- Granulomatous diseases of the nose and paranasal sinuses can be caused by infections like tuberculosis and leprosy, as well as non-infectious conditions. - Tuberculosis of the nose commonly manifests as lupus vulgaris, presenting with nodular or ulcerative lesions. Leprosy can involve the nasal mucosa and cause destruction of the nasal cartilage and bone. - Syphilis may cause primary chancres or mucous patches in the nasal vestibule and pharynx during its secondary stage.

1. GRANULOMATOUS
DISEASES OF
NOSE & PNS
D R . D I V YA R A A N A
M S E N T P G

2. GRANULOMA
• Organized collection of macrophages, known as epithelioid
cells, which fuse to form multinucleated giant cells.
• Encountered in a number of infective, inflammatory and
neoplastic conditions of the nose and sinuses.
• Also seen in various conditions in which vasculitis plays a role.

4. INFECTIVE
CONDITIONS

5. BACTERIAL

6. TUBERCULOSIS
OF THE NOSE
Portal of Entry – Inhalation (Most
common), Ingestion or Inoculation.
Almost always associated with
Primary Pulmonary Tuberculosis.
Main Pathogens – Mycobacterium
Tuberculosis & M. Bovis
Most Common type affecting the Nose is Lupus Vulgaris / Tuberculosis Luposa / Cutaneous Tuberculosis.

7. LUPUS VULGARIS
• Cutaneous tuberculosis associated with painful skin lesions  face around
the nose, eyelids, lips, cheeks, ears & neck.
• Macroscopic appearance:
1. Nodular form (Most Common)
2. Ulcerative form
3. Sinus granuloma
Nodular form:
• Indolent & Chronic form.
• Affects skin & mucous membrane of nose.
• Young Female : Male = 2:1
• Temperate climate > In Tropics.

8. • Usually begins in the Vestibule (M.C. site =
Mucocutaneous Junction of the nasal septum) &
then extends to the adjoining skin & mucosa.
HISTOPATHOLOGY:
• Tuberculous granuloma
• In the center these lesions show collection of
reticuloendothelial cells. These cells soon necrose
& coalesce. Necrotic center is surrounded by a
ring of living RE cells which further are
surrounded by ring of Lymphocytes, Plasma Cells
& Fibroblasts. Giant cells are found scattered
throughout the tubercle & are usually
multinucleated.

9. SYMPTOMS:
• Nasal discharge
• Nasal obstruction
• Presence of non-foul smelling crusts
• Epistaxis
• Evening rise of temperature
• The typical early lesion in the nose is a reddish firm nodule at the
mucocutaneous junction of the nasal septum. These nodules are known
as “Apple Jelly Nodules”
• The nodules may coalesce & break down to form characteristic ulcers
with a pale granular base & undermined edges

10. DIAGNOSTIC FEATURES OF LUPUS NODULES:
1. Blanching
2. Bacterial examination
3. Biopsy
Blanching – To highlight the apple jelly nodules, blood should be
expressed from adjoining tissues by applying pressure with a glass slide,
this will make the pinkish lupus nodules to stand out in contrast.
Bacteriological examination may demonstrate Acid Fast Bacilli.
Culture of the organism – Lowenstein-Jensen slope, Culture can now be
accelerated by BACTEC.
Biopsy is always diagnostic.

11. COMPLICATIONS:
• Pulmonary tuberculosis, Dacryocystitis,
Ulceration, Nasopharyngeal Lupus, Lupus Of
Face, Atrophic Rhinitis
ULCERATIVE FORM:
• Ulcers are commonly found on the anterior
part of the nasal septum, inferior turbinate &
anterior choanae.
• This usually involves the cartilaginous nasal
septum or the inferior turbinate.
• Ulceration of nasal mucosa is usually followed
by fibrosis & contraction causing distortion of
alae nasi.

12. • If turbinates are involved lining ciliated
columnar epithelium is not renewed 
lead to formation of secondary atrophic
rhinitis.
• Septum may also undergo perforation,
but in these cases classically the
cartilaginous portion alone is involved &
hence there is no sinking of the nasal
bridge.

13. SINUS GRANULOMA
• Isolated sinus involvement may occur without any signs or symptoms
the nose.
• Diffuse soft tissue swelling & multiple discharging sinuses in the
supraorbital region secondary to osteomyelitic involvement of the
frontal bone is seen.
• Involvement of the orbit & its nerves may occur.
• CT & MRI are nonspecific – demonstrate a soft tissue mass with or
without bone destruction
The diagnosis is established by an External or Endoscopic Biopsy.

14. SPECIFIC INVESTIGATIONS:
• Montaux test, AMTD (Amplified Mycobacterium Tuberculosis Detection),
PCR, Fluorescence microscopy
AMTD - uses principles of transcription mediated amplification of rRNA
Mycobacterium TB. Detection of tRNA indicates the presence of actively
multiplying MTB.
TREATMENT:
• Responds well to antituberculous drugs.

15. LEPROSY
• Causative Organisms – M. Leprae.
• M. Leprae  cannot be cultured on an artificial
medium, it can nevertheless be inoculated into
experimental animals, particularly immunologically
deficient mice, to produce a disease similar to that
in man.
• Based on the Host's Immunological Status &
Clinical, Histological & Microbiological features
leprosy divided into types.

16. 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

17. 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
eyebrows.
• Highly infectious nasal discharge.

18. 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.

19. • 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

20. 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 involvement suggests progression to LL.

21. Diagnosis
• Early diagnosis is essential since nasal discharge is principle route of
transmission of the disease
• Clinical findings of anaesthesia on the skin, thickened peripheral nerves
and skin lesions, & supplemented by bacteriological examination.
• Confirmed by demonstration of M. leprae on microscopy of the nasal
discharge or scrapings of nasal mucosa, or histopathology of nasal
mucosa.
• Skin biopsy in tuberculoid leprosy

22. 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

23. • Rifampicin stopped after 3 months
• Rest 2 continued till 9 months
• Intranasal Rifampicin much faster than oral route
• Nasal douching for removal of crusts

24. SYPHILIS
• Nasal syphilis can occur at any age
• Uncommon as signs symptoms difficult to elicit in
early stages especially if antibiotics are 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.

25. • 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.

26. 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
with enlarged, rubbery, and non
tender lymphadenopathy

27. • A sore or chancre develops at the site of inoculation 10-90 days(average21
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 perchloride of mercury
and the surface smeared with 2% yellow mercuric oxideointment.
• The following points should be borne in mind : Cultures from the surface of
the lesion will always be negative.

28. • Smears when examined under
dark ground illumination will
show the spirochete Treponema
palladium
• Serological tests for syphilis may
be positive– VDRL, TPHA, FTA-
ABS. (TheVDRL is a non
treponemal test and is relatively
nonspecific but becomes
positive early (four to fiveweeks)
and correlates well with disease
activity.)
• A biopsy from suspicious lesion
may confirm the diagnosis

29. Secondary Syphilis
• Most infectious of all the three stages
• Symptoms6-10 weeksafter 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 &
of nasal vestibule
• Other secondary lesions like mucous patches in the
pharynx are also seen
• Roseolar / papular skin rashes ,enlarged non tender
lymph nodes.
• The scar of the primary lesion in the genitals or
elsewhere may be visible.

30. • Serological tests for syphilis are
positive.
• Dark-field examination of moist or
intentionally a braided 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.

31. 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.
• Thebony portion of the nasal septum is frequently affected causing
septal perforation.

32. • Rarely the following portions of then nose can also be involved :
Lateral nasal wall
Frontal sinus
Nasal bones
Floor of the nose
Symptoms include:
• 1. Pain / headache(worseduring night)
• 2. Swelling / obstruction of nose- swelling may bediffuse / 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 collapseof the
bridge of the nose can cause structural damage to the nasal architecture
• 5. There may also be associated secondary atrophic rhinitis.

33. • 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

34. • Treatment :
• 1.DOC for all stages– parentral Penicillin
• 2.Alkaline nasal douches one to three times a day.
• 3.Local yellow mercury oxide ointment. Diluted 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.

35. Congenital syphilis “Snuffles”:
• Any of the lesions of secondary
and tertiary forms of syphilis of
nose can occur.
• Classically begin during the3rd
week of life.
• May appear around 3rd month
also
• At First – simple catarrhal rhinitis.
• Later becomes purulent with
fissuring and excoriation of the
nasal vestibule and upper lip.

36. • Gummatous and destructive lesions
occur most commonly at puberty.
• Mucous membrane, periosteum and
bonemay all beaffected.
• Other stigmata of syphilis,
particularly Hutchinson's incisors and
Moon’s molars, interstitial keratitis,
corneal opacitiesand sensorineural
deafnessmay bepresent, and there
may be a family history of syphilis,
miscarriages and still births.
Serologic tests for syphilis will
invariably be negative

37. 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
oxide ointment may be applied frequently to the nasal vestibules

38. RHINOSCLEROMA
• 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.

39. Pathology :
• Caused by gram negative bacilli Klebsiella rhinoscleromatis/ Frisch bacilli /
diplo bacillus.
• F>M,
• 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
lymphocytes and eosinophils.

40. • Scattered large foam cells (Mikulicz cells)
which have a central nucleus and a
vacuolated cytoplasm containing frisch
bacilli and Russel bodies.
• 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

41. 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

42. 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) , hebra 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.

44. • Complications :
• 1. External nosedeformity
• 2. Vestibular stenosis
• 3. Cicatrization of soft palate
• 4. Nasal regurgitation
• 5. Tracheal stenosis

45. Diagnosis :
• Levin test (complement fixation test) : High titres of antibodies against
K. Rhinoscleromatis has been demonstrated.
• 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

46. 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
six weeks and are continued until two consecutive cultures from biopsy
material are proven negative. Traditionally Used : streptomycin (im 1gm
for 4-6wks) and tetracyclines.
• Recently used : Oral rifampicin (450mg for a period of 6 weeks),
sulphamethoxazole-trimethoprim combination, and ciprofloxacin.

47. • Local application of 2 % acriflavin for a period of 8 weeks has been
noted to be both efficacious and nontoxic.
• 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

48. FUNGAL
CONDITIONS

49. 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

51. Classification :
• Benign :
Nasal - Septal Mucous Membrane, Floor of Nose/
Spur
Nasopharyngeal - Nasopharyngeal surface of soft
palate.
Mixed - Nasolacrimal
Bizzare - Conjuctival / Cutaneous
• Malignant : Generalised

52. Clinical Manifestations :
• Nose is the 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
• A/R – reveals leafy, papular or nodular,
smooth-surfaced lesions that become
pedunculated and acquire a
papillomatous or proliferative
• The lesions are pink, red or purple in
colour, vascular (bleeds on touch)
studded with white dots

53. • Spontaneous remission is unusual and, left
untreated, the mass 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 cauterization of base
• No drug treatment has proved effective
• Dapsone is said to prevent maturation of
sporangia & promotes fibrosis, 50mg BD, 3
months preop and 6 months postop.

54. INFLAMMATORY
CONDITIONS

55. SARCOIDOSIS
• Systemic granulomatous condition of unknown aetiology.
• May affect any part of the body, but most frequently involves the lungs
and intra-thoracic lymph nodes, in over 90% of cases.
• 6–16 per 1,00,000 population per annum.
• Young adults with a peak onset between the third and fourth decades.
• F > M

56. AETIOLOGY:
• The aetiology of sarcoidosis remains unknown, but theories suggest an
immunological response to an unidentified antigen in genetically
predisposed individuals.
• The antigens may be infectious or environmental, and possibilities
mycobacteria, fungi, chemicals such as beryllium and zirconium, pine
pollen and peanut dust.
• There are abnormalities of both the cell-mediated and humoral immune
systems.

57. Macrophages and T-cell
activation
Release of cytokines including
tumour necrosis factor (TNF)
Granuloma formation

58. Clinical features:
• Multisystem disease
• It involves the upper respiratory tract in up to 18% of cases.
• Nasal symptoms commonly include obstruction, crusting, bleeding or
facial pain
• A staging system for sinonasal involvement has been described to help
treatment:
Stage I : Reversible involvement of the nose alone.
Stage II : Moderate but potentially reversible disease of the nose and/or
limited sinus involvement
Stage III : Severe, irreversible and extensive sinonasal disease.

59. • The nasal mucosa often has a characteristic
granular appearance, sometimes referred to as a
‘strawberry skin’ because of the tiny pale
granulomas against hypertrophic erythematous
mucosa.
• Friable ,ulceration, crusting and adhesions.
• The nasal bones may be involved; patients may
present with a soft-tissue mass or expansion of
the nasal bridge.
• This may be associated with thickening and
purplish discoloration of the overlying skin
known as lupus pernio, which is a cutaneous
manifestation of chronic systemic sarcoid.

60. • Salivary gland enlargement is seen in 5–10% of cases.
• More rarely associated with facial palsy and uveitis when it is termed
Uveoparotid fever or Heerfordt’s syndrome.
• The larynx is involved in 1–5% of cases, most commonly the supraglottis
(85%), with symptoms of cough, hoarseness, dysphagia and more rarely
stridor.
• Tracheal involvement is rare but bronchial sarcoid is common.
• Chronic progressive pulmonary fibrosis occurs in 25% of cases of
chronic pulmonary sarcoid.

61. DIAGNOSIS:
• No test is pathognomonic.
• Diagnosis relies on a combination of clinical features, imaging, histology,
biochemical and serological testing and the exclusion of other
granulomatous diseases.
• Biopsy should target potentially affected tissue such as the lung, skin and
lymph nodes; lip biopsy of minor salivary glands can be useful.
• Serum angiotensin converting enzyme (ACE) is elevated in up to 85%
active disease, but this is non-specific
• Imaging of the lower respiratory tract shows varying degrees of pulmonary
involvement, which allow staging of the disease
• Plain X-rays of the nasal bones may show rarefaction or punctate osteolysis
• CT will also demonstrate secondary involvement of the sinuses though will
not distinguish between active disease and secondary infection.

63. TREATMENT:
• Variable clinical course that often correlates with the mode of
presentation;
• Acute onset with arthritis and erythema nodosum is usually self-
more insidious onset with lupus pernio.
• Sinonasal involvement tends to be more chronic.
• Two-thirds of patients will spontaneously remit.
• 10–30% follow a chronic course with relapses despite systemic therapy.
• Steroids remain the mainstay of treatment for severe disease (lack of
controlled studies), with the addition of agents such as methotrexate to
reduce the steroid dose if required.
• Hydroxychloroquine  cutaneous and sinonasal sarcoidosis, response
rate is less than 50% and there is a risk of ocular toxicity.
• TNF-alpha inhibitors such as infliximab and etanercept

64. • Nasal symptoms  intra-nasal steroids, glucose and glycerine drops,
and nasal douching.
• Surgery has a limited role, particularly in the presence of active disease,
although endoscopic sinus surgery can be undertaken to remove
obstructing lesions or for secondary bacterial infection.
• Septal surgery should be avoided if possible as an increased rate of
septal perforation.
• Symptomatic laryngeal disease that fails to respond to systemic
treatment may be treated with transoral laser and intra-lesional steroid
injections

65. GRANULOMATOSIS
WITH
POLYANGIITIS
(WEGENER’S)
• Systemic condition characterized by
granulomatous inflammation of the
respiratory tract and necrotizing
vasculitis affecting small to medium-
sized vessels with focal or
proliferative glomerulonephritis.
• Triad of upper airway, lung and renal
disease
• 40–50 years.
• Men and women are equally affected

66. Aetiology
• Autoimmune disease, given its strong association with antineutrophil
cytoplasmic antibodies (ANCA).
• ANCA binds to and activates neutrophils  release oxygen radicals,
inflammatory cytokines and lytic enzymes and adhere to and kill vascular
endothelial cells.
• ANCA may also induce immune complex formation.
In GPA, cytoplasmic ANCA (c-ANCA) are specific.
There is evidence that a mimic peptide may be introduced via an
exogenous infective agent such as Staphylococcus aureus. This theory is
supported by the higher rate of chronic nasal S. aureus carriage in
with GPA, in whom it is associated with more frequent relapses.

67. 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
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

68. • 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 : Most common - 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.

69. • Laryngeal and Tracheal
symptoms : Laryngeal
involvement is unusual, but
when present the subglottis
and upper trachea are most
commonly affected
• May lead to
obstruction
• Biopsies nonspecific and
contraindicated leading to
further inflammation and
scarring.
• positive c-ANCA test seen
idiopathic subglottic
stenosis

70. 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
• Presenceof vasculitis
• Granulomasareof epitheloid cell type
• Multinucleated giant cells have also been demonstrated

71. Treatment :
• The following drugs can be used in combination.
1. Steroids – Prednisolone60 – 80mg/day
2. Cyclophosphamide – 2mg/kg/day (side effects including alopecia,
haemorrhagic cystitis, opportunistic infections and malignancies; leukaemia and
lymphoma are 11 times more likely, and there is a 33-fold increase in the risk of
bladder cancer. )
3. Azathioprine – can be used instead of Cyclo phosphamide in doses of 200mg
/day.
• The multicentre randomized double-blind RAVE (Rituximab for ANCA-
Vasculitis) trial also suggested that rituximab may be superior in relapsing
disease.

72. EOSINOPHILIC
GRANULOMATOSIS
WITH POLYANGIITIS
(CHURG STRAUSS)
• ‘Eosinophil-rich and granulomatous
inflammation involving the
respiratory tract, and necrotizing
vasculitis affecting small to medium-
sized vessels, associated with asthma
and eosinophilia.’
• Rarest of the ANCA-associated
vasculitis
• Annual incidence of 0.5–4 per million.
• In asthmatics this incidence rises to
67 per million.
• The mean age at diagnosis is 50
years (range 4–75 years).
• M=F

73. AETIOLOGY:
• High levels of eosinophils and immunoglobulin E (IgE) in vessels and
tissues also suggests a direct role in the development of the vasculitis,
possibly an allergic response to an unknown allergen.
Autoimmune disease
Th2-cells Activate eosinophils
and neutrophils.
Tissue injury
release of cationic
proteins and cytokines

74. Clinical features :
• Asthma occurs in approximately 99% of patients
• Sinonasal symptoms are present in up to 93% .
• Allergic rhinitis and chronic rhinosinusitis with nasal polyps occur in up
to 75% of cases.
• Nasal obstruction, rhinorrhoea, anosmia and sneezing, as well as
crusting and epistaxis
• Neurological symptoms : mononeuritis multiplex, peripheral
polyneuropathy
• Cutaneous symptoms : Papules and nodules
• Cardiac : granulomatous myocarditis.

75. • Phase one: prodromal  persist for
several years and is characterized by
adult-onset asthma and upper
respiratory tract inflammation.
• Phase two : characterized by
peripheral eosinophilia, usually with
pulmonary infiltrates and eosinophilic
gastroenteritis.
• Phase three : systemic vasculitis,
including constitutional symptoms,
neurological and cutaneous
involvement.

76. Treatment :
• The following drugs can be used in combination.
1. Steroids – Prednisolone60 – 80mg/day
2. Cyclophosphamide – 2mg/kg/day (side effects including alopecia,
haemorrhagic cystitis, opportunistic infections and malignancies; leukaemia
and lymphoma are 11 times more likely, and there is a 33-fold increase in the
risk of bladder cancer. )
3. Azathioprine – can be used instead of Cyclo phosphamide in doses of
200mg /day.

77. GIANT CELL
GRANULOMA
• Granuloma-like aggregates of
giant cells in a fibrovascular
stroma characterize this benign
condition otherwise referred to
as ‘giant cell reparative
granuloma’ or ‘giant cell
reaction of bone’.
• children and young adults
• Female to male preponderance
of 2:1.

78. Clinical features :
• Pain and swelling over the affected
bone  common symptom
• Diplopia, hearing loss, vertigo and
tinnitus have also been reported.
• The maxilla and mandible are most
affected followed by the sphenoid and
temporal bones.
• Bilateral symmetrical involvement of
the jaws cherubism, a rare inherited
childhood condition
Diagnosis :
• CT shows expansile lytic lesions with a
‘soap bubble’ centre and well-
demarcated edges.

79. • Histologically, the granuloma has a cellular fibroblastic stroma containing
aggregates of giant cells. These cells are smaller and have fewer nuclei
than in a true giant cell tumour
Treatment
• Curettage alone is associated with recurrence in 15% of cases and
excision should be undertaken where possible.

80. NEOPLASTIC

81. EXTRANODAL
NK/T-CELL
LYMPHOMA
• PreviouslyMidline lethal granuloma
• Destruction of the midface
• Most common in south-east Asia and
South America
• most common in the fifth or sixth
decades.
• M>F
Aetiology
• ENKTCL appears to be caused by the
Epstein-Barr virus.

82. Clinical features :
• Arises in the nasal cavity
• Spreads to involve adjacent
structures including the orbits, oral
cavity, skin and paranasal sinuses
• Initially causes symptoms of
obstruction, discharge and bleeding.
• Progressive destruction of the nasal
skeleton and overlying skin occurs
Diagnosis :
• Early diagnosis is important, as the
prognosis for wide- spread disease
much worse.
• Biopsy material from tissue beneath
the slough and crust is essential.

83. • Histologically, the infiltrates are polymorphic and atypical cells may to be arranged in a
necrotizing angiocentric growth pattern.
• The tumour consists of neoplastic T-lymphocytes with a significant inflammatory
infiltrate.
• Immunohistochemistry is usually positive for CD56, CD2 and cytoplasmic CD3.
Treatment
• Very good response to radiotherapy.
• Local recurrence rates range from 31–67%, with an over all 5-year survival rate of 38–
45%.

84. THANK YOU