Granulomatous Diseases
of nose and PNS
Presenter – Dr Pulkit Agarwal
Moderator – Dr K.P.Basavaraju
What is Granuloma ?
 Immune system tries to wall off foreign substances
but fails
 Bacteria, Fungi, keratin, suture material.
 Histopathologically – Chronic inflammation
• Epitheloid cells
• Multinucleate giant cells
• Lymphocytes & fibroblasts
 Necrosis and Vasculitis.
Granuloma as misnomer
“Granuloma" loosely to mean "a small nodule".
Vocal cord granuloma
Pyogenic granuloma
Granulation tissue
Intubation granuloma
Pulmonary hyalinizing granuloma i.e. keloid-like
fibrosis in the lung
 Radiologists - calcified nodule on X-ray or CT
scan of the chest.
 Most accurate use – Pathologists who observe
stained tissue under microscope.
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Tuberculosis :
 Nasal Mucosa involvement rare.
 Portal – Inhalation (commonest), ingestion,
inoculation.
 Asso with Primary pulm. Tuberculosis
 M. tuberculosis and M. bovis - main pathogens.
 Most common affection of nose – Lupus Vulgaris
 Macroscopic appearance:
• Nodular form (Lupus)
• Ulcerative form
• Sinus granuloma
 Indolent and chronic from of tb infection affecting
the skin and mucous membrane of nose.
 F:M = 2:1 (young adults)
 Temperate Climate disease
 Starts at Vestibule, extends to adjoining skin and
mucosa with mucocutaneous junction as MC
location.
 Pathogenesis – Direct dermal inoculation (patient
having immunity to infection due to previous
exposure)
 Scarring of Vestibule common in later stages
 HPE – Shows tuberculous granulomas, RE cells at
centre which soon necrose & coalesce,
surrounded by ring of living RE cells.
 Around it is ring of Lymphocytes, plasma cells &
fibroblasts.
 Multinucleate giant cells scattered throughout
Symptoms :
Nasal discharge
Nasal obstruction
Presence of non foul smelling crusts
Epistaxis
Evening rise of temperature
Mild fetor if ulcerated
Pain is unusual
Apple jelly nodules (Early lesions) - reddish firm
nodules at mucocutaneous junction of nasal septum.
 May later coaslesce & break down to form characteristic
ulcers with a pale granular base and undermined
edges
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Diagnostic Features :
 Blanching (glass slide)
 Bacterial examination
 Biopsy (diagnostic)
 Culture - Lowenstein-Jensen slope
 Recent - BACTEC
Complications:
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Pulmonary tuberculosis
Corneal ulceration
Dacryocystitis
Nasopharyngeal lupus
Lupus of face
Atrophic rhinitis
Development of epithelioma (rare sequlae)
Sudden increase in size, hardness of the node in an
elderly with increasing tendency to bleed should
arouse the suspicion of epithelioma formation.
Ulcerative Form
 Ulcers are commonly found on the anterior part of
the cartilagenous nasal septum, inferior turbinate
and anterior choanae.
 Ulceration  Fibrosis  Distortion of Nasal Alae
 If turbinates are involved extensively, the lining
ciliated columnar epithelium is not renewed
leading to secondary atrophic rhinitis.
 Spread usually occurring in a backward direction
from the primary site
 Only cartilagenous septum involvement so no
sinking of nasal bridge.
Sinus Granuloma
 Isolated sinus involvement may occur without any signs
or symptoms in the nose.
 Usually presented with a diffuse soft tissue swelling
and multiple discharging sinuses in the supraorbital
region secondary to osteomyelitic involvement of the
frontal bone.
 The computed tomography (CT) and magnetic
resonance imaging (MRI) characteristics are
nonspecific and demonstrate a soft tissue mass with or
without bone destruction.
 Inv. of the orbit & its nerves may occur.
 Diagnosis by an external or endoscopic biopsy.
Specific Investigations
 Montaux test
 AMTD (Amplified Mycobacterium Tuberculosis
Detection)
 PCR
 Fluorescence microscopy
Treatment
 Antituberculous drugs
 Short Course Chemotherapy :
 Extends over 6-9 months and consists of
Rifampicin, INH, Ethambutol and Pyrizinamide
 DOTS :
 Initial Phase - 2 mnths - HRZE
 Rifampicin (R) - 450mg
 INH
(H) - 600mg
 Ethambutol (E) - 1200mg
 Pyrizinamide (Z) - 1500mg
Treatment
 Maintainence treatment (4 months) - (HR)3 (thrice
weekly under observation)
 Vit D supplementation - hastens sputum culture
conversion (only in vit D deficient cases)
 Surgical correction
Leprosy
 Caused by M. Leprae which is morphologically
similar to M. Tuberculi
 Non culturable, inoculated into experimental
animals
 Based on the host's immunological status and
clinical, histological and microbiological features
leprosy divided into types.
 Types of leprosy :
Tuberculoid leprosy:
 Immunocompetent patients
 Solitary lesions (anesthetic patches)
 Involvement of one / more related sensory or
motor nerves.
 Nasal Vestibule involved without mucosa
involvement
 Isolated cranial nerve palsies (V and VIIth) have
also been documented
Lepromatous Leprosy :
 Least immunological resistance to the organism.
 Diffuse infiltration of skin, nerves and mucosal
surfaces.
 Cutaneous infiltration is more common over the
edges of pinna, chin, nose and eyebrows.
 Highly infectious nasal discharge.
Signs and Symptoms
 Earliest sign - nodular thickening of nasal mucosa
 Crust formation
 Nasal obstruction (out of proportion)
 Sero sanguineous discharge
Nodules are paler than the surrounding mucosa with
a yellowish tinge.
Commonly begin at the anterior end of inferior
turbinate, progresses to gross inflammation of
nasal mucosa.
 Both the bony and cartilaginous portions of nasal
septum are destroyed due to perichondritis and
periosteitis (nasal bridge collapse)
 Anterior nasal spine destruction seen
 Radiographs showing the absence / erosion of
anterior nasal spine are virtually diagnostic of
Lepromatous leprosy
 Hyposmia is seen in 40%
 Nasal mucosa scraping microscopy shows typical
cigar pattern Lepra bacilli
Borderline leprosy
 Poor immunological resistance
 May progress to lepromatous leprosy
 Skin lesions are more numerous and are seen
around eyes, nose and mouth
 In pure borderline lesions - nasal mucosa
involvement is not seen
 Nasal mucosal inv suggests progression to LL.
Diagnosis
 Early diagnosis is essential since nasal discharge
is principle route of transmission of the disease
 Clinical findings of anaes. on the skin, thickened
peripheral nerves and skin lesions, &
supplemented by bacteriological examination.
 Confirmed by dem. of M. leprae on microscopy of
the nasal discharge or scrapings of nasal
mucosa, or histopathology of nasal mucosa.
 Skin biopsy in tuberculoid leprosy
Treatment
 Dapsone reduces the bacilli count of nasal discharge to
zero or near zero within a couple of months BUT
increased resistance
 Modern drugs like Rifampicin and Clofazimine can
reduce the bacilli count to zero within 10 days
 Triple therapy:
 Rifampicin
– 600 mg on first two days of a month
taken before breakfast
 Clofazimine
– 100 mg on alternate days for three
times a week
 Dapsone
– 100 mg a day
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Rifampicin stopped after 3 mnths
Rest 2 continued till 9 mnths
Intranasal Rifampicin much faster than oral route
Nasal douching for removal of crusts
Betnovate (1 part) in Unguentum (2 parts) has
been used with good results
syphilis
 Nasal syphilis an occur at any age
 Uncommon as signs symptoms difficult to elicit in early
stages especially if antibiotics given
 Causative org. – Treponema Pallidum(Spirochete)
 Pathogenesis - The organisms usually reside and
multiply in the perivascular lymphatics of the blood
vessel wall.
 Local host reaction results in an infiltration by
polymorphs, activated lymphocytes (CD4+ and CD8+),
plasma cells and histiocytes which are typical of syphilis.
 The resultant endarteritis of small blood vessels
with secondary hypertrophic changes in the
endothelium, may lead to endarteritis obliterans
and luminal obliteration.
 HPE - Diagnosis is purely histopathological
 Characterized by oedema, stromal infiltration with
lymphocytes, plasma cells and endothelial cells
 Perivascular cuffing by these cells and endarteritis
will cause a reduction in the lumen of blood
vessels causing necrosis and ulceration
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Nasal syphilis can be classified into:
Primary syphilis
Secondary syphilis
Tertiary syphilis
 Primary syphilis :
 Also known as Chancre, seen at external nose or
inside the vestibule.
 Appears as a hard, non painful ulcerated papule
always associated with enlarged, rubbery, and non
tender lymphadenopathy
 A sore or chancre develops at the site of
inoculation 10-90 days (average 21 days)
following infection
 These lesions undergo spontaneous resolution
within 6-10 weeks
 Should be differentiated from malignant
neoplasms and furunculosis
 Malignant neoplasms – affect older ages, relentless
progression
 Furunculosis – Painful condition, progresses to
suppuration.
 Treatment
 Anti syphilitic Rx - i.m. penicillin.
 The chancre may be cleansed with 1:2000 solution of per
chloride of mercury and the surface smeared with 2%
yellow mercuric oxide ointment.
 The following points should be borne in mind :
 Cultures from the surface of the lesion will always be
negative
 Smears when examined under dark ground
illumination will show the spirochete Treponema
palladium
 Serological tests for syphilis may be positive –
VDRL, TPHA, FTA-ABS. (The VDRL is a
nontreponemal test and is relatively nonspecific
but becomes positive early (four to five weeks)
and correlates well with disease activity.)
 A biopsy from suspicious lesion may confirm the
diagnosis
 Secondary Syphilis
 Most infectious of all the three stages
 Symptoms 6-10 weeks after inoculation
 Secondary syphilis is rarely recognized in the
nose, as mucous patches hardly ever occur
on such a thin, attenuated mucous
membrane.
 The symptoms include:
 Simple catarrhal rhinitis (persistent)
 Crusting & fissuring of nasal vestibule
 Other secondary lesions like mucous patches in
the pharynx are also seen
 Roseolar / papular skin rashes
 Shotty enlarged non tender lymph nodes.
 The scar of the primary lesion in the genitals or
elsewhere may be visible.
 Serological tests for syphilis are positive.
 Dark-field examination of moist or intentionally
abraided dry lesions may demonstrate the
organism.
 Lymph node aspirates or tissue specimens
(lymph nodes, liver, skin or mucous membrane)
may also be examined by silver stains or
immunofluorescence for detection of
spirochaetes
 These patients respond to antisyphilitic drugs.
Tertiary Syphilis :
 Only 1/3rd of secondary syphilis cases progress to show
clinical manifestations of tertiary syphilis.
 This stage is commonly encountered in the nose.
 Lesion is also known as gumma.
 This lesion invades the mucous membrane, periosteum
and bone.
 The bony portion of the nasal septum is frequently
affected causing septal perforation.
Rarely the following portions of the nose can also
be involved :
 Lateral nasal wall
 Frontal sinus
 Nasal bones
 Floor of the nose
Symptoms include:
1. Pain / headache (worse during night)
2. Swelling / obstruction of nose - swelling may be
diffuse / localized associated with offensive discharge,
bleeding and crusting of the nose
3. Olfactory acuity diminishes
4. Perforation of bony portion of nasal septum associated
with collapse of the bridge of the nose can cause
structural damage to the nasal architecture
5. There may also be associated secondary atrophic
rhinitis
Nasal complications of gumma:
1. Secondary infections with pyogenic organisms
2. Sequestration
3. Perforation of bony portion of nasal septum, palate or
nasal walls
4. Collapse of bridge of nose with deformity of nose
5. Scarring / stenosis of nasal passages
6. Atrophic rhinitis
7.Intracranial complications due to involvement of
meninges
Treatment :
1. DOC for all stages – parentral Penicillin
2. Alkaline nasal douches one to three times a day.
3. Local yellow mercury oxide ointment. Dilute
mercuric nitrate ointment should be applied freely to
the nasal vestibules.
4. Atrophic rhinitis and deformity may persist after the
disease is cured and this may need further
surgery.
Congenital syphilis “Snuffles”: :
 Any of the lesions of secondary and tertiary forms of
syphilis of nose can occur.
 Classically begin during the 3rd week of life.
 May appear around 3rd month also
 At First – simple catarrhal rhinitis.
 Later becomes purulent with sec.
fissuring and excoriation of the
nasal vestibule and upper lip.
 Gummatous and destructive lesions occur
most commonly at puberty.
 Mucous membrane, periosteum and bone may
all be affected.
 Other stigmata of syphilis, particularly
Hutchinson's incisors and Moon's molars,
interstitial keratitis, corneal opacities and
sensorineural deafness may be present, and
there may be a family history of syphilis,
miscarriages and still births.
 Serologic tests for syphilis will invariably be
negative
 Treatment :
 In snuffles, the airway must be restored for suckling
 The nasal discharge is removed by gentle suction
and irrigation and the use of local drops of 0.5
percent ephedrine solution or simple normal saline,
and by hyper extending the head before feeding
 In the tertiary forms, simple nasal toilet by
syringing with isotonic alkaline douche solution
will remove the crusts and discharge, and yellow
mercuric oxide ointment may be applied frequently
to the nasal vestibules
Yaws (framboesia)
 Closely resembles syphilis in its pathology.
 Causative organism : Treponema Pertenue
 Morphologically this organism is indistinguishable from
Treponema pallidum.
 Transmission : Is by direct extra genital contact. It has a
high incidence in infancy and childhood.
 Clinical features :
 Primary, secondary and tertiary stages occur as in syphilis
 Yaws principally affects the skin.
 Mucous membrane are usually spared but for the
mucocutaneous junctions.
 Nasal lesions are very rare and do not differ in
appearance from that of syphilis.
 Advance nasal lesions are associated with extensive
destruction of the nose, palate.
 Destruction also may involve the whole of the maxilla,
face and pharynx.
 Serologic tests for syphilis is positive.
 These lesions characteristically respond to
conventional antisyphilitic treatment.
Rhinoscleroma (scleroma)
 Progressive granulomatous disease commencing in the
nose and later extending into the nasopharynx and
oropharynx, the larynx and sometimes the trachea
and bronchi.
 Laryngeal involvement may occur in almost half the
cases and the disease is perhaps better designated as
respiratory scleroma, rather than rhinoscleroma.
 Aetiology is multifactorial and usually seen in low socio
economic status and poor domestic hygiene
Pathology :
 Caused by gram negative bacilli Klebsiella
rhinoscleromatis / Frisch bacilli / diplo bacillus.
 F>M, any age
 This organism could be a secondary invader following
a viral infection.
 Resides intracellularly and can be difficult to isolate
in the laboratory.
 The characteristic histological features include
granulomatous tissue infiltrates in the submucosa,
characterized by the presence of plasma cells,
lymphocytes and eosinophils.
 Besides, there are scattered large foam cells (Mikulicz
cells) which have a central nucleus and a vacuolated
cytoplasm containing frisch bacilli and Russel bodies.
 The Mikulicz cells are transformed macro phages which
have ingested the bacillus, but the bacillus being
resistant to digestion by the macrophage persists
intracellularly.
 The Russel bodies resemble plasma cells with an
eccentric nucleus and deep eosin staining cytoplasm.
 Histochemical studies have indicated a high content of
mucopolysaccharides around the walls of the bacillus
 it is hypothesized that this may be responsible for the
protection afforded to the organism from antibiotics and
the hosts antibodies
Clinical features :
Three stages –
1. The atrophic stage
The features resemble that of atrophic rhinitis, including
crust formation and a foul smelling discharge
(carpenter's glue)
2. Granulomatous or proliferative (or nodular) stage.
Nonulcerative nodules develop which are at first bluish
red and rubbery and later become paler and
indurated.
These nodules never break down but fibrose and
progressively decrease in size. Regional lymph node
involvement is extremely infrequent.
3. Cicatrizing stage
 Adhesions and stenosis distort the normal anatomy
 The disease may extend to involve the lacrimal sac,
maxillary sinus, nasopharynx, hard palate, trachea
and main bronchi
 Bone involvement has also been reported
 The shape and contour of the nose changes causing a
condition known as “Tapir’s nose”(woody feeling on
palpation of nose)
 Lymphatic spread is uncommon because of extensive
fibrous tissue deposition
 This deposition blocks the lymphatics. Occasionally,
malignant change has been reported
Gothic Sign - because of fibrosis the pharyngeal
wall is pulled up and resembles the top of a
church.
Complications :
1.
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3.
4.
5.
External nose deformity
Vestibular stenosis
Cicatrization of soft palate
Nasal regurgitation
Tracheal stenosis
Diagnosis :
Levin test (complement fixation test) :
High titres of antibodies against K. Rhinoscleromatis has
been demonstrated.
This indicates humoral immunity to be intact in these
patients
Diagnosis in the atrophic stage is extremely difficult
and the disease is usually recognized only in the
granulomatous or cicatrizing stage
MRI in the hypertrophic stage shows a soft tissue mass
with mild to marked high signal intensity in both
Tl- and T2-weighted images.
Microbiological examination and a confirmatory Biopsy
Treatment :
Usually self limiting course of its own accord ending in
the cicatrizing stage, the organism can nevertheless
be extremely difficult to eradicate by
antimicrobials.
Bactericidal antibiotics in large doses are given for a
minimum of four to six weeks and are continued
until two consecutive cultures from biopsy
material are proven negative.
Traditionally Used : streptomycin (im 1gm for 4-6wks)
and tetracycline
Recently used :
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Oral rifampicin (450mg for a period of 6
weeks), sulphamethoxazole-trimethoprim
combination, and ciprofloxacin.
Local application of 2 % acriflavin for a
period of 8 weeks has been noted to be
both efficacious and nontoxic.
Intralesional steroids have been tried
Kailasa Regime :
Carbolic acid (0.2ml) + Glacial acetic acid (0.2ml)
+ Glycerine (0.4ml) + 10ml distilled water is
injected locally as 1 to 2 ml twice weekly at
multiple sites of the lesion. Usually 8-10
injections lead to complete regression of
granuloma and restoration of normal nasal
patency. This mixture causes chemical
necrosis of granuloma
Rifampicin :
Nasal Instillation - 1 Amp of rifampicin 125mg
diluted in 20mL saline solution and 2mL of
the solution is applied twice daily locally for a
period of 8 weeks.
Nasal Infiltration - 1 Amp of rifampicin 125mg
infiltrated into granulomas every other day
Irradiation to a total dose of 3000-3500 Gy over
three weeks destroys scleroma but, with
the currently available antimicrobials, is
unlikely to be required
Actinomycosis
 Caused by Actinomyces species
such as Actinomyces israelii or
A. gerencseriae.
 The condition is likely to be polymicrobial aerobic
anaerobic infection.
 Actinomycosis occurs rarely in humans but rather
frequently in cattle as a disease called lumpy jaw
 Cervicofacial disease is caused most commonly
secondarily to dental infection, intraoral trauma or
manipulation
Signs and symptoms :
Characterised by the formation of painful abscesses in
the mouth.
In severe cases, they may penetrate the surrounding bone
and muscle to the skin, where they break open and
leak large amounts of pus, which often contains
characteristic granules (sulphur granules).
The purulent leakage via the sinus cavities contains
"sulphur granules," not actually sulphur-containing
but resembling such particles. These granules
contain progeny bacteria
Diagnosis :
In addition to microbiological examinations (requires
anaerobic processing of culture specimens)
magnetic resonance imaging and immunological
blood analyses may also be helpful
Treatment :
Generally sensitive to penicillin
In cases of penicillin allergy, doxycyclin is used
Sulfonamides such as sulfamethoxazole may be used as
an alternative regimen at a total daily dosage of 2-4
grams. Response to therapy is slow and may take
months.
Fungal conditions :
Rhinosporidiosis
 Chronic granulomatous infection that affects the nasal
mucosa, ocular conjunctiva and other mucosa.
 Aetiological Agent : Rhinosporidium seeberi
 15-40 yrs age group, M>F
 It has long been unclear whether this organism is a
fungus
 Natural habitat - stagnant pools of fresh water
 The disease is most prevalent in rural districts, among
persons bathing in public ponds or working in stagnant
water, such as rice fields
Classification :
Benign :
Nasal - Septal Mucous Membrane
Floor of Nose / Spur
Nasopharyngeal - Nasopharyngeal surface of soft palate.
Mixed
- Nasolacrimal
Bizzare
- Conjuctival / Cutaneous
Malignant :
Generalised
Clinical Manifestations :
Nose commonest site (70% cases)
The fungus causes the production of large sessile or
pedunculated lesions that affect one or both
nostrils
Goes unnoticed till nasal obstruction develops
Nasal discharge & epistaxis
ARE - reveals leafy, papular or nodular, smooth-surfaced
lesions that become pedunculated and acquire a
papillomatous or proliferative appearance
The lesions are pink, red or purple in colour, vascular
(bleeds on touch) studded with white dots
Spontaneous remission is unusual and, left untreated,
the polyps will continue to enlarge
Diagnosis :
HPE of tissue sections. These contain large, round or oval
sporangia up to 350 µm in diameter, with a thick
wall and an operculum
Management Options :
The treatment of choice is surgical excision of lesions,
with or without cauterization of base
No drug treatment has proved effective
Dapsone said to prevent maturation of sporangia &
promotes fibrosis, 50mg BD, 3 months preop and 6
months postop
Aspergillosis
 Causative Organisms include :
 Aspergillus fumigatus (90%)
Aspergillus niger, Asp. flavus
 Granulomatous invasive fungal rhinosinusitis :
 An asymptomatic period frequently occurs, symptoms
appearing only when the orbit or skull base are
involved
 Pain is the main symptom
 Chronic headache, proptosis and cranial nerve deficits
 Maxillary sinus - major site
Nasal endoscopy reveals nasal congestion or polypoid
mucosa and sometimes a soft tissue mass covered
by a normal or ulcerated mucosa
Radiological appearances usually show opacification
with bone erosion extending to the orbit and/or
skull base
Histopathology reveals fungal invasion of the tissue:
bone, mucous membrane, vessels.
Mycologic cultures lead to identification of the
species.
Treatment
Most patients have been treated with a combination of
surgery and antifungal chemotherapy (Itraconazole
and amphoterecin B)
Mucormycosis
 Caused by fungi in the order Mucorales
 Generally, species in the Mucor, Rhizopus, Absidia, and
Cunninghamella genera are most often implicated
 This disease is often characterized by hyphae growing in
and around vessels
Rowe Jones Classification :
 Non invasive
 Semi invasive
 Invasive
Types :
1.
2.
3.
4.
5.
Cerebral
Ocular
Pulmonary
Superficial
Disseminated
Signs & Symptoms :
MC Oral or cerebral mucormycosis
Involves sinuses, brain, or lungs as the areas of infection
Can also manifest in the GIT, skin &other organ systems.
Rarely maxilla may be affected
The rich vascularity of maxillofacial areas usually
prevents fungal infectionsl
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Athough more virulent fungi, such as those
responsible for mucormycosis, can often overcome
this difficulty
Key signs which point towards mucormycosis
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Fungal vascular invasion resulting in thrombosis
and death of surrounding tissue
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If the disease involves the brain then symptoms may
include a one-sided headache behind the eyes,
facial pain, fevers, nasal stuffiness that progresses to
black discharge, and acute sinusitis along with
swelling of the eye
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Affected skin may appear relatively normal during
the earliest stages of infection
This skin quickly progresses to an erythemic
(reddening, occasionally with edema) stage, before
eventually turning black due to necrosis
Diagnosis
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As swabs of tissue or discharge are generally
unreliable, the diagnosis of Mucormycosis tends to
be established by a biopsy specimen of the involved
tissue
Treatment
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Amphotericin B - 4–6 weeks
Posaconazole shown effective against mucormycosis
Liposomal drug available but expensive
Non Specific Inflammatory
Granulomatous Conditions
Sarcoidosis
 May affect any part of the body, but most frequently
involves lymph nodes, skin, lungs, eyes, liver, spleen
and the small bones of the hands and feet
 F:M = 2:1, 3rd to 5th decade
Aetilogy :
 Chemicals (including beryllium and zirconium)
 Pine pollen
 Peanut dust
Immunology in patients with Sarcoidosis:
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Type IV delayed hypersensitivity is depressed
Cell mediated immunity is normal
Type I humeral immunity is normal
Total plasma protein levels are raised
There is an increase in the amount of circulating
immune complexes
Process initiated by APC (probably alveolar
macrphage)  IL1 IL2 mediated T lymphocyte
proliferation IL8 attracts monocytes and held at
the site of reaction by macrophage migration
inhibition factor  Monocyte activation by IFN γ
Monocyte activation leads to Calcitriol production 
causes macrophage fusion to epithelioid cells and
granuloma formation
The persistence of the granuloma may be ascribed to
1. Continued antigenic stimulation,
2. Failure of suppressor-regulating mechanisms
3. Abnormalities in the regulation of the cytokine
network
Histology
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Scadding and Mitchell defined Sarcoidosis as a
disease characterized by formation in all of the
several affected organs / tissues of epitheloid
tubercles, without caseation.
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These granules either resolve on their own or
get converted into hyaline fibrous tissue
So Epithelioid cells surrounded by
lymphocytes and fibroblasts but devoid of
caseation
Crystalline or calcified inclusion bodies are
sometimes seen, e.g. Schaumann bodies.
Histological picture is not diagnostic and
similar granulomas can occur in a number
of other conditions including berylliosis and
fungal disease
Clinical features:
1.
2.
3.
4.
5.
6.
7.
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Nasal stuffiness / obstruction
Crusting
Blood stained discharge
Purulent discharge
Facial pain
Mucoid discharge
Anosmia
Nasal cavity mucosa has a characteristic granular
appearance (i.e. also called as strawberry skin).
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Nasal mucosa hyperemic and friable leading on to
blood stained discharge
There may also be crusting of the nasal mucosa
associated with mucopurulent discharge.
The anterior portion of the nasal septum may
perforate, especially if traumatized for example
during surgery.
The paranasal sinuses may also be affected by the
process and/or become secondarily infected
As a consequence, facial pain is experienced by
one in five patients with nasal sarcoid.
Sense of smell may be affected due to mechanical
obstruction of the olfactory cleft by crusting and
fibrosis, or due to direct neuropathy
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Adenoid enlargement or lymphoid hyperplasia may
lead to middle ear effusion
The soft tissue of the supraglottic larynx may
become thickened and increasingly swollen
producing dyspnoea and change in voice quality
Diagnosis :
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Diagnosis usually relies on a combination of
histology, imaging, haematology and clinical
acumen.
Kveim test : intradermal injection of a filtered
extract of spleen from a patient with sarcoid. Six
weeks later a skin biopsy is taken. The test is positive
when the histology shows features of Sarcoidosis
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Withdrawn in the UK over fears of virus and
prion transfer though without any substantive
evidence for this
Elevated Angiotensin converting enzyme (83% of
cases)
But it is also elevated in a number of other
conditions such as tuberculosis, leprosy and
primary biliary cirrhosis
ESR levels, serum globulin and serum and urinary
calcium levels are sometimes raised
Full blood count may show mild anaemia,
leucopenia, thrombocytopenia and eosinophilia,
but clearly none of these tests are specific
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Other investigations include (chest x-ray,
computerized tomography (CT), perfusion
studies, bronchoalveolar lavage and
gallium-67 scanning)
Biopsy of nasal mucosa is only of use if it
appears abnormal as random biopsy of
macroscopically normal mucosa is usually
negative (in 92 percent of cases).
Plain x-rays of the nasal bones may show
rarefraction or punctate osteolysis in up to a
quarter of cases and this, together with soft
tissue changes, may be shown by CT
scanning
Treatment :
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Some patients with limited disease undergo
spontaneous remission without specific
treatment
Rx consists of Combination of oral steroids,
methtotrexate and hydroxychloroquine,
depending on the severity of the disease.
Topical treatment may be offered for
nasal symptoms including intranasal
steroids, either sprays or drops, glucose
and glycerine drops and various forms of
nasal douching and irrigation
Wegner's Granulomatosis
 Classically pulmonary, renal, and head and neck
manifestations and skin involvement
 Nose and PNS MC affected site in the head and neck.
 Affects age gp of 15 – 70 yrs M:F = 1:1
 Aetiology : Unknown It could represent some form of
hypersensitivity reaction to an unknown stimulus
 The stimulus could be a form of inhaled bacteria.
 This could explain the frequency with which the
respiratory tract is involved
Pathology :
 Necrotizing vasculitis affecting small- to medium-sized
vessels (e.g. capillaries, venules, arterioles and arteries)
with necrotizing glomerulonephritis, is common
 The pathological hallmark is the coexistence of
vasculitis and granulomas and classically involves a
triad of airway, lung and renal disease
 Immunology:
 Antibodies reacting with the cytoplasm of granulocytes
and Monocytes have been demonstrated by van der
Woude.
 Two main forms of anti nuclear cytoplasmic antibodies
have been demonstrated. Perinuclear (pANCA) and
cytoplasmic (cANCA).
Clinical features :
 Necrotizing granulomas with vasculitis of the upper
and lower respiratory tracts.
 Focal necrotizing glomerulonephritis.
 Nose and sinuses involved in 80% cases
 There may be intra nasal destruction of bone and
cartilage leading to collapse of nasal bridge
 Rapid diagnosis remains of great importance since a
fulminating course with a fatal outcome can occur in
as little as 48 hours
Nasal symptoms :
 blood stained nasal discharge, crusting, granulation and
can even lead to septal perforations
 Minor nasal surgery and/or repeated biopsies during
this period may add to the problem
 many patients may complain of significant facial pain
 There can be destruction of the eye, orbit, palate, oral
cavity,oropharynx and sometimes middle ear
Oral Symptoms
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MC - hyperplastic granular lesion of the gingiva
If a tooth is lost, the socket may fail to heal
Extensive ulcerative stomatitis has also been reported
Otological Symptoms
AOM, SOM
There may be deafness, pain and suppuration.
Facial nerve paralysis has also been recorded
 Wegener's granulomatosis of the temporal bone
with necrotizing granulation tissue filling the
tympanic cavity has been documented
 Laryngeal and Tracheal symptoms
 Laryngeal involvement is unusual, but when
present the subglottis and upper trachea are most
commonly affected
 May lead to laryngotracheal obstruction
 Biopsies nonspecific and are contraindicated
leading to further inflammation and scarring.
 positive c-ANCA test seen in idiopathic subglottic
stenosis
 Extensive laser resection of subglottic stenosis
caused by Wegener's is frequently not only
unsuccessful, but also causes further stenosis.
Diagnosis :
 The cANCA test is positive in 95 percent of
patients with generalized active disease.
 ESR is found to be elevated
 Serum angiotensin enzyme levels are increased
 Presence of vasculitis
 Granulomas are of epitheloid cell type
 Multinucleated giant cells have also been
demonstrated
ACR criteria for diagnosis of wegner’s granulomatosis:
 Nasal oral inflammation
 Abnormal x-ray chest
 Urinary sediment
 Biopsy
: Development of painful /
painless oral ulcers /
purulent or bloody
nasal discharge.
: Shows presence of nodules,
infiltrates and cavities.
: Micro hematuria
: Granulomatous
inflammation within the
wall of an artery / arteriole
Treatment :
The following drugs can be used in combination.
1. Steroids
2. Cyclophosphamide
3. Azathioprine
– Prednisolone 60 – 80mg/day
– 2mg/kg/day
– can be used instead of
Cyclo phosphamide in doses of
200mg /day.
Neoplastic Conditions :
Non Healing Midline Granuloma
 Also known as Stewart's granuloma, Midline lethal
granuloma, Polymorphic reticulosis and Sinonasal
lymphoma, T / NK cell lymphoma
 Classical destruction of the midface.
 In the past, death resulted from intercurrent infection
of disseminated lymphoma due to a failure of diagnosis
or insufficiently aggressive oncologic treatment.
 Recently, similar appearances of aggressive mid facial
destruction caused by cocaine abuse have been
reported in which tests for granulomatous diseases
and lymphoma have been negative
Classification:
• Generalized lymphoma involving the Sinonasal tract
• Lymphomas of waldayer’s ring extending to the
nasopharynx
• Peripheral – Sinonasal lymphoma the classic T cell
lymphoma
 T/NK cell lymphoma may occur at any age from the 1st –
9th decade though the median is 5th – 6th decade, M>F
Clinical Features :
Three Stages
Prodromal stage:
Only Nasal obs & Rhinorrhoea, may last many years.
Period of activity:
Areas of necrosis develop on and around the nasal cavity
with purulent nasal discharge, crusting and tissue loss.
There is also progressive destruction of the nasal
framework, palate, upper lip extending in to the pharynx.
Orbit and skull base also could be involved.
Terminal stage:
Gross mutilation of the face, exhaustion and eventual death.
Systemic metastasis is also seen in this stage
HPE :
 Biopsy material from tissue beneath the slough and
crust is essential for diagnosis.
 Atypical cell (Atypical T lymphocytes) infiltrates are found
dispersed in necrotic areas.
 Immuno histochemistry using a panel of monoclonal
antibodies against T cell differentiation antigen should be
applied as 80% of peripheral T cell lymphomas show
aberrant phenotypes.
 Thrombosis and necrosis are common findings
 Granulomas and giant cells are not present in this
condition.
Treatment :
 Initially treated with low-dose radiotherapy but no
long term cures were found.
 Now, radical full course radiotherapy of 55 Gy or
more with wide field coverage including the nose,
sinuses and palate.
 Recurrence or development of disseminated lymphoma
worsens the prognosis and chemotherapy may be
advocated in those lesions classified as high grade