Rhinoscleroma: A Chronic Granulomatous Condition of the Nose
Chronic
granulomatous
conditions of
nose
Rhinoscleroma
Rhinoscleroma is a chronic
granulomatous condition of
the nose and other
structures of the upper
respiratory tract.
It is a result of infection by
the bacterium Klebsiella
rhinoscleromatis.
Rhinoscleroma is contracted by
means of the direct inhalation of
droplets or contaminated
material.
The disease probably begins in
areas of epithelial transition such
as the vestibule of the nose, the
subglottic area of the larynx, or
the area between the
nasopharynx and oropharynx.
Rhinoscleroma usually affects
the nasal cavity, but lesions
associated with rhinoscleroma
may also affect the larynx;
nasopharynx; oral cavity;
paranasal sinuses; or soft
tissues of the lips, nose,
trachea, and bronchi
.
Epidemiology
It is endemic to regions of Africa
(Egypt, tropical areas), Southeast
Asia, Mexico, Central and South
America, and Central and Eastern
Europe.
Rhinoscleroma tends to affect females
somewhat more often than it does
males.
Typically, rhinoscleroma appears in
patients aged 10-30 years.
Disease may start as a single
nodule or in groups in nasal
cavity.
The lesions do not suppurate
or ulcerate
They heal by dense
cicatrization, almost cartilage
like hardness
Possible history findings
Nasal obstruction (most common
complaint)
Rhinorrhea
Epistaxis
Dysphagia
Nasal deformity
Anesthesia of the soft palate
Difficulty breathing that progresses to
stridor
Dysphonia
Anosmia
Pathological development
Diffuse stage resembling
atrophic rhinitis
Stage of localisation and
organisation
Stage of cicatrization
DD
Atrophic rhinitis
Tertiary syphilis
Lupus
Leprosy
Cancer
Treatment
Streptomycin
Tetracyclines
Chlorophenicol
Steroids
Locally
Systemically
Wegner’s
Granulomatosis
A condition characterized by
granulomatous inflammation
involving the respiratory tract
and necrotizing vasculitis
affecting small to medium
sized vessels.
The pathological hallmark is
the co-existence of vasculitis
and granulomas and
classically involves a triad of
airway, lungs and renal
disease.
Age and Sex
Significant number of
patients below 25 years of
age.
Younger patients present
with a generalized form.
Aetiology
Aetiology remains unknown.
Its inflammatory nature and
resemblance to polyarteritis
nodosa suggests that it
represents some form of
hypersensitivity reaction.
It might be related to inhaled
bacteria.
Clinical Features
Most patients start with minor ENT
symptoms
Variable degree of epistaxis
Nasal Obstruction
Bloody crusts
Destruction of intranasal structures
including septum may follow leading
eventually to nasal collapse.
Patients may complain of significant
facial pain
.
Clinical Features
Patients frequently complain
of progressive malaise,
pyrexia, weight loss and feel
very unwell.
Nose and paranasal sinuses
are involved in 80% patients.
Intranasal destruction of
cartilage and bone leads to
septal perforation.
Pulmonary symptoms
Cough
Haemoptysis
Pleuritic pain
Cavitation
Encapsulated lung
abscess
Renal Symptoms
Between 30% to 90%
patients develop renal
symptoms.
Microscopic haematuria
Segmental or diffuse
glomerulonephritis.
Ocular manifestations
Conjuctiviitis
Dacrocystitis
Corneal ulceration
Optic neuritis and retinal
artery occlusion.
Blindness unilateral or
bilateral
Otologic sympoms
Acute otitis media
Otitis media with effusion
Deafness
Otalgia
Both conductive and
sensorineural hearing loss
Diagnosis
cANCA test is positive in
95% of patients.
A full blood count
ESR
Renal Profile
Urine analysis
Biopsy from septum or turbinates
Vasculitis
Granulomas of epithelial
cell type
Multinucleated giant
cells
Mucosal
thickening
Bone
destruction
New bone
formation
Treatment
S
t
e
r
o
i
d
s
a
n
d
a
v
a
r
i
e
t
y
o
f
c
y
t
o
t
o
x
i
c
d
r
u
g
s
i
m
p
r
o
v
e
s
h
o
r
t
t
e
r
m
p
r
o
g
n
o
s
i
s
b
y
9
0
%
.
N
a
s
a
l
s
y
m
p
t
o
m
s
m
a
n
a
g
e
d
b
y
t
o
p
i
c
a
l
p
r
e
p
a
r
a
t
i
o
n
s
.
A
u
g
m
e
n
t
a
t
i
o
n
r
h
i
n
o
p
l
a
s
t
y
Septal Perforation
Causes
T
r
a
u
m
a
Surgical
Repeated cautery
Digital trauma
M
a
l
i
g
n
a
n
t
d
i
s
e
a
s
e
Malignant tumours
Malignant granuloma
C
h
r
o
n
i
c
i
n
f
e
c
t
i
o
n
s
Syphilis
Tuberculosis
Leprosy
P
o
i
s
o
n
s
Industrial
Cocaine addicts
I
d
i
o
p
a
t
h
i
c
Most are iatrogenic in origin
Repeated cautery of the septum
Occupational: Commonest cause is
penetration of the nasal mucosa by one
of the hexavalent forms of chromium.
Other causes include exposure to soda
ash, arsenic and its compounds, organic
compounds of mercury, cocaine and
snuff.
There are often four well
marked stages of
development:
Redness and congestion of
mucosa
Blenching and anaemia
Necrosis and development
of crusts
Final extension of crusts in
to cartilage and perforation
Symptoms
Most septal perforations are
asymptomatic.
Development of large crusts may
cause nasal obstruction
Separation of crusts may lead to
bleeding
Whistling noise
The larger the perforation more
symptoms it would produce
Treatment
Cure the causative disease process
Perforations never heal spontaneously
Less severe cases can be satisfactorily
controlled by nasal douching
Silastic Obturators can be used to close
large perforations up to 4 cm in diameter.
If obturators fail to deliver consider surgery
Perforations larger than 2 cm are difficult to
close
Rhinoscleroma is a chronic granulomatous condition of the nose caused by infection with the bacterium rhinoscleromatis. It primarily affects the nasal cavity but can also involve other respiratory tract structures. The disease is contracted through inhalation and exhibits distinct pathological stages. Common symptoms include nasal obstruction, rhinorrhea, and epistaxis. Rhinoscleroma is endemic in certain regions and tends to affect females more frequently.
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Presentation Transcript
Chronic granulomatous conditions of nose
Rhinoscleroma is a chronic granulomatous condition of the nose structures of respiratory tract. It is a result of infection by the bacterium rhinoscleromatis. and the other upper Klebsiella
Rhinoscleroma is contracted by means of the direct inhalation of droplets or material. The disease probably begins in areas of epithelial transition such as the vestibule of the nose, the subglottic area of the larynx, or the area nasopharynx and oropharynx. contaminated between the
Rhinoscleroma usually affects the nasal cavity, but lesions associated with rhinoscleroma may also affect the larynx; nasopharynx; paranasal sinuses; tissues of the trachea, and bronchi. oral cavity; or lips, soft nose,
Epidemiology It is endemic to regions of Africa (Egypt, tropical Asia, Mexico, Central America, and Central and Eastern Europe. Rhinoscleroma tends to affect females somewhat more often than it does males. Typically, rhinoscleroma appears in patients aged 10-30 years. areas), Southeast and South
Disease may start as a single nodule or in groups in nasal cavity. The lesions do not suppurate or ulcerate They heal by dense cicatrization, almost cartilage like hardness
Possible history findings Nasal obstruction (most common complaint) Rhinorrhea Epistaxis Dysphagia Nasal deformity Anesthesia of the soft palate Difficulty breathing that progresses to stridor Dysphonia Anosmia
Pathological development Diffuse stage resembling atrophic rhinitis Stage of localisation and organisation Stage of cicatrization
DD Atrophic rhinitis Tertiary syphilis Lupus Leprosy Cancer
Treatment Streptomycin Tetracyclines Chlorophenicol Steroids Locally Systemically
Wegners Granulomatosis
A condition characterized by granulomatous involving the respiratory tract and necrotizing affecting small sized vessels. The pathological hallmark is the co-existence of vasculitis and granulomas classically involves a triad of airway, lungs disease. inflammation vasculitis medium to and and renal
Age and Sex Significant patients below 25 years of age. Younger patients present with a generalized form. number of
Aetiology Aetiology remains unknown. Its inflammatory nature and resemblance nodosa suggests represents some hypersensitivity reaction. It might be related to inhaled bacteria. to polyarteritis that form it of
Clinical Features Most patients start with minor ENT symptoms Variable degree of epistaxis Nasal Obstruction Bloody crusts Destruction of intranasal structures including septum may follow leading eventually to nasal collapse. Patients may complain of significant facial pain.
Clinical Features Patients frequently complain of progressive pyrexia, weight loss and feel very unwell. Nose and paranasal sinuses are involved in 80% patients. Intranasal destruction cartilage and bone leads to septal perforation. malaise, of
Pulmonary symptoms Cough Haemoptysis Pleuritic pain Cavitation Encapsulated lung abscess
Renal Symptoms Between 30% to 90% patients develop renal symptoms. Microscopic haematuria Segmental or diffuse glomerulonephritis.
Ocular manifestations Conjuctiviitis Dacrocystitis Corneal ulceration Optic neuritis and retinal artery occlusion. Blindness unilateral or bilateral
Otologic sympoms Acute otitis media Otitis media with effusion Deafness Otalgia Both conductive and sensorineural hearing loss
Diagnosis cANCA test is positive in 95% of patients. A full blood count ESR Renal Profile Urine analysis
Biopsy from septum or turbinates Vasculitis Granulomas of epithelial cell type Multinucleated giant cells
Mucosal thickening Bone destruction New bone formation
Treatment Steroids and a variety of cytotoxic drugs improve short term prognosis by 90%. Nasal symptoms managed by topical preparations. Augmentation rhinoplasty
Causes Trauma Surgical Repeated cautery Digital trauma Malignant disease Malignant tumours Malignant granuloma Chronic infections Syphilis Tuberculosis Leprosy
Poisons Industrial Cocaine addicts Idiopathic Most are iatrogenic in origin Repeated cautery of the septum Occupational: penetration of the nasal mucosa by one of the hexavalent forms of chromium. Other causes include exposure to soda ash, arsenic and its compounds, organic compounds of mercury, snuff. Commonest cause is cocaine and
There are often four well marked stages of development: Redness and congestion of mucosa Blenching and anaemia Necrosis and development of crusts Final extension of crusts in to cartilage and perforation
Symptoms Most asymptomatic. Development of large crusts may cause nasal obstruction Separation of crusts may lead to bleeding Whistling noise The larger the symptoms it would produce septal perforations are perforation more
Treatment Cure the causative disease process Perforations never heal spontaneously Less severe cases can be satisfactorily controlled by nasal douching Silastic Obturators can be used to close large perforations up to 4 cm in diameter. If obturators fail to deliver consider surgery Perforations larger than 2 cm are difficult to close
