocyte counts.13
The disease usually arises at the junction between epithelial surfaces; for
example, in the nose It occurs at the junction between the stratified sequamous
epithelium of the anterior nares and the deeper columnar ciliated epithelium. Iron
deficiency can lead to sequamous metaplasia and this might be an explanation for the
association with poor nutrition and the higher incidence in women of child-bearing
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age.
Rhinoscleroma is divided clinically and pathologically into three stages:
atrophic, granulomatous and sclerotic. In the atrophic stage (sometimes called
ozaena), there is foul-smelling purulent nasal discharge which persists for months.
There may be unilateral or bilateral nasal obstruction, the mucosa may be atrophic. In
the granulomatous stage, the patient complains of epistaxis and other problems
depending on the other areas affected, e.g. hoarseness with laryngeal involvement.
Nasopharynx and pharynx are involved in most of cases which present with shrunken
and deformed "Gothic palate" and nasopharyngeal stenosis. Most cases are diagnosed
in this stage, when the lesion appears as a bluish-red rubbery granuloma, indurated
granulomatous mass. In the fibrotic stage there is nasal deformity and may be
destruction of the bone.9
On histological examination there may be atrophy or hyperplasia, although the
latter is more common.7 The hyperplasia is termed pseudo-epitheliomatous
hyperplasia and contains an infiltrate of chronic inflammatory cells, monocytes,
lymphocytes and histiocytes (macrophages) with numerous vacuoles containing
viable or non-viable bacteria known as (Mikulicz cells) which can be found beneath
the basal lamina together with Russell bodies which are eosinophillic structures
within the cytoplasm of plasma cells. In the sclerotic stage there is increasing
deformity and stenosis, with granulomatous areas surrounded by fibrotic tissue. At
this stage Russell bodies and Mikulicz cells are diificult to demonstrate.2,9,14,
Rhinoscleroma of the larynx is uncommon and usually occurs in the subglottic
region, with the major deleterious effect is the airway obstruction which usually
requires endoscopic treatment.15 The aim of this study was to describe the laryngeal
presentations in patients with clinically diagnosed Rhinoscleroma in our endemic area
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during the daily practice.
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Materials and methods
Our prospective study included 50 patients presented to our otolaryngology
department, Minia University hospital, Minia, Egypt, from Jan.2103 to Jan.2014.
These patients presented with various clinical manifestations of RS. Over the study
period, 85 patients were presented to our outpatient clinic with manifestations of
Rhinoscleroma in our highly endemic area. We selected only those 50 patients who
had dysphonia as their main concern.
All patients were subjected to full history taking, with special emphasis on
contact with diseased relatives, the duration of their main complaint, other nasal,
pharyngeal or laryngeal symptoms, the history of previous medications, the history of
previous head and neck operations, history of gastro-esophageal reflux disease,
history of common causes of immunodeficiency such as human immunodeficiency
virus, long term steroid therapy, diabetes mellitus, or history of immunosuppressive
therapy.
Thorough otorhinolaryngeal examination was done for all the patients
including nasal endoscopic examination. Biopsies were taken from each nasal cavity
separately from the muco-cutaneous junction under local anesthesia for
histopathological and microbiological examination. Each patient had computed
tomography (CT) scan for the neck region.
For each patient fibroscopic laryngeal examination was done followed by
endoscopic examination of the upper respiratory tract under general anesthesia and
other biopsies were taken from any suspected unhealthy lesions in the larynx, these
biopsies were also examined histologically and bacteriogically. The bacteriological
evaluation was done through culture in MacConky agar and bacteriological
identification by using gram staining. The pathological examination of the biopsies
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was done by light microscope.
We considered Rhinoscleroma as a definite diagnosis with the presence of
positive bacteriological culture of the klebesiella Rhinoscleromatis and the typical
histopathological picture in the form of pseudo-epitheliomatous hyperplasia with an
infiltrate of chronic inflammatory cells, monocytes, lymphocytes, Mikulicz cells and
Russell bodies, with no pathological evidence of malignancy. These requirements of
diagnosis was a must for both the nasal and laryngeal biopsies.
In addition, laboratory screening for diabetes mellitus, Human
immunodeficiency virus infection, complete blood count and routine pre-anesthetic
assessment were performed for all patients. Patients in the study received intermittent
medications in the form of variable antibiotics (Rifampicin, streptomycin and
ciprofloxacin), alkaline nasal douching and multivitamins for short duration ranged
from 1 to 6 weeks before the inclusion in the study.
We excluded from the study patients with negative nasal or laryngeal
bacteriological cultures or with atypical histopathological results, even if the patient
had the characteristic clinical manifestations of Rhinoscleroma. Also we excluded
patients with gastro-esophageal reflux disease and patients with previous nasal or
laryngeal surgeries. The study was approved by the Institutional Review Board at the
Minia University. Because the study involved no deviation from existing standard
therapy for these patients and no new drugs, individual consent was not required by
the board. Statistical analysis was done using the Statistical Package for the Social
Science software programe (SPSS, Inc., Chicago, IL).
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Results and analysis
Our study included 50 patients including 17 (34%) male patients and 33 (66%)
female patients, their ages ranged from 14-48 years old, with mean age 33 years.
Fifteen patients (30%) were farmers, 30 patients (60%) were house wives, 3 patients
(6%) were students and 2 patients (4%) were drivers. Forty eight patients (96%) live
in rural areas and 2 patients (4%) live in urban areas.
Ten patients (20%) were cigarette smokers, 8 patients (16%) had diabetes
mellitus, 48 patients (96%) had microcytic hypochromic anemia with marked iron
deficiency. Fifteen patients (30%) had positive family history of Rhinoscleroma in
one of their house hold relatives.
The duration of the patients complaint ranged from 2 to 10 years with a mean
about 4 years. The duration of dysphonia ranged from 6 to 18 months with a mean
about 12 months.
Presentation of nasal lesions:
All the patients in study had nasal obstruction, 45 patients (90%) had bilateral
nasal crustations, 43 patients (85%) had bilateral greenish nasal discharge, 25
patients (50%) had total anosmia, 25 patients (50%) had hyposmia with 5 patients
(10 %) had cacosmia. Twenty patients (40%) had epistaxis either unilaterally or
bilaterally. We didn't report any patients with atypical nasal manifestations e.g.
(nasolacrimal involvement or external swellings). Also we didn't report any
patients with primary laryngoscleroma ( i.e. laryngeal manifestations in the
absence of nasal lesions).
On endoscopic nasal examination the following findings were reported:
- Forty three patients (86%) had bilateral roomy nose with thick greenish
discharge (atrophic stage). Six patients (12%) had bilateral roomy nose with
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granular surface (granuomatous stage) and 1 patient (2%) had intranasal adhesions
with external nasal deformity (Fibrotic stage).
- 20 patients (40%) had pharyngeal involvement with the characteristic shrunken and
deformed soft palate "Gothic palate".
Presentation of laryngeal lesions:
All patients in the study had dysphonia with different grades, 2 patients (4%) had mild
to moderate stridor and respiratory distress on exertion.
On fibroptic and laryngeal examination under general anesthesia the following
findings were reported:
- Twenty five patients (50%) had congested and unhealthy vocal folds mucosa with
intact focal folds mobility (Fig. 1).
- Fourteen patients (28 %) had congested unhealthy vocal folds mucosa with
subglottic and suproglottic sticky greenish disharge with intact focal folds mobility
(Fig.2).
- Five patients (10%) had ventricular fold hypertrophy with irregular unhealthy
mucosa with intact focal folds mobility (Fig.3).
- Four patients (8%) had subglottic narrowing with sticky greenish disharge (Fig.4).
- Tow patients (4%) had subglottic narrowing with thick membrane (Fig.5). These 2
patients were females and had the preoperative stridor and respiratory distress. One of
these 2 patients developed more distress during the recovery from the anesthesia
which necessitates urgent trachestomy. CT neck findings were consistent with the
endoscopic findings. We didn't report any of our patients with tracheal involvement.
Table 1 correlates between the nasal stages and the laryngeal findings.
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Table I. Correlation between the nasal stages, laryngeal findings and duration of
dysphonia in the study patients.
Laryngeal lesions
# of
Nasal stage
patients
congested and unhealthy vocal folds
Duration of
dysphonia
25
25 patients with atrophic stage.
6-10 months
14
14 patients with atrophic stage.
8-12 months
5
- 4 patients with atrophic stage.
10-15 months
mucosa with intact focal folds mobility.
congested
mucosa
unhealthy
with
vocal
folds
subglottic
and
suproglottic sticky greenish disharge
with intact focal folds mobility.
ventricular
fold
hypertrophy
with
irregular unhealthy mucosa with intact
-1 patient with granulomatous
focal folds mobility.
stage.
subglottic
narrowing
with
sticky
4
4 patients with graulomatous stage. 10-18 months
2
- 1 patient with graulomatous 12-18 months
greenish disharge.
Subglottic
narrowing
with
thick
membrane and with limited bilateral vocal
stage.
folds mobility.
- 1 patient with sclerotic stage.
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Fig.1
Congested and unhealthy vocal folds mucosa.
Fig.2
Congested unhealthy vocal folds mucosa with subglottic and suproglottic sticky
greenish disharge.
Fig. 3
Ventricular fold hypertrophy with irregular unhealthy mucosa.
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Fig. 4
Subglottic narrowing with sticky greenish disharge
Fig.5
Subglottic narrowing with thick membrane
Discussion
Rhinoscleroma is a chronic specific granulomatous disease. The majority of
1
cases affect the upper airways particularly the nose. Rhinoscleroma is one of the most
common granulomatous diseases among the Egyptian population. This graulomatous
disease occurs sporadically in western Europe usually in immigrant populations
arriving from contries where the disease is endemic. The disease is transmitted by air
and humans are the only identified host.16 Poor hygiene, malnutrition and crowded
living conditions are believed to increase the potential for infection and
transmission.17 Patients are immunocompetent except in ineffective phagocytosis by
the Mikulicz histocytosis.17-22 The lesions initially start at the muco-cutaneous
junction in the nasal vestibule then spread to nasopharynx. The epithelial transition at
the subglottic are is the next area of affection.17 The aim of this study was to study the
possible laryngeal presentations of Rhinoscleroma.
The age of patients in our study ranged from 14-48 years with mean age 33
years, de Pontual 23 found that the median patients age at diagnosis of Rhinoscleroma
to be 35.7 years with range from 5-72 years. This wide range can be attributed to the
very long contact time needed and the long duration of the complaints, misdiagnosis
of many patients by general practioners or non compliance of those poor patients and
neglicance of taking medications for long duration.24
In our study, females were affected more significantly than males, this result
matches the results of Hart and Rao who suggested the association of Rhinoscleroma
with poor nutrition as in rural areas with higher incidence in women of childbearing
age.25
Cellular immunity may be impaired in patients with scleroma, however, their
humoral immunity is preserved, otherwise patients are immunocompetent in every
regard except for the ineffective phagocytosis.17-22 In our study all the patients were
immunocompetent by exclusion of the common causes of immunodeficiency such as
1
human immunodeficiency virus infection, history of long term steroids therapy or
history of immunosuppressive therapy, except for the presence of diabetes mellitus in
8 patients, which didn't significantly affect the presentation. The duration of patients
complained ranged from 2 to10 years , de Pontual et al.23 reported that the probable
duration of exposure to klebesiella rhinoscleromatis in endemic areas varied widely
from 0 to 28 years and the clinical features and the outcome also varied considerably
among cases.
Nasal involvement occurred in all the study patients, also the Rhinoscleroma
evidence in the larynx in these patients were positive, only 40% of patients had
pharyngeal involvement. This important finding in our study opens a new aera in the
research of how this scleroma spread through the upper respiratory system. Gamea et
al. 26 observed pharyngeal involvement in one third of the cases and mentioned that
the nasopharyngeal and oropharyngeal scleroma develops as a result of contiguous
extension along the palate, lateral wall of nasopharynx and faucial pillars. However,
primary involvement of pharynx and larynx has been observed without any
demonstrable lesion in nose.23
Only 6 patients (12%) in our study presented with typical presentation of
laryngoscleroma in the form of subglottic involvement. Other patients in the study
presented with atypical laryngeal involvement, which raised the possibility of other
laryngeal diseases, however, the histopathological and bacteriological results for
Rhinoscleroma were positive. Razek27 in a recent study addressed the CT findings of
laryngo-tracheal scleroma and stated that it appears as symmetrical or asymmetrical
circumferential subglottic stenosis with extension into trachea and the bronchi, it may
appear as a thickened epiglottis, aryepiglottic fold and vocal cords, and, rarely, there
is concentric transglottic narrowing. In our study we didn't report cases with tracheal
1
involvement. In a more recent study, Razek et al.28 used MRI in cases with
laryngoscleroma, and they found that laryngoscleroma was seen more commonly in
the subglottic, but can occur at the suproglottic region, and they found that
laryngoscleroma at granulomatous stage appeared as diffuse circumferential soft
tissue mass with high or mixed signal intensity on T2- weighted images, and
laryngoscleroma at fibrotic stage was seen as a diffuse asymmetrical circumferential
thickening of the subglottic region with low signal intensity on T2-weighted images.
Dolci et al.29 in his report revealed that respiratory scleroma affected the larynx in
40%, and the principal findings were glottic and subglottic stenosis.
This atypical presentation of scleroma is not confined to the larynx, Fawaz et
al.25 reported 18 patients with different atypical presentations of Rhinoscleroma in the
form of intraorbital extension, check swelling, Potts puffy tumor, cutaneous
involvement and unilateral nasal affection.
It was also clear from our study that patients with early nasal stages (atrophic
and granulomatous stages) had less severe laryngeal lesions in comparison with
patients with late nasal stages ( sclerotic stage) who had advanced laryngeal lesions
in the form of subglottic narrowing with thick membrane and respiratory distress.
This very important finding also opens a new era for studying the correlation between
the nasal and laryngeal stages in a wider range of patients and emphasize on the role
of early diagnosis and adequate treatment of this granuloma which may help to
prevent a possible serious complications. Our study, up to our knowledge, is the
largest series studying patients with laryngeal scleroma and the 1st to correlate
between nasal and laryngeal presentations in a hope to alert physians about this
important endemic disease.
Conclusion
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From our study, we can conclude that Rhinoscleroma in endemic areas can
have atypical laryngeal presentations and the advanced nasal stages are usually
accompanied by more affection of the larynx. In order to avoid misdiagnosis and
delayed treatment in endemic or non-endemic areas, it is important to keep in mind
these atypical manifestations of laryngeal scleroma.
Acknowledgments: None.
1
Financial Support : None.
Conflict of interest: None
Ethical Standards: The authors assert that all procedures contributing to this work
comply with the ethical standards of the relevant national and institutional guidelines
on human experimentation (Osama Awad) and with the Helsinki Declaration of 1975,
as reviewed in 2008". And the authors assert that all procedures contributing to this
work comply with the ethical standards of the relevant national and institutional
guides on the care and use of laboratory animals (Osama Awad)."
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1
Summary

The observed prevalence of glottic and suproglottic manifestations of
Rhinoscleroma was higher than in previous reports.

The results point to that advanced laryngeal manifestations of Rhinoscleroma
were associated with late nasal stages of the disease.

The observed laryngeal manifestations of Rhinoscleroma in our endemic
area differ than other reports from non-endemic areas.
1
Tables Legend
Table I: Correlation between the nasal and laryngeal findings in the study patients
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