The document discusses various granulomatous diseases affecting the upper respiratory tract, including Wegener's granulomatosis, sarcoidosis, Churg-Strauss syndrome, and other infectious systemic diseases such as tuberculosis and leprosy. It provides detailed information on the etiology, clinical features, histology, diagnosis, and treatment options for these conditions. The significance of granuloma formation as a response of the immune system to indigestible agents is emphasized throughout the document.

1. Granulomatous conditions in
ENT

2. Granuloma formation
• Response of immune system to an indigestible agent
• Usually neutrophils remove agents by phagocytosis &
digestion
• Macrophages phagocytose indigestible agents, loose their
mobility & accumulate at site of injury
• Undergo structural changes: Larger with more cytoplasm 
epitheloid cells
• Epitheloid cells fuse  Multinucleated giant cells
• Nuclei arrange in horse shoe pattern  Langerhans giant
cells
• All these cells are surrounded by a collar of lymphocytes

3. Granuloma

4. Granuloma
• Noncaseating or Caseating
– Central area of necrosis
– “Cheese-like” appearance
– Often visible macroscopically

5. Granulomatous diseases
• Infective
• Inflammatory
• Neoplastic
• Forms part of a spectrum of conditions in which
vasculitides play a role
• Many are systemic conditions
– Preferentially target or present in the upper
respiratory tract

6. Granulomatous diseases

7. INFLAMMATORY CONDITIONS

8. Wegener’s Granulomatosis
• Relatively common disease of upper airway
• Friedrick Wegerner
– 1939: Necrotizing granulomas and vasculitis of upper
& lower airway, occurring either together or as
separate components
• Coexistence of vasculitis & granulomas
• Classically involves triad:
– Airway
– Lung
– Renal disease

9. Wegener’s Granulomatosis
• Aetiology = unknown
• Hypersensitivity reaction to an unknown
stimulus
• Affects all ages and both males & females

10. • 3 main forms of WG
– Type 1
• Limited form
• Symptoms of URTI persisting for weeks & not responding to
A/b
• Pain over the dorsum of the nose
• Epistaxis & variable degree of nasal obstruction
• Very large crusting of the nasal cavities & nasopharynx
bilaterally
• When crusts are removed – mucosa is very friable
• Septal perforations & eventual nasal collapse in advanced
disease
Wegener’s Granulomatosis

11. Wegener’s Granulomatosis
• Early changes
– Angry mucosa
– Mucous hypersecretion

12. Wegener’s Granulomatosis
• End result of advanced
disease

13. Wegener’s Granulomatosis
• End result of advanced
disease

14. Wegener’s Granulomatosis
• End result of advanced
disease
– Infiltration of orbit
– Sclerosis and opacification of
nasal cavity
– Septal erosion

15. Wegener’s Granulomatosis
• End result of advanced
disease
– Infiltration of orbit
– Septal destruction
– Hyperostosis
– Opacification of sinuses

16. • 3 main forms of WG
– Type 2
• Sicker patient with more systemic symptoms
• Pulmonary involvement
– Cough & pleuritic pain
– Haemoptysis & cavitating lesions on CXR
– Encapsulated lung abscess
Wegener’s Granulomatosis

17. • 3 main forms of WG
– Type 3
• Widely disseminated with involvement of multiple organs
– Airway
» Short segment of subglottic/upper tracheal stenosis
– Pulmonary
– Renal
» Hematuria & abn urinary sediment & segmental/diffuse
glomerulonephritis
– Cutaneous
» Tick-bite like lesions on the distal limbs
– Oral
» Hyperplastic granular lesion of gingiva; ulcerative stomatitis
– Otological
» OME ± mastoiditits & profound SNHL
– Eye
» Conjunctivitis; Dacrocystitis; Episcleritis; Orbital pseudotumour
Wegener’s Granulomatosis

18. Wegener’s Granulomatosis

19. Wegener’s Granulomatosis
• Diagnosis
– cANCA
• 95% sensitive in generalized disease
• 60% sensitive in localized disease
– FBC, ESR, CRP, CXR, Urine analysis for casts
– Histology: Tissue from septum & all turbinates
– CT nose & paranasal sinuses
• Non-specific mucosal thickening; bony destruction; new
bone formation

20. Wegener’s Granulomatosis

21. Wegener’s Granulomatosis
• Main histological features:
– Vasculitis involving medium & small vessels
– Granulomatous necrosis (can be non-necrotic);
Large epitheloid cells; lined with histiocytes
– Scattered multinucleated giant cells

22. Wegener’s Granulomatosis
• Treatment:
– Prednisone 1mg/kg/day + Cyclophosphamide 2mg/kg/day x
1/12
• Prednisone tapered to alternate days x 2/12 & then discontinued once
complete response achieved
• Cyclophosphamide continued x 6/12 to 1 year & then tapered over a
few months
– Other
• Azathioprine
• Methotrexate
• Plasma exchange Ig infusion
– Nose
• Topical steroids, nasal douching & irrigation
– Surgical reconstruction
• Wait until disease has been in remission for some time

23. Sarcoidosis
• Systemic condition of unknown aetiology
• Can involve any organ including many H&N structures
• Incidence = 64/100,000 in Scandinavia
• Condition of young adults between 3rd & 5th decade
• Female preponderance = 2:1
• Ratio of black to Caucasian = 12:1
• Nasal manifestation almost always part of multisystem
sarcoid which have been present for some years

24. Sarcoidosis

25. Sarcoidosis
• Histology
– Epitheloid cells surrounded by lymphocytes &
fibroblasts, but devoid of caseation
– Crystalline or calcified inclusion bodies sometimes
seen (Schaumann bodies)

26. Sarcoidosis
• Nasal involvement
– Presenting complaints

27. Sarcoidosis
• Nasal involvement
– External
• Lupus pernio
• Predilection for cold
sensitive areas, such as
tip of nose

28. Sarcoidosis
• Lupus pernio of the
cervical skin

29. Sarcoidosis
• Nasal involvement
– Mucosa
• Granular appearance (“Strawberry skin”)
– Tiny pale granulomas against a erythematous mucosa
• Very friable mucosa
• Crusting, nasal congestion, mucopurulent discharge
• Anterior septal perforation & later nasal bridge collapse
• Paranasal sinus involvement & infection

30. Sarcoidosis
• Nasal involvement
– Sclerosis & osteolysis of
nasal bones
– Soft tissue mass in nose

31. Sarcoidosis
• Salivary glands
– Bilateral non-tender, firm, smooth parotid swelling
• Lymphoid hyperplasia
– Adenoids – OSA; OME
• Supraglottic larynx
– Dyspnoea; dysphonia
– Sub sites
• Epiglottis  arytenoid  aryepiglottic folds  false
cords

32. Sarcoidosis
• Laryngeal involvement

33. Sarcoidosis
• Laryngeal involvement

34. Sarcoidosis
• Diagnosis
– Combination of histology, imaging & haematology
– Exclude other causes for non-caseating
granulomatous changes
– ↑ serum ACE (83% of patients with active sarcoid)
• Also in TB, Leprosy, 1ᴼ biliary cirrhosis
–  ESR, CRP & urinary calcium
– Mild anaemia, leucopenia, thrombocytopenia,
eosinophilia

35. Sarcoidosis
• Treatment
– Oral steroids, Methotrexate ± Hydroxychloroquine
– Topical intranasal steroids (spray/drops)
– Nasal douching/irrigation
– Surgery contraindicated for both cosmetic &
functional indications

36. Churg-Strauss Syndrome
• 1st described 1951 by Churg & Strauss
– Syndrome of systemic vasculitis & asthma
• Eosinophil-rich & granulomatous infl involving
the resp tract & necrotizing vasculitis affecting
small to medium sized vessels & associated
with asthma & eosinophilia

37. Churg-Strauss Syndrome
• 3 phases
– Prodromal phase
• May persist for years
• Allergic disease (allergic rhinitis, nasal polyposis,
asthma)
– Peripheral blood & tissue eosinophilia
• Chronic eosinophilic pneumonia & eosinophilic
gastroenteritis
– Life threatening systemic vasculitis

38. Churg-Strauss Syndrome
• Histo
– Necrotizing giant cell vasculitis, interstitial
granulomas & eosinophilic pulmonary infiltrates

39. Churg-Strauss Syndrome
• Nasal involvement
– Usual polyposis
– May also have crusting & septal perforation
– Doesn’t display diffuse mucosal destruction like
with WG

40. Churg-Strauss Syndrome
• Treatment
– Oral steroids
– Polyposis
• Topical steroids ± surgery

41. Giant cell Granuloma
• “giant cell reparative granuloma” or “giant cell
reaction of bone”
• Benign condition of jaws & other craniofacial
sites
• Commonly occur in children & young adults

42. Giant cell Granuloma
• Clinical features
– Maxilla & mandible most commonly affected
–  sphenoid bone  temporal bone
– Pain & swelling over affected bone
– Diplopia; frontal headache; hearing loss; vertigo;
tinnitus

43. Giant cell Granuloma
• Imaging
– Expansile lytic lesions
– “Soap bubble” centre
– Well demarcated edges

44. Giant cell Granuloma
• Cherubism
– Bilateral symmetrical
involvement of jaws
– Rare inherited childhood
condition

45. Giant cell Granuloma
• Cherubism
– Bilateral symmetrical
involvement of jaws
– Rare inherited childhood
condition

46. Cholesterol granuloma
• Granulomatous reaction to cholesterol crystals
precipitated in tissues
• Presumed 2ᴼ to haemorrhage and/or trauma
• Lesions may affect maxilla, frontal sinus,
temporal bone
• Produce expansion of bone, cosmetic
deformity & displacement of adjacent
structures

47. Cholesterol granuloma
• Imaging
– Cyst-like expansion of bone/sinus
– Opaque on CT & not contrast enhancing
• Histo
– Granulation tissue of giant cells surrounding clefts
created by cholesterol granulation
• Treatment
– Surgical excision & complete removal of granulation

48. INFECTIOUS SYSTEMIC DISEASES
BACTERIAL

49. Rhinoscleroma
• Chronic granulomatous disease of the resp tract
– Nose; larynx; trachea & bronchi
• Klebsiella rhinoscleromatis
• Occur @ any age in either sex
– > common in middle aged females
• Central & South-eastern Europe; North Africa;
Indian subcontinent; Indonesia; South America

50. Rhinoscleroma
• 3 stages
– Catarrhal stage
• Foul-smelling purulent rhinorrhoea (weeks – months)
– Atrophic stage
• Large, foul-smelling nasal plaques or crusts
• Same as lesions in atrophic rhinitis
– Granulomatous or proliferative stage
• Multiple granulomatous, non-ulcerative nodules throughout
nose, pharynx, larynx, trachea & bronchi
• Bluish red & rubbery and later paler & harder
– Cicatrizing stage
• Scaring, adhesions, stenosis & distortion of normal anatomy

51. Rhinoscleroma

52. Rhinoscleroma
• Histo
– Scattered large foam cells (Mikulicz cells)
• Vacuolated cells with a central nucleus
• Contain the indigestible bacilli & Russel bodies
• Diagnosis
– Clinical features
– Microbiology
– Histological features

53. Rhinoscleroma
• Treatment
– Treatment must be intense & prolonged
– Streptomycin 1g/day + Tetracycline 2g/day x 4/52
– Continued till 2 x consecutive negative cultures
– Rifampicin, Bactrim & Ciprofloxacin
– Surgical debridement prior to A/b in granulomatous
stage
– Surgery to “core out” scar tissue in Cicatrizing phase

54. Tuberculosis
• Mycobacterium tuberculosis
• Uncommon in nose
• More common in cervical LN, pharynx, larynx
& ear

55. Tuberculosis
• Nasal involvement
– Lupus Vulgaris
• Begins in vestibule, then extends to adjoining skin &
mucosa
• Apple jelly nodules (Does not blanch)
• May form ulcers with undermined edges

56. Tuberculosis
• Lupus vulgaris

57. Tuberculosis
• Nasal involvement
– Lupus Vulgaris
• Begins in vestibule, then extends to adjoining skin & mucosa
• Apple jelly nodules (Does not blanch)
• May form ulcers with undermined edges
– Ulcerative form
• Involves cartilaginous nasal septum or inferior turbinate
• Nasal floor is spared
• Nasal obstruction, pain, discharge, crusting, epistaxis
• Septal perforation, but not dorsal saddling
– Sinus granuloma
• Soft tissue mass with/without bone destruction

58. Tuberculosis
• Sinus granuloma
– Sino-nasal mass
– Surrounding infiltration
– Bone destruction

59. Tuberculosis
• Laryngeal involvement
– Occurs in association with pulm TB
– Dysphonia, Dysphagia, Otalgia
– Diffusely oedematous & reddened larynx
• Predominantly affects post ⅓ of larynx
• May have ulceration (confused with Sq cell CA)

60. Tuberculosis
• Laryngeal involvement

61. Tuberculosis
• Diagnosis
– Histology (AFB)
– CXR
– PCR & BACTEC
• Treatment
– As other TB

62. Nontuberculous Mycobacteria
• Mycobacterium fortuitum
• Mycobacterium scrofulaceum
• Mycobacterium szulgai
• Mycobacterium xenopi
• Mycobacterium bovis

63. Syphilis
• Spirochaete: Treponema pallidum
• Can affect all ages and both sexes equally
• Primary
– Infectious!!
– Chancre / hard non-painful ulcerated nodule
– Self limiting (6 – 10 weeks)
– Diff: furunculosis / malignant neoplasm

64. Syphilis
• Secondary
– Most infectious!!
– Persistent catarrhal rhinitis
– Persistent crusting or fissuring of vestibule
– Mucous patches on tongue, gingiva, pharynx
– Rarely larynx
• Diffuse erythematous papules on larynx
– Epiglottis & aryepiglottic folds

65. Syphilis
• Secondary

66. Syphilis
• Tertiary
– Luckily much less infective….
– Nose
• Pain (always worse @ night), swelling & obstruction
• Tenderness over nasal bridge (characteristic sign)
• Gumma
– Begins as a subcut nodule  punched out destructive ulcer
• Bony portion of septum most comonly involved

67. Syphilis
• Tertiary
– Ulcerated gumma
– Cicatrization of bony
nasal dorsum

68. Syphilis
• Tertiary
– Luckily much less infective….
– Larynx
• Nodular infiltrates coalescing to painless ulcers
– Epiglottis & aryepiglottic folds
• Appearance = similar to TB & CA
– Ear
• SNHL, OM, Meniere’s symptoms

69. Syphilis
• Diagnosis
– TPHA/VDRL / RPR
– Biopsy in doubtful cases
• Treatment
– Parenteral Penicillin

70. Leprosy
• Hansen’s disease
• AFB: Myrobacterium leprae
• Epidemiology
– 12 – 15 million people affected world wide
– Brazil, India, Indonesia, Myanmar, Nigeria: 82% of
cases
– Commonly manifests ages 10-20 years
– Nasal d/c = principal route of transmission

71. Leprosy
• 7th WHO Expert Committee on Leprosy
– Hypopigmented or reddish skin lesion w definite
loss of sensation
– Involvement of peripheral nerves (thickening or
loss of sensation
– Skin smear + for AFB,s

72. Leprosy
• 2 distinct polar forms of disease w spectrum in
between
– Tuberculoid Leprosy:
• Strong host resistance
• Non-infective
• Localized
– Lepromatous Leprosy
• Poor host resistance
• Infective
• Systemic with widespread involvement of tissues

73. Leprosy
• Nasal involvement
– Tuberculoid Leprosy
• Solitary skin lesion w cutaneous anaesthetic patches
• Vestibule skin involvement, but no mucosal
involvement
– Lepromatous Leprosy
• Skin, nerve & mucosal involvement
• Nasal obstruction, crust formation & blood-stained d/c
– Nasal d/c contain AFB’s
• Advanced: Atrophic Rhinitis, septal perforation & dorsal
saddling

74. Leprosy
• Laryngeal involvement
– Only with concurrent systemic illness
– Principal area = supraglottis
• Epiglottis – nodular oedema & ulceration
– Present w dysphonia (muffled voice)

75. Leprosy
• Diagnosis
– Clinical
– Bacteriological examination: Nasal d/c or
scrapings of nasal mucosa
– Histology
• Treatment
– Dapsone (Single drug treatment  resistance)
– Rifampicin & clefozamine – acts rapidly
– Triple therapy = best results

76. Actinomycosis
• Anaerobes: Actinomycoses bovis / israeli
• Chronic suppurative disease
• Poor dental hygiene
• Soft tissue involvement of submandibular or
cervical regions
– Abscess & sinus tract formation
• Secondary laryngeal involvement
– Erythematous, swollen wooden larynx

77. Actinomycosis
• Diagnosis:
– Microbiology
– Classic “Sulphur
Granules” on biopsy
• Treatment
– Penicillin
– Tetracycline

78. INFECTIOUS SYSTEMIC DISEASES
FUNGAL

79. Histoplasmosis
• Histoplasmosis capsulatum
• Granulomatous fungal disease
• Soil from chicken houses / bat & bird faeces
• Extremes of ages
• Mostly Larynx & tongue, but also nose &
paranasal sinuses

80. Histoplasmosis
• Always accompanied pulm involvement
– Cough, chest pain, hoarseness
Diffuse miliary Chronic cavitating

81. Histoplasmosis
• Tongue & Larynx more commonly affected
• Either nodules or ulcers

82. Histoplasmosis
• Tongue & Larynx more commonly affected
• Either nodules or ulcers

83. Histoplasmosis
• Diagnosis
– Biopsy
• Epitheloid / histiocytic granuloma
• Organism seen with methenamine silver nitrate stain
• Treatment
– Amphotericin B

84. Rhinosporidiosis
• Rhinosporidium seeberi
• Asia & Africa
• Immersion in contaminated waters
• Nasal mucosal lesions
– Initial: flat & sessile lesion
– Later: Painless polypoid growth filling the nasal
cavity

85. Rhinosporidiosis
• Clinical manifestation
– Painless slow growing nasal mass
• Friable, polypoid and vascular
• Strawberry appearance
– Nasal obstruction, watery rhinorrhoea

86. Rhinosporidiosis
• Diagnosis
– Histology
• Pseudoepitheliomatous squamous metaplasia overlying
multiple globular cysts (sporangia)
• Prominent accompanied granulomatous reaction of
fibrosis tissue
• Treatment
– No medical management
– Complete surgical excision

87. Aspergillus/Mucor
• Fungal Rhinosinusitis
• Usually in immune-compromised individuals
• Non-invasive fungal sinusitis / rhinosinusitis
• Invasive fungal rhinosinusitis

88. Aspergillus/Mucor
• Non-invasive fungal sinusitis / rhinosinusitis
– Fungus ball
• Only described in adults in the literature
• Immunocompetent patients
• Aspergillus most common
• Tangled mats of hyphae in one or more sinuses
• Extramucosal disease
• Maxillary sinus  sphenoid sinus  other sinuses
• Symptom = Post-nasal d/c , asymptomatic
• CT: heterogeneous opacification
• Surgical removal by endonasal under endoscopic guidance

89. Aspergillus/Mucor
• Non-invasive fungal sinusitis / rhinosinusitis
– Fungus ball

90. Aspergillus/Mucor
• Non-invasive fungal sinusitis / rhinosinusitis
– Allergic fungal sinusitis
• Immunocompetent patients
• Younger patient group < 30years
• Hypersensitivity to fungus residing in mucous
• Dematiaceous species
• “allergic mucin” – eosinophilic mucin with or without fungus
• Unilateral/Bilateral polyps with complete opacification of sinus
cavity on CT
– Frequently bony expansion
• Diagnosis
– Presence of hyphae in mucin
• Treatment
– Prednisone, nasal steroids & removal of all mucin

91. Aspergillus/Mucor
• Non-invasive fungal sinusitis / rhinosinusitis
– Allergic fungal sinusitis

92. Aspergillus/Mucor
• Invasive fungal rhinosinusitis
– Chronic or indolent invasive fungal rhinosinusitis
• Rare
• Mostly in immuno-competent patients
• Aspergillus most frequent
• Pain = Main symptom
• Chronic headache, proptosis, cranial nerve deficits
• Maxillary sinus most commonly affected
• Bony erosion extending to orbit or skull base

93. Aspergillus/Mucor
• Invasive fungal rhinosinusitis
– Acute fulminant fungal rhinosinusitis
• Immuno-compromised patients
• Fatal outcome without prompt treatment
• Mycotic infiltration of mucosa & sinuses
• Initially: Fever of unknown origin or rhinorrhoea
• Later: Proptosis, opthalmoplegia, CN palsies
• Most common site: near middle turbinate, septum
• Thrombosis & necrosis

94. Aspergillus/Mucor
• Invasive fungal rhinosinusitis
– Acute fulminant fungal rhinosinusitis

95. NEOPLASTIC

96. T/NK cell Lymphoma
• “midline destructive granuloma”
• Classical destruction of the midface
• Similar appearance of aggressive midfacial
destruction by cocaine abuse
• All decades of life (mean = 50years)
• Both sexes
• Association with EBV

97. T/NK cell Lymphoma
• 3 stages:
– Prodromal
• Persistent nasal obstruction & rhinorrhoea
– Second stage
• Necrosis around nasal cavity
• Purulent nasal d/c, crusting, tissue loss
– Terminal stage
• Haemorrhage & gross mutilation of face
• Eventually lead to death

98. T/NK cell Lymphoma

99. T/NK cell Lymphoma
• Diagnosis
– Good quality representative biopsy beneath the
slough & crusts
• Imaging
– Dramatic destruction of midline soft tissue & bone
without a gross tumour mass
– Treatment
• Radical full course radiotherapy 55 Gy
• Wide field coverage incl nose, sinuses & palate