granulomatous conditions of nose

1. Granulomatous
conditions of Nose
Dr Mayur Ingale

2. Introduction
◼ The nose and paranasal sinus are the site of election
of some dramatic ulcerative granulomatous diseases.
◼ McBride (1897) described a patient with a rapid
destruction of nose and face proceeding to fatal
termination.
◼ Kraus (1929) described a patient with granuloma and
extensive destruction of nose, oral cavity, pharynx
and suggested the term “granuloma
gangraenescens”.

3. ◼ The principle element of a granulomatous condition is a self
evident granuloma consisting of macrophages, epitheloid
cells and a multinucleated giant cell.
◼ Inflammation of blood vessels may be primary or secondary
depending upon the size of vessels affected.

4. .
◼ Large > Giant cell arteritis
medium > Granulomatous arteritis
small
◼ Medium > Polyarteritis nodosa
small >Wegener’s syndrome
◼ Misc > Behcet’s disease
> Kawasaki disease
◼ Secondary vasculitis
◼ Infection related vasculitis
◼ Serum sickness
◼ Malignancy related

5. Classification of Nasal granulomas:
Specific:
1. Tuberculosis
2. Leprosy
3. Syphilis
4. Yaws
5. Scleroma
6. Actinomycosis
7. Aspergillosis
8. Mucormycosis
9. Rhinosporidiosis
10. Leishmaniasis
Nonspecific:
1. Sarcoidosis
2. Wegner's granulomatosis
3. Sinonasal lymphoma
4. Eosinophilic granuloma

6. Tuberculosis:
◼ Tuberculous lesions involving nasal mucosa is very rare. It is
always associated with primary pulmonary tuberculosis.
◼ Macroscopic appearance:
1. Ulcers
2. Polypoidal lesions
3. Nodular lesions
◼ Ulcers are commonly found on the anterior part of the nasal
septum, inferior turbinate and anterior choanae

7. .
Lupus vulgaris
◼ Chronic from of tuberculous infection affecting the skin and
mucous membrane of nose.
◼ The mucocutaneous junction of the nasal septum is the most
common site.
◼ Females more affected than males.
◼ Histopathology:
Sections of these lesions show the classic features of tuberculous
granuloma. These lesions show collection of reticuloendothelial
cells. These cells soon necrose . This necrotic centre is
surrounded by a ring of Reticuloendothelial cells. Lymphocytes,
plasma cells and fibroblasts.
◼ Giant cells are found scattered throughout the tubercle and are
usually multinucleated.

8. ◼ Clinical features
◼ Symptoms:
1. Nasal discharge / obstruction
2. Presence of non foul smelling crusts
3. Epistaxis
◼ Ulceration of nasal mucosa is usually followed by fibrosis
◼ 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”
◼ Septal perforation (cartilage part)

9. ◼ Diagnostic features of Lupus nodules:
1. Blanching
2. Bacterial examination for AFB
3. Biopsy is diagnostic
◼ Complications:
1. Dacryocystitis
2. Lupus of face
3. Atrophic rhinitis
4. Development of epithelioma

10. Treatment
Antituberculous drugs:
◼ Rifampicin 450-600 mg per day or 10 -12 mg /kg body weight
and for intermittent therpy 900mg
◼ Isoniazid 4-5 mg /kg body weight daily and for intermittent
therpy its 14-15 mg /kg body weight+ pyrodoxine10-20 mg daily
◼ Streptomycin 0.75-1gm daily
◼ Pyrazinamide 30 mg/kg body weight or 45- 50 mg /kg body
weight twice weekly
◼ Ethambutol 1200 mg /kg

11. Leprosy
◼ This is a chronic granulomatous disease caused by M. Leprae
◼ Types of leprosy:
◼ Tuberculoid leprosy: are solitary lesions involvement of one or more
sensory or motor nerves
◼ Paralysis of muscles
◼ Skin lesions may extend up to the nasal vestibule
◼ Lepromatous leprosy:
◼ In this type of leprosy there is diffuse infiltration of skin, nerves and
mucosal surfaces by the bacteria
◼ Cutaneous infiltration is more common over the edges of
pinna, chin, nose and eyebrows
◼ crust formation, nasal obstruction and Serosanguinous discharge.
◼ Nasal bridge collapse is commonly seen in these patients

12. .
◼ Diagnosis can always be confirmed by the scrapings of nasal
mucosa. They demonstrate the typical cigar pattern Lepra bacilli.
◼ Commonly positive scrapings can be obtained from the anterior end of
inferior turbinate
◼ Borderline leprosy:
◼ These patients have poor immunological resistance
◼ In pure borderline lesions involvement of nasal mucosa is not seen

13. .
Treatment
◼ Triple therapy:
1. Rifampicin – 600mg on first two days of a month taken before
breakfast
2. Clofazimine – 100mg on alternate days for three times a week
3. Dapsone – 100 mg a day
◼ These drugs are continued for 3 months after which Rifampicin
is stopped. Other two drugs are continued till 9 months are
completed.
◼ Nasal douching with saline help in removal of crusts.

14. .
Syphilis:
◼ Nasal syphilis can affect any age group.
◼ Histopathology:
◼ Diagnosis is purely histopathological. It is characterized by
oedema,stromal infiltration with lymphocytes, plasma cells and
endothelial cells. Perivascular cuffing and endarteritis will cause a
reduction in the lumen of blood vessels causing necrosis and ulceration.
◼ Nasal syphilis can be classified into:
1. Primary syphilis: (chancre)
◼ Hard, non tender ulcerated papule always associated with enlarged,
rubbery, and non tender lymphadenopathy.

15. .
◼ Diagnosis is based upon:
1. Cultures from the surface of the lesion will always be negative
2. Smears when examined under dark ground illumination will
show the spirochete Treponema palladium
3. Serological tests for syphilis is positive – VDRL,FTA-ABS
4. A biopsy from suspicious lesion may confirm the diagnosis.
◼ Secondary syphilis:
This is the most infectious of all the three stages of syphilis.
Symptoms usually appear 6-10 weeks after inoculation.
The symptoms include:
◼ Simple catarrhal rhinitis (persistent)
◼ Crusting & fissuring of nasal vestibule
◼ Other secondary lesions like mucous patches in the pharynx.
◼ Papular skin rashes
◼ Enlarged non tender lymph nodes
◼

16. .
Tertiary syphilis:
◼ This stage is commonly encountered in the nose. The 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:
1. Lateral nasal wall
2. Frontal sinus
3. Nasal bones
4. Floor of the nose

17. /
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. septal perforation
5. There may also be associated secondary atrophic rhinitis
6. The lesion is usually unilateral, but if septum is involved then
swelling may be present in both sides of the nasal cavity.
7. Tenderness over the bridge of the nose is a characteristic sign

18. .
◼ Diagnosis is based upon
1. The swollen nasal mucosa does not shrink when
vasoconstrictors are used.
2. Radiographs show rarefaction of bone.
3. Serological tests for syphilis are positive
4.Biopsy from the lesion is diagnostic.

19. Treatment
◼ The first-choice treatment for uncomplicated syphilis remains a
single dose of intramuscular penicillin .
◼ Doxycycline and tetracycline are alternative choice; however, it
cannot be used in pregnant women
◼ Antibiotic resistance has developed to a number of drugs
including macrolides, clindamycin,and rifampin.
◼ Ceftriaxone is also effective.

20. .
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

21. .
Yaws
◼ This condition is also known as “Framboesia”. This disease
closely resembles syphilis in its pathology.
◼ Seen mostly in central Africa, Jamaica
◼ Causative organism: Treponema Pertenue.
◼ Transmission: Is by direct extra genital contact.

22. .
Clinical features:
◼ Primary, secondary and tertiary stages occur as in syphilis. Yaws
characteristically affects the skin.
◼ Mucous membrane are usually spared .
◼ Advance nasal lesions are associated with extensive destruction
of the nose, palate etc.
◼ Destruction also may involve the whole of the maxilla, face and
pharynx

23. .
Rhinoscleroma: “Scleroma”
◼ This is a progressive granulomatous lesion beginning in the nose
and eventually extending into the Nasopharynx and oropharynx.
Rarely larynx, trachea and lower airway may also be involved.
◼ Scleroma may occur at any age.
◼ Both sexes may equally be affected

24. .
◼ Pathology: Organism Klebsiella Rhinoscleromatis.
◼ Granulomatous tissue infiltrates the submucosa and is
characterized by the presence of accumulation of plasma cells,
lymphocytes and eosinophils among which are scattered
large foam cells (Mikulicz cells). These foam cells have a central
nucleus.
◼ Russell bodies have also been demonstrated.
Investigations:
◼ Levin test: This is a complement fixation test.
◼ Serum with suspensions of K. Rhinoscleromatis.
◼ High titres of antibodies against K. Rhinoscleromatis has been
demonstrated. This indicates humoral immunity to be intact in these
patients

25. .
◼ Clinical features:
Three different stages have been documented:
1. Atrophic stage: In this stage changes appear in the nasal mucous
membrane.These changes resemble that of atrophic rhinitis. Foul smelling
crusts are seen.
2. Granulation / nodular stage: Nodules are non ulcerative in nature. Initially
these
nodules are bluish red and rubbery. Later these nodes become a little paler
and harder.
◼ 3. Cicatrizing stage: Adhesions and stenosis distort the normal nasal anatomy.
The shape and contour of the nose changes causing a condition known as
“Tapir’snose”. The disease may extend to involve the maxillary sinus, naso
lacrimal duct, Nasopharynx, trachea and bronchi. Lymphatic spread is
uncommon becauseof extensive fibrous tissue deposition. This deposition
blocks the lymphatics

26. .
◼ Diagnosis is confirmed by biopsy.
◼ Bactericidal antibiotics should be administered
in large doses. It should be administered atleast
for a period of 4-6 weeks.
Surgical debridement should be considered
when extensive cicatrization is present.

27. Non specific granulomas
Sarcoidosis
◼ This granulomatous lesion is a systemic condition of unknown
etiology.
◼ This may affect any part of the body
◼ Sarcoidosis affect young adults,commonly between their 3rd and
5th decades of life. Females/males by a ratioof 2:1.
◼ Etiology: Etiology is unknown. Various theories have been
proposed.
◼ Infective agents (? Viral)
◼ Chemical (Beryllium & Zirconium)
◼ Pine pollen
◼ Pea nut dust

28. .
◼ Immunology in patients with Sarcoidosis:
1. Type IV delayed hypersensitivity is depressed
2. Cell mediated immunity is normal
3. Type I humeral immunity is normal
4. Total plasma protein levels are raised
5. There is an increase in the amount of circulating immune
complexes

29. .
Histology:
◼ Sarcoid granuloma is characterized by epitheloid cells
surrounded by lymphocytes and fibrocytes, but there is no
caseation.
◼ Crystalline / calcified inclusion bodies are seen. These bodies are
known as Schaumann bodies.

30. .
Clinical features:
1. Nasal stuffiness / obstruction
2. Crusting
3. Blood stained discharge
4. Purulent discharge
5. Facial pain
6. Mucoid discharge
7. Anosmia
◼ Nasal cavity mucosa has a characteristic granular appearance
(strawberry skin).
◼ The anterior portion of the nasal septum may perforate,
especially if traumatized for example during surgery
◼ Adenoid enlargement may lead to middle ear effusion

31. /
◼ Diagnosis: Is by a combination of histology, imaging,
hematology and clinical acumen.
◼ 1. Kveim test: This is a skin test which helps in the diagnosis of
Sarcoidosis.
This test is performed by 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.
◼ 2. Angiotensin converting enzyme is found to be elevated in
patients during acute phase of the disease
3. ESR levels are raised
4. X-ray chest will identify pulmonary lesions

32. .
Treatment:
◼ Some patients may be lucky enough to have the benefit of
spontaneous resolution.
Majority of the patients are treated with a combination of oral
steroids, methotrexate and hydroxychloroquine.
◼ The treatment modality depends on the severity of the disease.
Nasal douching may be needed to remove the crusts.
Endoscopic sinus surgery may be needed to treat secondary
bacterial infection

33. Churg Strauss Syndrome
◼ Defined as eosinophil-rich and granulomatous inflammation
involving the respiratory tract and necrotizing affecting small to
medium sized vessels associated with asthma & eosinophillia.
◼ Lesion show necrotising giant cell vasculitis,granuloma
formation and eosinophillic pulmonary infiltrates.
◼ There may be nasal crusting & septal perforation
◼ Treatment includes :
◼ Oral steroids
◼ Surgical resection of the polyp.

34. Eosinophilic granuloma
◼ Proliferation of langerhans cells associated with inflammatory
infiltrates of eosinophillia, histocytes, plasma cells and
neutrophils.
◼ Males more affected than females.
◼ Clinical features include
◼ Painful sweeling of the bone (temporal ,frontal ,parietal)
◼ Cervical lymphadenopathy
◼ Mandibular lesion produce toothache, gum ulceration, loose teeth.
◼ Histology :langerhans cells are mixed with eosinophils, plasma
cells, neutrophils.
◼ During healing phase stoma becomes hard.

35. ◼ Treatment includes:
◼ In unifocal disease curettage and excision is done.
◼ Chemotherpy regime include etoposide and steroids given over a
period of 12 months
◼ Recently alpha interferon and bone marrow transplantation is
done.

36. Giant cell granuloma
◼ Benign condition generally seen in young adults.
◼ Clinical features include:
◼ Pain and seliing
◼ Diplopia
◼ Maxilla and mandible are most commonly affected.followed
by sphenoid and temporal bones.
◼ Histology : cellular fibroblastic stoma containing giant cells
◼ Treatment: curettage and excision.

37. .
◼ Cholesterol granulation
◼ Cholesterol precipitated in the tissue resulting in
haemorrhage or trauma.
◼ Males more affected than females
◼ Lesion may affect the maxilla , frontal sinus producing
expansion of the bone cosmetic deformity and
displacement of the adjacent structures.
◼ Histology shows apperance of granulation tissue containing
forgien body type giant cells created by cholestrol crystals.
◼ Treatment : excision

38. Wegener’s granulomatosis
◼ The condition was named after Dr. Friedrich Wegener
in 1936.
◼ It is a rare condition characterized by granulomatous
inflammation involving the respiratory tract and
necrotising vasculitis affecting small to medium sized
vessels.
◼ The disease is seen in 3rd decade of life.
◼ Males are more affected than females 4:1

39. .
◼ May attack the respiratory system, sinuses, nose,
kidney.
◼ Rarely seen affecting the central nervous system

40. Clinical features
◼ Rhinitis is the first sign in most of the patients.
◼ There may be associated symptoms like fever,
weight loss, anemia, joint pain, night sweats, fatigue.
◼ The disease may progress despite of antibiotic
treatment causing odema of the face, eyes,
proptosis, antroalveolar fistula or even saddle
deformity of the nose due to perforated septum.

41. .
◼ Ears : Conductive hearing loss due to auditory tube
dysfunction sometimes SSN.
◼ Oral cavity : gingivitis, bone destruction causing
loosing of teeth, nonspecific ulceration throughout
oral mucosa.
◼ One of the main feature is
vasculitis.

42. .
◼ Other affected organs:
◼ Lungs : may complain of pain, cough, haemoptysis.
◼ Bronchial stenosis is found in 20% of the cases
◼ Kidney : rapidly progressive glomerulonephritis seen in
75% of the cases leading to CRF.
◼ Trachea : may cause subglottic stenosis.
◼ Eyes : scleritis, conjuctivitis, episcleritis.
◼ Nervous system : occasionally sensory neuropathy (10%)

43. Pathophysiology
◼ It is widely presumed that ANCA’s are
responsible for inflammation of wegener’s.
ANCA’s Neutrophil Endothelium
damage to
endothelial cells

44. Histopathology
◼ Presence of multinucleated giant cells is helpful in
correct interpretation of the presenting lesion.
◼ The giant cells in Wegener granulomatosis resemble
those of tuberculosis (langhans type).
◼ The cytoplasm is more compact, homogenous and
eosinophilic.
◼ Necrotising arteritis is the essential feature of
microscopical picture.

45. .
◼ Vessel walls are infiltrated by acute inflammatory
cells and show a partial fibrinoid necrosis.
◼ Patchy necrosis is also the essential feature for
diagnosis.

46. Criteria for diagnosis
◼ In 1990 ACR accepted criteria for WG
◼ Two or more positive criteria have a senstivity of
88.2% and specificity of 99.2% of describing
Wegener’s Granulomatosis.
◼ Nasal or oral inflammation :
◼ Painful or painless oral ulcer.
◼ Purulent or bloody nasal discharge.
◼ Lungs :
◼ Nodules
◼ Infiltrates
◼ Cavities

47. .
◼ Kidney :
◼ Microhematuria
◼ Red cells casts
◼ Granulomatous inflammation within the arterial wall or in
the perivascular area.

48. .
◼ According to Chapel Hill conference on
nomenclature of systemic vasculitis diagnosis is
based:
◼ Granulomatous inflammation involving the respiratory
tract.
◼ Vasculitis of small to medium size vessels.

49. Diagnosis
◼ Wegener granuloma is suspected when the patient has
unexplained symptoms.
◼ Determination of ANCA’s be a useful aid in diagnosis
◼ Cytoplasmic staining ANCA react with enzyme proteinase-3
and are associated with wegener’s.
◼ If the patient has renal failure then biopsy should be sent.
◼ Urine test may detect protein, red blood cells and albumin.

50. .
◼ Blood tests may show increased ESR. (30 -60 mm/hr)
◼ CT scan of sinus may show bony erosion

51. Treatment
◼ Initially treatment is started with corticosteroids
1mg/kg/day and cyclophosphamide 2mg/kg/day.
◼ Occasionally cyclophosphamide can be given by I.V
route also.
◼ Monitoring of WBC is important in CYC therpy.
◼ Once remission is attained ( after 3-6 months )
treatment can be changed to azathioprine or
methotrexate which are less toxic drugs.

52. .
◼ Corticosteroids can be tappered to a low dose of
5-10 mg/day.
◼ Plasmapheresis may be benificial in severe cases or
pulmonary haemorrhage.
◼ In localized disease treatment with cotrimoxazole is
recommended.
◼ In CRF plasma exchange is benificial.

53. .
◼ Other drugs used are:
◼ Mycophenolate mofetil
◼ Antithymocyte globulin
◼ Rituximab
◼ Some patients with severe subglottic stenosis
tracheostomy is required to maintain airway.

54. Prognosis
◼ About 60% of the patients die within a year of
contacting the disease and 90% die within 2 years.
◼ 50% of the patients have been reported a have
relapse after proper medical management.

55. Idiopathic Midfacial Pleomorphic Granuloma
(Stewart’s Type)
◼ Most commonly seen in elderly people in 6th and 7th
decade of life.
◼ Seen more in white people.
◼ Males are more affected than females ( 4:1 )

56. ,
◼ Friedmann (1964) described pleomorphic granuloma
as a “ non healing or malignant granuloma of the
nose, an autoimmune disorder”.
◼ It is a multinating process that progressively destroy
the nose, paranasal sinus, palate.

57. Clinical features
◼ Clinical features can be described in to three stages:
◼ Prodomal stage of nasal stuffiness with serous
rhinorrhoea.
◼ Active stage with nasal obstruction caused by granulation
tissue covered with crusts.
◼ Terminal stage with wide spread invasion sometimes
toxemia and death.

58. .
◼ Ulceration can spread and destroy the soft tissue, cartilage
and bone.
◼ The ulcerated mucosa is covered by a sticky black or
brownish yellow crusts, removal of which reveals granulation
tissue.
◼ Exposure of bone may lead to formation of sequestra while
ulceration of the septum and turbinate may spread rapidly
through the nose involving the hard palate which may at
times perforate.
◼ Bacterial infection may lead to inflammatory odema of the
lips, cheek and subcutaneous abscess may follow.

59. .
◼ Extensive destruction may result in exposure of the roof of
the maxillary sinus, involvement of the orbit and loss of tooth.
◼ Similar destruction may involve the nasopharynx, oropharynx
and hypopharynx.
◼ Cachexia, haemorrhage and recurrent infection may lead to
death of the patient.

60. Histopathology
◼ Microscopic picture of polymorphic reticulosis is
seen
◼ Various pattern can be seen :
◼ Non specific pleomorphic cellular granulation tissue
containing waves of fibrous tissue.
◼ Non specific granulation tissue with histiocytes
predominating.
◼ Non specific granulation tissue with necrotising changes
predominating.

61. .

62. Investigation
◼ Biopsy is mandatory if the results are equivocal then
biopsy should be repeated.
◼ Radiographic examination of the skull shows bony
destruction and will demonstrate opacity of
paranasal sinus.
◼ Haematological examination shows
◼ Increased ESR
◼ Blood picture reveals
◼ Microcytic anemia.
◼ Eosinophilia.
◼ Chest x-ray shows periarteritis nodosa.

63. .
◼ Urinalysis may show presence of red blood cells
albumin, casts.
◼ Serum studies to exclude syphilis.

64. Treatment
◼ Radiotherpy is the choice of treatment.
◼ Surgery is required for biopsy and debridement if
required.
◼ Corticosteroids
◼ Prednisolone 60 mg .
◼ Dexamethasone 8mg in three divided doses.

65. Thanks you