The document discusses several granulomatous diseases that can affect the nose and paranasal sinuses, including tuberculosis, leprosy, syphilis, rhinoscleroma, rhinosporidiosis, and Wegener's granulomatosis. It provides details on the causative organisms, clinical presentations, diagnostic approaches, and treatment options for each condition. The diseases are classified as infectious, inflammatory, or neoplastic in etiology and can cause nasal obstruction, crusting, ulceration, and deformities of the nose if left untreated. Accurate diagnosis involves microbiological testing, histopathological examination of biopsy specimens, and imaging studies.

1. Granulomatous Diseases of Nose
andPNS
•Presenter
Dr Amit Jha
1st year PG Resident
ENT-HNS

2. Granulomatous diseases of
Nose and PNS
Moderator – Dr Md. Kamaluddin Rain
Lecturer , ENT-HNS , National Medical College
Birgunj

3. Clinical Presentation
•It has 50 Slides and it will take around 45 minutes
•System – Respiratory System

4. Objectives :
•General objectives :
1) To understand about different granulomatous
diseases of nose

5. Specific objectives :
1) Definition of Granuloma
2) Types of Granulomatous diseases of nose
3) Clinical features and Management of
Granulomatous diseases of Nose

6. Contents
• Definition
• Classification
• Tuberculosis of nose
• Leprosy diseases
• Syphilis
• Rhinoscleroma
• Rhinosporidiosis
• Wegener’sgranulomatosis

7. Focal area of chronic
inflammation produced by
circulating monocytes as
part of an immunologic
process (Scott and Brown’s
Otolaryngology 8th edition )
Granuloma

8. Classification

9. 1. Infective
• Bacterial
– T
uberculosis, Lupus vulgaris, Leprosy, Scleroma,
Syphilis, Actinomycosis
• Fungal
– Rhinosporidiosis , Aspergillosis, Mucormycosis,
Candidiasis, Histoplasmosis, Blastomycosis
• Protozoal -Leishmaniasis
• Viral -AIDS

10. 2. Inflammatory
– Sarcoidosis
– Wegener’s granulomatosis
– Eosinophilic granuloma, Chrug Strauss syndrome
(Allergic granulomatosis), Cholesterol granuloma ,
Foreign body granuloma
3. Neoplastic
– Tcell lymphoma , Stewart’sGranuloma

11. • Associated with primary pulmonary tuberculosis
anterior part of inferior
• Affects anterior septum and
turbinate
• C/F :
– Nasal obstruction, discharge, pain
– Red, nodular thickening, ulceration +/-
– Perforation /scarring of the cartilagenousseptum
Tuberculosis

12. Lupus vulgaris (Cutaneoustuberculosis)
• Indolent and chronic form of
tuberculous lesions of the skin of
nose
• May cause epitheliomatous
reaction of squamous epithelium
• Apple jelly nodules ondiascopy
• Scarring more severe

13. Diagnosis
• History of previous TBor active pulmonary TB
• Mantoux test : Skin test for delayed hypersensitivity
by injection of purified proteinderivative
• Microscopy : Ziehl-Neelsen stain
• Culture (6-8 weeks) : Lowenstein -Jensen medium,
Middlebrook's medium
• Polymerase chain reaction (PCR)
• Histopathological examination of biopsyspecimen

15. Treatment
• 2 months course of therapywith
– Isoniazid (5 mg/kg)
– Rifampin (10 mg/kg)
– Streptomycin (15 mg/kg ) / Ethambutol (15 mg/kg)
• 6 more months of isoniazid & rifampin
• 2 HRE+6HR
• Surgical reconstruction

16. Leprosy (Hansen’s Disease)

17. • Organism: Mycobacterium leprae
• Nasal Findings:
– Excessive nasal discharge, red swollen mucosa,
crusting & bleeding
– Nodular thickening of mucus membrane, ulcer,
perforation of cartilaginous septum
– Leonine facies: (characteristic of lepromatous )
nasal destruction, lack of eye brow & eyelashes,
altered pigmentation & ocularalterations

19. • Late sequelae:
– Atrophic rhinitis
– Saddle nose deformity
– Destruction of anterior nasalspine
– Retraction of columella
• Diagnosis: scraping of mucosa and biopsy, acid fast
bacilli in foamy appearing histiocytes (lepracells)
• Treatment:
– Dapsone, Rifampicin, Clofazimine

20. Syphilis

21. T
reponema
• Organism :
pallidum
• Endarteritis
blood vessels
of small
with
secondary hypertrophic
changes in endothelium
leads to endarteritis
obliterans and luminal
obliteration

22. Congenital syphilis
• Nosealways involved
• In Infants :
– ‘Snuffles’ 3 weeks -3 months
– Simple catarrhal rhinitis becomes purulent
with secondary fissuring and
excoriation of nasal with suckling and
vestibule, upper lip
– Nasal obstruction interferes

23. Snuffles

24. Intraoral mucous patches and facial skinlesions in
congenitalsyphilis

25. • At puberty
– Gummatous & destructive
lesions in the mucous
membrane, periosteum &
bone of nasal septum
– Ulceration & destruction
secondary
leads to
atrophic rhinitis and
saddle nose deformity

26. Tertiary syphilis
• Most commonly involves the
nose
• Pathological lesion: Gumma
• Begins as a subcutaneous
nodule, progresses to involve
overlying skin and breaks
down to form punched out
destructive ulcer

27. • Posterior bony septal perforation and extensive
necrosis of nasal and facial tissues  Saddle nose
deformity
• Early symptoms:
– Pain over the nasal bridge (worse at night), swelling
and obstruction
– Offensive discharge, bleeding, crusting, anosmia
(secondary atrophic rhinitis)
• Severe scarring of nose

28. Saddle nose deformity Hard palate perforation

29. T
reatment
• Parenteral penicillin: drug of choice for allstages
– Benzathine penicillin G(2.4 IUIM, singledose)
• Local treatment
– Clearance of crusts and regular cleansing by
copious alkaline douches (1-3 times aday)
– Yellow mercury oxide ointment applied locally
• Reconstructive surgery

30. Rhinoscleroma (Respiratoryscleroma)
• Progressive granulomatous disease commencing in nose and
extending into the nasopharynx, oropharynx, larynx ,
sometimes to trachea and bronchi caused by Klebsiella
rhinoscleromatis (Frisch bacillus)

31. Stages
• Catarrhal : Foul smelling purulent rhinorrhea persisting for
weeks to months
• Atrophic : Foul smelling large nasal plaques or crusts
(simulates atrophic rhinitis )
• Granulomatous (proliferative/nodular) :
– Granulomatous nodules enlarge and coalesce (Tapir nose)
– Pathologic changes most characteristic
– K. rhinoscleromatis most frequently isolated
• Cicatrizing :Adhesions and stenosis distort normal anatomy
(Hebra nose)

32. Tapir nose Hebranose

33. Diagnosis
• High index of suspicion :Coalescent, enlarged granulomatous nodules
at or near thenasal vestibule, diffuse andbilateral
• Microscopy : Silver impregnation stains, Wartin -
Starryor Giemsa stain
• Culture of infected tissue: K.rhinoscleromatis in98%
• Complement fixationtest
• Biopsy showing characteristic histologicalfeatures

34. Histopathology
• Mikulicz cells
– Scattered large foam cells with a
central nucleus and vacuolated
cytoplasm containing bacilli
(transformed macrophages that
have ingested the bacillus)
• Russel bodies
– Resemble plasma cells with an
eccentric nucleus and deep eosin
staining cytoplasm

35. Treatment
• High dose of bactericidal antibiotics
– Minimum of 4-6 weeks , continued until 2 consecutive
cultures from biopsy material are provennegative
• Streptomycin (1 g/day), Tetracycline(2g/day)
• Rifampicin, Sulphamethoxazole – trimethoprim, Ciprofloxacin
• Local application of 2% acriflavin for 8weeks
• Irradiation: 3000-3500 Gyover 3 weeks
• Surgical debridement
• Reconstructive surgery

36. Rhinosporidiosis
• Chronic granulomatous infection that affects the nasal mucosa
(70%), ocular conjunctiva and other mucosa
• Organism: Rhinosporidium seeberi
• Common in Eastern terai belt of Nepal (Janakpur, Rajbiraj)
• Contacted by immersion in contaminated water of ponds
containing animal dungs

37. Clinical Features
• Insidious onset of painless, gradual nasalobstruction
• Nose
– Large sessile or pedunculated lesions
– Leafy, papillomatous or proliferative lesions studded with
white dots
– Pink, red or purple in colour
• Vascular and bleed on touch

39. • Histopathology (Diagnostic)
– Pseudoepitheliomatous squamous cell metaplasia
overlies numerous multisized, microscopic globular
and an
tissue,
cysts called sporangia with thick wall
operculum
– Large sporangia filled with endospores
– Granulomatous reaction of fibrous
neutrophils, plasma cells, and lymphocytes

40. Treatment :
• Complete surgical excision with cauterization of thebase
• Dapsone
– 100mg/day for 6 months
and accentuated
– Arrest maturation of sporangia
granulomatous response with fibrosis
• Amphotericin B
• Medical management is not veryeffective

41. Wegener’s granulomatosis
(Rhinogenic granulomatosis)

42. • Triad of
–Granulomatous inflammation of the upper
and lower respiratory tract
–Necrotizing vasculitis affecting
smalmedium sizedvessels
– Necrotizingglomerulonephritis

43. • Etiology
–Inflammatory, hypersensitivity reaction with
an immune response
–Deposition of immune complex leads to
vasculitis

44. T
ypes
serosanguinous
crusting , septal
• Type1: Limited to nose
– Chronic nonspecific rhinitis,
nasal discharge , nose pain,
perforation
• Type2: More systemic symptoms
– Hemoptysis, cavity in chest
• Type3: Widely disseminated
– Multiple organ failure

45. • C/F
– Progressive malaise, pyrexia, weight loss
– Nose and sinuses involved in > 80 %
• Mucosal edema , Nasal obstruction, sinusitis
• Crusting
• Septal perforation
• Saddle nose
– Palatal ulcers/ perforation

46. Palatal changes

47. • Oral cavity: hyperplastic granular lesion of gingiva,
ulcerative stomatitis
• Ocular symptoms: conjunctivitis, episcleritis , corneal
ulceration, optic neuritis, retinal artery occlusion,
proptosis (20%)
• Pulmonary symptoms: cough, hemoptysis , pleuritic
pain
• Renal symptoms (30-90%): hematuria

48. • Otological symptoms: Recurrent AOM, OME
• Laryngeal and tracheal symptoms: unusual, most
commonly subglottic & uppertrachea
• Central nervous system: (10-15%)
• Others: ulceration of skin in distal arms & legs,
polymyalgia, poly arthritis

49. Investigations
: Raised
: +ve
• Erythrocyte sedimentation rate (ESR)
• C-reactive protein (CRP)
• C– ANCA: positive in 95% -60% if localizeddisease
• Serum angiotensin converting enzyme (SACE): Raised
• Blood urea, Serum creatinine
• Urine RE/ME
• Chest X-ray/ CTchest : Nodular lesions withcavitation
• Tissue biopsy : typical histological features

51. • CT/ MRI:
– Non-specific mucosal thickening in nose or
PNS (86%)
– Evidence of bone destruction (75%)
– New bone formation in walls of sinus(50%)
– Orbit affected (30%)
– MRI shows fat signal from sclerotic sinuswall
– Chest may show progressive cavitation and fibrosis

52. • Histological features
– Fibrinoid vascular necrosis
– Granulomas are epithelial cell type (large,irregular
and lined with histiocytes)
– May show fibrinoid necrosis or benon-necrotic
– Multinucleated giant cells +eosinophils

53. Criteria for the diagnosis of Wegener’s granulomatosis
Criteria Definition
Nasal or oral
inflammation
Painful or painless oral ulcers or purulent or
bloody nasal discharge
Abnormal chest x-ray
Presence of nodules, fixed infiltrates orcavities
Urinary sediment
Microhematuria (over 5 RBC/HPF) or red cell
casts in urine sediment
Granulomatous
inflammation onbiopsy
Histology showing granulomatous inflammation
within wall of artery or inperi or extravascular
area
Any 2 or more of four criteria

54. Treatment
• Prednisone (1 mg/kgper day) + Cyclophosphamide (2 mg/kg
per day) for 1 month
– Prednisolone tapered to alternate days for 2 months 
stopped after achievement of completeresponse
– Cyclophosphamide continued for 6 months to 1 year 
tapered over a few months
• Trimethoprim - Sulphamethoxazole, Azathioprine (200
mg/day)
• Methotrexate, Cyclosporin, Rituximab

55. Other treatment options
• Trimethoprim - sulphamethoxazole
• Azathioprine (200 mg/day)
• Methotrexate
• Cyclosporin
• Rituximab

56. •Any Questions ?

57. References :
1. Scott and Brown’s Otolaryngology 8th edition
vol I ,vol II
2. Operative otolaryngology Head and Neck –Eugene
N Myers vol I
3. Diseaes of Nose ,Throat , Ear – Logen Turner
4. Text book of Otolaryngology and head and neck
surgery -Byron &Bailey
5. An atlas of head & neck surgery-Lore’ 3rd edition

58. •THANK YOU