granulomatous diseases of nose

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