Granulomatous Diseases of the nose

2. Granulomatous diseases of
the nose
Dr. KhalidMahmoud

3. An uncommon group of conditions causing
chronic inflammation in the nasal cavity &
paranasal sinuses characterized histologically by
granuloma formation.
Granulomatous diseases of the nose

4. organized collection of histiocytes (macrophages)
Granuloma

5. Organized : so tightly clustered that the
borders of the individual cells are difficult to
appreciated.
Often , but not invariably
fuse to form multinucleated giant
cells(langerhans giant cells) .

6. Organized : so tightly clustered that the
borders of the individual cells are difficult to
appreciated.
Ofetn , but not invariably
fuse to form multinucleated giant
cells(langerhans giant cells) .
On the other hand
loosely dispersed macrophages are not
considered to be granuloma

7. The macrophages in granulomas are often reffered to as “
epitheliod macrophages ”
This term refers to the vague resemblance of these
macrophages to epithelial cells.

8. These macrophages differ from the ordinary ones in that:
1. elongated larger nuclie that often resemble the sole of a
slipper or shoe .
2. The cytoplasm is typically more pink when stained with
eosin .

9. These changes are thought to be a consequence of
“ activation” of the macrophage by the offending
antigen

10. 1.infection
2.Inflammation
3.Neoplasm
4.Autoimmune
5.Trauma
6.Substance abuse
Causes:

11. All these conditions can result in
granulomatous inflammation in
paranasal sinus tissues that can
be quite destructive.

12. Although many of these conditions
are localized, some may also be
associated with systemic
and life-threatening disease.

13. Evaluation of these patients
requires a careful history and
physical examination to
look for evidence of disease not
localized to the nasal area
such as arthritis, central or
peripheral nervous system
lesions, ocular inflammation, or
lower respiratory tract or
renal involvement

14. Common examination features
refractory inflammation with
Nasal crusting
Bleeding
friable mucosa
Often with necrosis of the nasal septum
occasionally with extension into
adjacent structures

15. The clinician must ascertain the
cause of the granulomatous
process in order to mitigate
destructive and fatal outcomes

16. Operative intervention
to obtain specimens for microscopic
examination and culture and to
relieve obstruction should be planned
and performed in a thoughtful
manner

17. Although surgical
excision may suffice in some
situations, others will
require systemic treatments

18. Rhinoscleroma

19. Rhinoscleroma
It is chronic , progressive granulomatous
disease commencing in the nose & extending
into the oropharynx , larynx (subglottic area) ,
trachea and bronchi
The causative organism
Klebsiella rhinoscleromatis
(Gram – negative Frisch bacillus)

20. The disease runs through the following stages
Atrophic Granulomatou
s
Cicatrical
It resembles atrophic rhinitis and is characterized
by foul-smelling purulent nasal discharge and
crusting.

21. The disease runs through the following stages
Atrophic Granulomatou
s
Cicatrical
Proliferative stage
Painless , non-ulcerative Granulomatous nodules form
in nasal mucosa. There is also subdermal infiltration
of lower part of external nose and upper lip giving a
“woody” feel

24. The disease runs through the following stages
Atrophic Granulomatou
s
Cicatrical
This causes stenosis of nares, distortion
of upper lip, adhesions in the nose, nasopharynx
and oropharynx. There may be subglottic stenosis
with respiratory distress

25. diagnosis
Biospy shows subdermal infiltrates of plasma cells,
lymphocytes, esinophils , MICULICZ cells & RUSSELL
bodies

26. Mikulicz cells : are large foam cells with a central neucleus and
vacuolated cytoplasm containing the bacilli

27. Russell bodies: are homogenous esinophilic inclusion bodies
found in plasma cells

28. Both streptomycin (1 g/day) and tetracycline (2 g/day)
are given together for a minimum period of 4–6
weeks and repeated, if necessary, after 1 month.
Treatment is stopped only when two consecutive
cultures from the biopsy material are negative.
Steroids can be combined to reduce fibrosis.
Surgical treatment may be required to establish the
airway and correct nasal deformity.

29. Rhinosporidiosis

31. mostly affects nose and nasopharynx;
other sites such as :
lip, palate, conjunctiva, epiglottis,
larynx, trachea, bronchi, skin, vulva and
vagina.

32. The disease is acquired through contaminated
water of ponds also frequented by animals.

33. The disease is acquired through contaminated
water of ponds also frequented by animals.

34. The disease is acquired through contaminated
water of ponds also frequented by animals.

35. In the nose, the disease presents as a leafy, polypoidal
mass, pink to purple in colour and attached to nasal
septum or lateral wall.

38. Sometimes, it extends into the nasopharynx
and may hang behind the soft palate.

39. The mass is very vascular and bleeds easily on touch.
Its surface is studded with white dots representing the
sporangia.

40. In early stages, the patient may complain of nasal
discharge which is often blood tinged and nasal
stuffiness.
Sometimes, frank epistaxis is the only presenting
complaint.

41. DIAGNOSIS
This is made on biopsy. It shows several
sporangia, oval or round in shape and filled
with spores which may be seen bursting
through its chitinous wall.
It has not been possible to culture the
organism or transfer the disease to
experimental animals..

42. TREATMENT
Complete excision of the mass with diathermy
knife and cauterization of its base.
Recurrence may occur after surgical
excision.
Not many drugs are effective against the
disease. Dapsone has been tried with some
success.

43. Mucormycosis

44. Aggressive opportunistic fungal infection
It is seen in uncontrolled diabetics
or in those taking immunosuppressive drugs

45. Mucomycosis differs from other fungi as it has
A remarkable affinity for blood vessels & arteries
leading to extensive endothelial damage &
thrombosis

46. The disease begins in the nose and PNS &
spread to
Orbits , cribriform plates , meninges and the brain

47. Typical finding:
Black necrotic mass seen filling the entire nasal
cavity

48. Erosin of the nasal septum and the hard
palate may be seen

50. Treatment include systemic Amphotercin B
and surgical debridement of the affected tissue

51. Orbital exenteration is mandatory in case of orbital
involvement (ophthalmopelgia and loss of vision)

52. Syphilis

53. Nasal syphilis is of two types
acquired congenital
Primary
Secondary
Tertiary
It manifests as primary chancre of the
vestibule of the nose. It is rare.
It manifests as simple rhinitis with crusting & fissuring
in the nasal vestibule. Diagnosis is suggested by the
presence of mucous patches in the pharynx , skin rash
, fever and generalized fatiguability
(Rarely recognized)

54. Nasal syphilis is of two types
acquired congenital
Primary
Secondary
Tertiary
Syphilis
1. Typical manifestation is the formation of
a gumma on the nasal septum.
2. Later, the septum is destroyed both in its bony and
cartilaginous parts.
3. Perforation may also appear in the hard palate.
4. There is offensive nasal discharge with crusts.
5. Bony or cartilaginous sequestra may be seen.
6. Bridge of the nose collapses causing a saddle nose
deformity.
This is the stage in which nose is commonly involved.

62. Nasal syphilis is of two types
acquired congenital
Early form
Late form
• It is seen in the first 3 months of life and
manifests as “snuffles.”
• Soon the nasal discharge becomes purulent.
• This is associated with fissuring and excoriation
of the nasal vestibule and of the upper lip.
Clinical picture is similar to that seen in tertiary
stage of acquired syphilis.
Gummatous lesions destroy the nasal structures.
Other stigmata of syphilis such as corneal
opacities, Deafness and Hutchinson’s teeth are
also present
Usually manifests around puberty.

64. DIAGNOSIS
It is made on serological tests (VDRL) and biopsy of the
tissue with special stains to demonstrate Treponema
pallidum.

65. TREATMENT
(a) 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.
(b) Nasal crusts are removed by irrigation with alkaline
solution.
(c) Bony and cartilaginous sequestra should also be
removed.
(d) Cosmetic deformity is corrected after disease becomes
inactive.

66. Tuberculosis

67. Primary tuberculosis
of nose is rare.
More often it is
secondary to lung
tuberculosis
Anterior part of nasal
septum and
anterior end of inferior
turbinate are the sites
commonly
involved.

68. First, there is nodular infiltration followed later by
ulceration and perforation of nasal septum in its
cartilaginous part.

70. Diagnosis can be made on biopsy and
special staining of sections for acid fast bacilli
and culture of organisms.
Treatment is antitubercular drugs.

71. Lupus vulgaris

72. It is a low-grade
tuberculous infection
commonly affecting
nasal vestibule or the
skin of nose and face.

73. The skin lesions manifest characteristically as brown,
gelatinous nodules called “apple-jelly” nodules.

75. In the vestibule, it presents as chronic vestibulitis.
Perforation may occur in the cartilaginous part of nasal
septum.

77. Diagnosis can be made on biopsy and
special staining of sections for acid fast bacilli
and culture of organisms.
Treatment is antitubercular drugs.

78. Leprosy

79. It is caused by Mycobacterium leprae. The nose is involved
as a part of systemic disease.

80. Infection starts in the anterior part of nasal septum and
anterior end of inferior turbinate.
Initially, there is excessive nasal discharge with red and
swollen mucosa.
Later, crusting and bleeding supervene.
Nodular lesions on the septum may ulcerate and cause
perforation.
Late sequelae of disease are atrophic rhinitis, depression
of bridge of nose and destruction of anterior nasal spine
with retrusion of the columella

81. Diagnosis can be made from the scrapings of
nasal mucosa and biopsy. Acid-fast lepra
bacilli can be seen in the foamy appearing
histiocytes called lepra cells.
Treatment is with dapsone, rifampin and
isoniazid.
Reconstruction procedures are required when
disease is inactive

84. Aspergillosis

85. Aspergillosis
The usual causative organisms are Aspergillus niger,
A. fumigatus or A. flavus. They invade nasal tissues when
host’s defence mechanisms are compromised due to
immunosuppressive drugs.

86. Aspergillosis
Clinical features are those of acute or subacute rhinitis or
sinusitis.
A black or greyish membrane is seen in the nasal mucosa.
Exploration of maxillary sinus reveals a fungus ball
containing semisolid cheesy-white or blackish material.

97. TREATMENT
Surgical debridement of the involved tissues
and antifungal drugs, e.g. Amphotericin B.
Repeated irrigation of the involved area with
application of 1% solution of gentian violet is
also useful.

99. Wegner Granulomatosis

100. AETIOLOGY
It is a systemic disorder of unknown aetiology involving
mainly :
1. Upper air ways
2. Lungs
3. Kidneys
4. The skin

101. Early symptoms of
Wegener’s
granulomatosis
include clear
or blood-stained
nasal discharge
which later becomes
purulent.
The patient often
complains of
“persistent cold”.
Local Nasal manifestations Systemic manifestations

102. Nasal findings
include crusting,
granulations, septal
perforation and a
saddle nose.
Destruction may also
involve eyes, orbit,
palate, oral cavity or
oropharynx. Middle
ear can also be
involved.
Local Nasal manifestations Systemic manifestations

103. Lung – Haemoptysis
X-ray chest may show a single
or multiple cavity lesions
Kidneys – Haematuria
Urine examination
will show red cells, casts and
albumin. Serum creatinine
level is raised. Renal failure is
the usual cause of death in
these patients.
Anaemia
Local Nasal manifestations Systemic manifestations

105. DIAGNOSIS:
Biopsy from the nose is diagnostic. It shows
necrosis and ulceration of mucosa, epithelioid
granuloma and necrotizing vasculitis involving
small arteries or veins.
ESR is raised.
CANCA is positive

106. Treatment:
It consists of systemic steroids and cytotoxic
drugs.
Cyclophosphamide and azathioprine, both are
found effective.

107. T-Cell Lymphoma
Extra-nodal nasal natural Killer (NK) T- cell lymphoma is a
unique type of non-Hodgkin lymphoma (NHL) that is almost
always associated with
Epstein-Barr virus

108. It is locally angioinvasive and
destructive and was previously called
lethal midline granuloma

109. Angiocentric and angioinvasive
Ischaemic necrosis and ulceration of the
involved tissues
Invasion and destruction of the vessels walls

110. The tumor is composed of small , medium-size ,
or large cells which are admixed with apoptotic
bodies and inflammatory cells .

111. Unlike Wegener’s granulomatosis, it is rapidly destructive
and usually devoid of systemic involvement; there is
absence of involvement of lung and kidneys
The disease is predominantly
localized to the upper aerodigestive
tract , most commonly in the nose.

112. It is a destructive lesion usually starting on one
side of nose involving the upper lip, oral cavity,
maxilla and sometimes even extending to orbit.

115. Diagnosis: Immunohistochemical studies of
biopsy material are necessary to establish
diagnosis of T-cell lymphoma.

116. Treatment: Localized T-cell lymphoma is
treated by radiation while a disseminated
disease requires chemotherapy

117. Sarcoidosis

118. Sarcoidosis
• It is a granulomatous
disease of unknown
aetiology resembling
tuberculosis on histology
but with the absence of
caseation.
• It is a systemic disorder
and the symptoms may
refer to involvement of
lungs, lymph nodes, eyes
or skin

120. In the nose, it presents with submucosal nodules
involving
septum or the inferior turbinate
with nasal obstruction, nasal pain and sometimes
epistaxis.
Nodules may also form in the nasal vestibule or
skin of face.

121. X-ray chest shows diffuse pulmonary infiltrate with
hilar adenopathy
This is an X ray of 28 – year – old man shows extensive
bilateral hilar & mediastinal LN enlargement with no
associated pulmonary abnormality

122. • Serum and urinary calcium levels are raised.
• Biopsy of the lesions helps to establish the diagnosis

123. Treatment is with systemic steroids. For nasal
symptoms, steroids can be used locally as nasal
spray

124. Any Questions