Presentation prepared and compiled by highly experienced ENT teacher, Dr. Krishna Koirala from Nepal, for teaching undergraduate and postgraduate ENT students in the field of otorhinolaryngology. A clear and concise explanation of the basic concepts in the subject matter concerned. He is the Head of department with a sound knowledge in the field of ENT to teach both undergraduate and postgraduate ENT students

1. Granulomatous Diseases
of Nose
Dr Krishna Koirala
MS (ENT-HNS)
2020-09- 09

2. Focal area of chronic
inflammation produced by
circulating monocytes as
part of an immunologic
process
Granuloma

3. Classification

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

5. 2. Inflammatory
– Sarcoidosis
– Wegener’s granulomatosis
– Eosinophilic granuloma, Chrug Strauss syndrome
(Allergic granulomatosis), Cholesterol granuloma ,
Foreign body granuloma
3. Neoplastic
– T cell lymphoma , Stewart’s Granuloma

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

7. Lupus vulgaris (Cutaneous tuberculosis)
• Indolent and chronic form of
tuberculous lesions of the skin of
nose
• May cause epitheliomatous
reaction of squamous epithelium
• Apple jelly nodules on diascopy
• Scarring more severe

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

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

11. Leprosy (Hansen’s Disease)

12. • 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 & ocular alterations

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

15. Syphilis

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

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

18. Snuffles

19. Intraoral mucous patches and facial skin lesions
in congenital syphilis

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

21. 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

22. • 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

23. Saddle nose deformity Hard palate perforation

24. Treatment
• Parenteral penicillin: drug of choice for all stages
– Benzathine penicillin G (2.4 IU IM, single dose)
• Local treatment
– Clearance of crusts and regular cleansing by
copious alkaline douches (1-3 times a day)
– Yellow mercury oxide ointment applied locally
• Reconstructive surgery

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

26. 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)

27. Tapir nose Hebra nose

28. Diagnosis
• High index of suspicion : Coalescent, enlarged granulomatous
nodules at or near the nasal vestibule, diffuse and bilateral
• Microscopy : Silver impregnation stains, Wartin -Starry or
Giemsa stain
• Culture of infected tissue: K. rhinoscleromatis in 98%
• Complement fixation test
• Biopsy showing characteristic histological features

29. 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

30. Treatment
• High dose of bactericidal antibiotics
– Minimum of 4-6 weeks , continued until 2 consecutive
cultures from biopsy material are proven negative
• Streptomycin (1 g/day), Tetracycline (2g/day)
• Rifampicin, Sulphamethoxazole – trimethoprim, Ciprofloxacin
• Local application of 2% acriflavin for 8 weeks
• Irradiation: 30-35Gy over 3 weeks
• Surgical debridement
• Reconstructive surgery

31. 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

32. Clinical Features
• Insidious onset of painless, gradual nasal obstruction
• 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

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

35. Treatment
• Complete surgical excision with cauterization of the base
• Dapsone
– 100mg/day for 6 months
– Arrest maturation of sporangia and accentuated
granulomatous response with fibrosis
• Amphotericin B
• Medical management is not very effective

36. Wegener’s granulomatosis
(Rhinogenic granulomatosis)

37. • Autoimmune multisystem disorder characterized
by a triad of
–Granulomatous inflammation of the upper
and lower respiratory tract
–Necrotizing vasculitis affecting small to
medium sized vessels
–Necrotizing glomerulonephritis
• Age: 4th – 5th decade
• Sex: M>F

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

39. Types
Type 1: Limited to nose
– Chronic nonspecific rhinitis,
serosanguinous nasal
discharge , nose pain,
crusting , septal perforation
Type 2: More systemic symptoms
– Hemoptysis, cavity in chest
Type 3: Widely disseminated
– Multiple organ failure

40. • 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

41. Palatal changes

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

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

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

46. • 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 sinus wall
– Chest may show progressive cavitation and fibrosis

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

48. 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 or cavities
Urinary sediment
Microhematuria (over 5 RBC /HPF) or red cell
casts in urine sediment
Granulomatous
inflammation on biopsy
Histology showing granulomatous inflammation
within wall of artery or in peri or extravascular
area
Any 2 or more of four criteria

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