rhinosporodiasis wegener's granulomatosis sarcoidosis rhinoscleroma syphillis langerhans cell histocytosis cocaine induced churg strauss syndrome

1. GRANULOMATOUS
DISEASES OF NOSE
Ashish K. Gupta
SMS medical college,jaipur

2. what is granuloma???
Granuloma is a focus of chronic inflammation
consisting of a microscopic aggregation of
macrophages that are transformed into
epithelium-like cells, surrounded by the collar of
mononuclear leukocytes, principally lymphocyte
& occasionally plasma cells.

3. CLASSIFICATION
BACTERIAL
 RHINOSCLEROMA
 SYPHILIS
 TUBERCULOSIS
 LUPUS
 LEPROSY
FUNGAL
RHINOSPORIDIOSIS
ASPERGILLOSIS
MUCORMYCOSIS
CANDIDIASIS
HISTOPLASMOSIS
BLASTOMYCOSIS
UNSPECIFIED
CAUSE
 WEGENER’S
GRANULOMATOSIS
 PERIPHERAL T-CELL
NEOPLASM (NON-
HEALING MIDLINE
GRANULOMA)
 SARCOIDOSIS
 CHURG-STRAUSS
SYNDROME

4. Rhinoscleroma
Causative organism-
Klebsiella rhinoscleromatis or Frisch bacillus.
 Endemic in india (northern)

5. Pathology
Progressive granulomatous lesion begins at the nose and
extends up to nasopharynx, Larynx(subglotic), trachea and
lower airway can also be involved.

6. Clinical features
Atrophic stage – The features resemble that
of atrophic rhinitis, including crust formation
and a foul smelling discharge.
(Carpenter’s Glue)
Granulation / nodular stage – (Sub dermal
infiltration)
Nodules are non ulcerative in nature.
These nodes are initially bluish red and
rubbery and upper lip giving a “woody” feel
Hebra nose-expansion of the anterior
nares and deformity of the upper lip

7. Cicatrizising stage – Adhesions and scarring is a feature
of this stage.
Stenosis distort normal nasal anatomy.
Shape of the nose changes and is classically name the
Tapir’s nose.
There may be glottic stenosis with respiratory distress.

8.  Accumulated inflammatory cells include: Plasma cells, lymphocytes, eosinophils and
scattered large foam cells (Mikulicz cells) & Russell bodies.
 Mikulicz cells- identified morphologically as a macrophage, not a plasma cell.
These foam cells have a central nucleus and a vacuolated cytoplasm containing
bacillli.
 Russell bodies-Homogenous eosinophillic inclusion bodies found in plasma cells.

9. Investigations
 Levin test – complement fixation test based on reaction of
patient’s serum with suspensions of K. Rhinoscleromatis High
titres of antibodies to K. rhinoscleromatis has been
demonstrated in these individuals
 Tissue biopsy is diagnostic
 CT-

10. Treatment
 Usually self limiting course of its own accord ending in the
cicatrizing stage
 Traditionally Used : streptomycin (1gm i.m) and tetracycline (2
g/day) for 4-6wks,
Recently used :
 Oral rifampicin (450mg for a period of 6 weeks),
sulphamethoxazole-trimethoprim combination, and ciprofloxacin.
 Local application of 2 % acriflavin for a period of 8 weeks has been
noted to be both efficacious and nontoxic.
 Intralesional steroids have been tried.

11. Kailasa Regime :
 Carbolic acid (0.2ml) + Glacial acetic acid (0.2ml) + Glycerine (0.4ml) +
10ml distilled water is injected locally as 1 to 2 ml twice weekly at
multiple sites of the lesion.
 Usually 8-10 injections lead to complete regression of granuloma and
restoration of normal nasal patency.
 S/E-chemical necrosis of granuloma.
Irradiation
 - Total dose of 3000-3500 Gy over three weeks

12. Syphillis
Can affect any age neonate to elderly.
Causetive organism-Treponema pallidum
Two types-
I.Congenital
II.Acquired
 1. Primary syphilis
 2. Secondary syphilis
 3. Tertiary syphilis

13. Hereditary/congenital syphilis
 Any of lesions of secondary and tertiary forms of syphilis of
nose may occur
 In infants- snuffles most common
 First appears as simple catarrhal rhinitis, then becomes
purulent with secondary fissuring and excoriation of nasal
vestibule and upper lip.
 Obstruction may interfere with suckling and nutrition.
 Gummatous and destructive lesions occur at puberty.

14. • Bulging of the frontal bones and
depression of the nasal bridge ("saddle
nose"), both due to periostitis
• Rhinitis from weeping nasal mucosal
lesions ("snuffles")
• Circumoral rash.

15. Hutchinson's triad
 Interstitial keratitis, sensory-
neural deafness and hutchinson's
teeth.
 Upper central incisors peg-
shaped and widely spaced.

16. Primary Syphillis
 Also known as Sore/chancre develops at site of inoculation with regional lymphadenitis.
 occur on external nose or inside vestibule
 Hard, non painful, ulcerated papule, associated with rubbery non tender nodes
 Self limiting lesion
 Diagnosis-
 Smears from ulcer or node examined by dark ground illumination shows the
spirochaete, Treponema pallidum.
 Serological tests-
VDRL
TPHA
FTA-ABS
Biopsy

17. Secondary syphillis
 Most infectious
 Manifests 6-10weeks after inoculation
 Clinical symptoms-
 Simple catarrhal rhinitis
 Crusting and fissuring of nasal vestibule
 Secondary syphilis rarely recognized in the nose, as
mucous patches hardly ever occur on such a thin, attenuated
mucous membrane.
 Diagnosis suggested by appearance of other secondary lesions-
mucous patches in pharynx, roseolar or papular rash, pyrexia,
enlargement of lymph nodes.

18. Tertiary syphilis
 Only 1/3rd of cases of secondary syphilis progress to tertiary
stage.
 Pathological lesion- gumma- begins as subcutaneous nodule,
progresses to involve overlying skin, and then breaks down to
form a punched out destructive ulcer.
 Bony portion of septum most commonly involved
 Lateral nasal wall, frontal sinus, nasal bones, floor of nose
may be involved late.

19. Ulcerated Gumma
Cicatrization of bony nasal dorsum

20.  Symptoms- pain, swelling, obstruction, offensive discharge,
crusting, bleeding, anosmia.
 Tenderness over bridge nose is characteristic sign
 Sequelae- collapse of bony support of nose, atrophic rhinitis.
 Diagnosis- characteristic organ involvement, histopathology,
serological tests, response to antibiotics

21. Treatment
 Penicillin is the drug of choice:
benzathine penicillin 2.4 million units i.m. every week for 3
weeks with a total dose of 7.2 million units.
 Nasal crusts are removed by irrigation with alkaline solution.
 Bony and cartilaginous sequestra should also be removed.
 Cosmetic deformity is corrected after disease becomes
inactive.

22. Complications
Vestibular stenosis
Perforations of nasal septum and hard palate
Secondary atrophic rhinitis
Saddle nose deformity.

23. NASAL TUBERCULOSIS
 Cause-Mycobacterium tuberculosis.
 Always associated with primary pulmonary tuberculosis
 Modes of infection-Inhalation(Most common),ingestion and
inoculation.
 Most common affecting form is-Lupus vulgaris/cutaneous
tuberculosis.

24. Lupus Vulgaris
 Associated with painfull skin lesion most often on face around
nose,eyelids,lips,cheek,ear,neck.
Macroscopic apperance-
1.Nodular foam (MC)
2.Ulcerative foam
3.Sinus granuloma

25. Clinical features-
 Nasal discharge / obstruction
 Presence of non foul smelling crusts
 Epistaxis
 Ulceration of nasal mucosa is followed by fibrosis, and contraction of
nares

26.  Apple jelly nodules –
Early lesion.
Reddish brown nodule at the
mucocutaneous junction of nasal
septum.
 Cartilagenous portion of nasal
septum undergoes destruction

27. Diagnosis lupus nodules:
 Blanching on pressure- When pressure is applied to adjoining
nasal mucosa using glass slide, pinkish nodules will stand out
because the adjacent areas undergo blanching
 Tissue biopsy- epitheloid cell granulomas, features of
caseation, AFB.
 Direct demonstration or culture of organism
 DNA amplification by PCR
 Monteaux test
 Associated pulmonary tuberculosis- chest x-ray

28. Treatment
Anti tubercular Therapy

29. Leprosy
 cause Mycobacterium leprae
 Mode of transmission- via nasal secretions or skin.
 The nose is involved as a part of systemic disease, more often in the
lepromatous than tuberculoid or dimorphous forms of disease.

30. Tuberculoid Type
 Skin lesion extend upto vestibule
 Cranial nerve palsy (Vth and VIIth)

31. Lepromatous Type
 These nodules are commonly seen in the
anterior end of inferior turbinate
 Nasal obstruction.
 Collapse of the anterior bridge of the nose with
destruction of anterior nasal spine is a feature.
 Both bony and cartilagenous portions of nasal
septum are destroyed,Saddle nose deformity.
Nasal crust formation, serosanguinous nasal discharge.
Presence of nodular thickening of nasal mucosa is the earliest feature.

32. Diagnosis
 Skin lesions, thickened nerves
 Demonstration of M. leprae on microscopy of the nasal
discharge or scrapings of nasal mucosa
 Histopathology of nasal mucosa.
 Skin biopsy may be required to demonstrate the bacillus.

33. Treatment
 Triple therapy-
 rifampicin 600 mg on the first two days of each month
 clofazimine 100 mg on alternate days three times a week, and
dapsone 100 mg daily.
 Direct intranasal administration of rifampicin
 Cosmetic surgery.

34. Rhinosporodiasis
 It is a chronic granulomatous disease caused by Rhinosporidium seeberi
which predominantly affects the mucous membrane of the nose and
nasopharynx.
 characterized by formation of papillomatous and polypoid lesions.
It has also been described
as a strawberry like
mulberry mass.

36. Incidence / geographical description
More than 90% of cases have been reported from India / Sri Lanka and
Pakistan.
In India disease is more common in southern states.
 It is prevalent in the states of Tamilnadu, Kerala, Madhya Pradesh,
Chhattisgarh, and Andhra Pradesh (Madurai, Ramnad, Rajapalayam, and
Sivaganga are endemic zones in Tamilnadu.)
Age: B/W 20 -40yrs
 M:F 4:1

37. The mode of infection
 Stagnant pools of fresh water
 Dust from the dung of the infected horses and cattle.
 Contact transmission by contaminated finger nails.

38. Etiologic agents
 Rhinosporidium seeberi
 Initially believed to be sporozoan
 Classified under fungus
 Recently placed under a protozoa or a fish parasite

39. Life cycle
 Spore is the basic infecting unit.
 Life cycle Of Rhinosporidium seeberi is completed in 3
stages. These are as follows:
1. MATURATION OF TROPHOCYTE
2. DEVELOPMENT OF SPORANGIA AND ENDOSPORES
3. RELEASE OF ENDOSPORES

41. Clinical features
 Mass in the nose 72 %
 Nasal obstruction 66%
 Bleeding from the nose 28%
 Nasal discharge 24%
 Change in voice 2-4%
 Headache 3-6%
 It present as a leafy, friable , bright pink to purple in
colour, highly vascular , polypoid mass in the nose
attached to nasal septum or lateral wall.

42.  Lateral wall 44%
 Nasal septum 36%
 Nasal floor 6%
 Nasopharynx 2%
 Multiple sites 12%
 Growth may extend posterior up to nasopharynx, oropharynx, and
anteriorly may hang up to lips .
Site distribution

43. Diagnosis
 Microscopically examination of nasal discharge will show
spores.
 Biopsy and histological examination shows several
sporangia, oval or round in shape and filled with spores
which may be seen bursting through it chitinous wall.

44. 1 Mature sporangium
2 Spores
3 ImmatureSporangium

45. Treatment
Surgical : Complete excision of the mass with diathermy knife and
cauterization of it base. Recurrence is not uncommon.
Medical : Recently Dapsone (diaminodophenylsulphone) has been
shown to be effective in controlling Rhinosporidiosis.
100 mg/day – 6 months

47. Aspergillosis
CAUSATIVE ORGANISMS
 Aspergillus niger
 Aspergillus fumigatus or Aspergillus flavus
Aspergillosis invades the nasal tissues
 in those who handles doves and other captive
birds
 when host’s defence mechanism are
compromised

48. Clinical features
 SYMPTOMS
 Nasal obstruction
 Sneezing and watery
 Mouldy – smelling discharge
 On examination
 The nasal mucous membrane is covered with greyish
(fumigatus) or black (niger) false membrane.
 The infection usually invades the antrum.
 Exploration of maxillary sinus reveals a fungus ball containing
semisolid cheesy-white or blackish material.

49.  DIAGNOSIS
 The organism can be seen on
culture examination of membrane
and special staining for fungus.
 TREATMENT
 The specific treatment consists of repeated cleaning and local
application of 1% aqueous solution of gentian violet and nystatin.
 Antifungal AMPHOTERICIN B may be given systemically.
 When the sinuses are involved, operative clearance should be
performed.

50. Sarcoidosis
Chronic systemic disease of unknown etiology which may
involve any organ with non- caseating (hard) granulomatous
inflammation
 Female preponderance 2:1
Etiology-
Unknown
immunological response to antigen (include mycobacteria, fungi,
chemicals{Exposure to Beryllium, zirconium, pine pollen and
peanut dust})

51. Clinical Features

52.  Nasal mucosa gives a characteristic
appearance- strawberry skin because of the
tiny pale granulomas against hypertrophic
erythematous mucosa.
 Anterior nasal septum(MC) may perforate,
collapse of nasal bridge may occur.
 Lupus pernio thickening and purplish
discoloration of the overlying skin.
 Salivary gland enlargement is seen in
5–10% of cases

53. Pulmonary involement Staging
 Stage I is bilateral hilar lymphadenopathy,
 Stage II is hilar lymphadenopathy and pulmonary infiltrates,
 Stage III is pulmonary infiltrates alone
 Stage IV is pulmonary fibrosis

54. Diagnosis
 Kveim test- withdrawn due to fears of
virus and prion transfer
 ACE, ESR, serum globulin and serum and
urinary calcium levels
 CXR, CT
 Biopsy
Coronal CT scan showing
sclerosis and osteolysis of the
nasal bones associated with a
soft tissue mass in nasal
sarcoid.

55. Treatment
 Limited disease- spontaneous resolution without specific
treatment
 Oral steroids, methotrexate, hydroxychloroquine
 Topical intranasal steroids, nasal douching.
 Recent interest in TNF-alpha inhibitors such as infliximab and
etanercept.
 Symptomatic laryngeal disease may be treated with transoral
laser and intra-lesional steroid injections.

56. WEGENER’S GRANULOMATOSIS
 Granulomatous inflammation involving the respiratory
tract and necrotizing vasculitis affecting small- to
medium-sized vessels (e.g. capillaries, venules,
arterioles and arteries) with necrotizing
glomerulonephritis.
 Triad of airways, lung and renal disease.

57.  Age of presentation: 15-75yrs
 Aetilogy unknown
 Hypersensitivity reaction with immune response to unknown
stimuli (inhaled bacteria such as S.aureus).

58. The European Vasculitis Study Group classification
Localized
 Respiratory tract
involvement only with no
features of systemic
vasculitis
Early systemic
 generalized disease

59. Clinical Features
 The head and neck is the most common site of involvement at
presentation, in 73–93% of cases.
 Nose and sinuses are involved in more than 80%
 Nasal obstruction, crusting, discharge and bleeding
 Destruction of the intra-nasal structures may follow, including the
septum, turbinates and sinuses with formation of a single large
cavity.

60.  Otitis media with effusion with associated
CHL
 Sensorineural hearing loss also occurs
in 35%.
 Facial nerve paralysis has been
recorded in 8–10%
 Subglottic or upper tracheal stenosis
occurs in 16% of cases
 Oral symptoms are rare but ulceration
may be seen and ‘strawberry’ gingival
hyperplasia is said to be
pathognomonic

61. Diagnosis
 cANCA (positive in 95% cases)
 CBC, ESR, CRP, ACE, serum urea and creatinine
 CXR
 Tissue biopsy-
A micrograph of biopsy depicts collections of
epithelioid histiocytes (granulomas)
surrounding small vessels

62.  Coronal CT showing typical
appearances of Wegener’s in
the nose with septal
destruction hyperostosis and
opacification of the sinuses
together with orbital infiltration.

64. Treatment
 Steroids and cytotoxic drugs
 Prednisolone (60-80mg/day)and cyclophosphamide
(2mg/kg)/azathioprine (200mg/day)
 Plasma exchange immunoglobulin infusion, methotrexate,
cyclosporin, mycophenolate mofetil.
 Nasal symptoms- intranasal steroids, glucose and glycerine
drops and nasal douching and irrigation.

65.  Deoxyspergualin
an immunomodulatory agent
inhibits lymphocyte differentiation, appears to be effective in
maintaining remission and reducing steroid requirements
 Surgery is generally reserved for cases that are refractory to
medical treatment,or for complications.

66. Eosinophillic Granuloma/
Langerhans cell histiocytosis/
Langerhans granulomatosis.
 A clonal proliferation of Langerhans cells associated with a
heterogeneous inflammatory infiltrate of eosinophils,
histiocytes, lymphocytes, plasma cells and neutrophils.
 now regarded as a neoplastic condition.

67. Pathology
 Macroscopically, the lesions are soft and
yellow or red-brown in colour
 Microscopically, the Langerhans cells are
mixed with other inflammatory cells.
 The cytoplasm of the Langerhans cells may
be eosinophilic and Charcot–Leyden crystals

68. Clinical Features
 Predominantly occurs in bones.
 Skull is a common site of involvement, in particular the
temporal, frontal and parietal bones.
 Involvement of the temporal bone may simulate acute
mastoiditis.
 Mandibular lesions produce toothache,
gum ulceration and loose teeth.

69. Diagnosis
Radiological
 punched out bony lesion.
 Lesion in the skull show bevelled
margin
due to angulated destruction of
cortical bone.

70.  In the jaws radiolucent
areas around the teeth.

71. Treatment
Solitary, or ‘type II disease’
 combination of curettage/excision
and radiotherapy is usually
curative
Generalized or ‘type Idisease’
 additional chemotherapy
 At present, the most effective
chemotherapy regime appears to
be etoposide and steroids given
for periods of 12 months
Recently, alpha interferon and bone marrow
transplantation have also been used successfully.

72. COCAINE-INDUCED MIDLINE
DESTRUCTIVE LESION
 Intra-nasal inhalation is known to cause mucosal inflammation
 Patients predisposed to produce ANCA
 Pathology-
 Nearly 90% have a positive p-anca against human neutrophil elastase
(HNE), which may increase apoptosis and the local inflammatory
response to injury

73. Clinical features
 Rarely systemic symptoms
 chronic nasal obstruction and bleeding,
 change in shape of the nose and nasal regurgitation
 variable degree of destruction of the septum, turbinates,
lateral nasal wall and floor

74. Diagnosis
 positive human neutrophil elastase ANCA
 Urine, blood and hair can be tested for cocaine if necessary

75. Treatment
 No role for immunosuppression
 Must stop using cocaine to prevent further progression.
 Conservative treatment includes nasal douching, debridement
of necrotic areas and topical or systemic antibiotic therapy.
 Surgical correction of septal perforation or nasal deformity
should not be attempted until the patient has been clear of
cocaine for at least 6–12 months.

76. NON-HEALING MIDLINE GRANULOMA
Synonyms
 Stewart's granuloma
 Midline lethal granuloma
 Polymorphic reticulosis
 Sinonasal lymphoma
 T / NK cell lymphoma
Aetiology- caused by the Epstein-Barr virus.

77. Clinical Features
 Usually present with aggressive destruction of the middle of
the face.
 Usually arises in the nasal cavity and spreads to involve
adjacent structures including the orbits, oral cavity, skin and
paranasal sinuses.
 intra-nasal granulomatous mass initially causes symptoms of
obstruction,discharge and bleeding.

78. early ulceration and significant central destruction

79. Diagnosis
 Histology-
infiltrates are polymorphic and
atypical cells may to be
arranged in a necrotizing
angiocentric growth pattern.
 Immunohistochemistry is
usually positive for CD56, CD2
and cytoplasmic CD3

80. Treatment
 Initially low dose radiotherapy was preferred
 Now a days full course of radiotherapy is administered
covering the entire area
 Chemotherapy is indicated only for high grade lesions

81. CHURG-STRAUSS SYNDROME
 Eosinophil-rich and granulomatous inflammation involving the
respiratory tract and necrotizing vasculitis affecting small to
medium-sized vessels associated with asthma and
eosinophilia.
 Bronchial asthma, nasal polyps, eosinophilia, systemic
vasculitis.
 Treatment- oral steroids, treatment of nasal polyps.

82. Conclusion
 Any patient with blood-stained discharge and crusting in the
nose has a granulomatous condition until proven otherwise.
 No one test is completely reliable and a combination of
clinical findings combined with diagnostic investigations is
required.
 Patients on long-term systemic steroids should be monitored
for complications.
 Surgery is generally reserved for refractory cases or
complications of disease.

83. Thank You