Granulomatous lesions of nose

Granulomatous lesions
of nose
BALASUBRAMANIAN THIAGARAJAN
OTOLARYNGOLOGY ONLINE

Introduction
Features of granula of nose:
1. Presence of granuloma which contains – macrophages, epithelioid cells, and multinucleated
giant cells
2. Presence of vasculitis which may be primary or secondary
3. Majority of these conditions have systemic manifestations

Classification of granulomatous lesions of
nose
1. Infective
2. Inflammatory
3. Neoplastic

Infective causes Bacterial
Tuberculosis – Mycobacterium tuberculosis
Leprosy – Mycobacterium Leprae
Rhinoscleorma – Klebsiella Rhinoscleromatis
Syphilis – Treponema Pallidum
Actinomycosis – Actinomyces israeli

Infective causes Fungal & protozoal
Aspergillus – Fumigatous, Flavus and Niger
Zygomycosis – Conidiobolus coronatus, Rhizopus oryae
Dermatacietes – Curvularia, Alternaria and Bipolaris
Rhinosporidiosis – Rhinosporidium seeberi
Blastomycosis – Blastomyces dermatitidis and Cryptococcus neoformans
Histoplasmosis – Histoplasma Capsulatum
Sporotrichosis – Sporotrichum schenkii
Coccidioidimycosis – Coccidioidi immitis
Leishmaniasis (protozoal) – Leishmania donovani

Inflammatory
Wegner’s granulomatosis
Sarcoidosis
Churg-strauss syndrome
Cholestrol granuloma
Eosinophilic granuloma

Neoplastic
T-cell lymphoma (Lethal midline granuloma)

Sarcoidosis
Systemic condition of unknown etiology
Can involve any part of the human body. Commonly involves nodes, lungs, upper airway, eyes,
liver and small bones of hand and feet
Commonly affects young adults between third and fifth decades
Nasal manifestations are part of multisystem involvement
Female preponderance 2:1

Etiology
Unknown
Infective agents suspected
Exposure to Beryllium, zirconium, pine pollen and peanut dust have been implicated
Type 4 delayed hypersensitivity reactions depressed in these patients
Cell mediated immunity is normal
Gamma globulin levels are increased
The entire process seems to be initiated by antigen presenting cells of alveoli
Failure of suppressor regulatory mechanism is responsible for persistence of granuloma

Clinical Features - Sarcoidosis
Nasal stuffiness / obstruction
Crusting
Blood stained nasal discharge
Purulent discharge
Facial pain
Mucoid discharge
Anosmia (rare)

Nasal findings - sarcoidosis
Granular in appearance – “Strawberry skin”
Erythematous mucosa with tiny pale granulomas
Nasal mucosa is friable causing nasal congestion, bleeding
Mucosal crusting are also evident
Perforation of nasal septum (anterior portion). If septoplasty is ventured in these patients there
could be associated collapse of the bridge of nose
Nasal bones may be involved causing expansion of the dorsum of the nose and thickening of skin
in that area. (Responds well to medical management).

Histology - Sarcoidosis
Non caseating granuloma
Presence of epithelioid cell tubercles
Fibrinoid necrosis could be seen at the center which gets converted into hyaline fibrous tissue
on healing
Schaumann bodies (crystalline/calcified inclusion bodies) are seen

Persistent granuloma - causes
Increased production of calcitriol by monocytes
Continued antigenic stimulation
Failure of suppressor mechanism
Abnormalities in the regulation of cytokine network

Other systems - sarcoidosis
Lung infiltrates
Peripheral node involvement
Skin involvement
Liver
Eye
Spleen
Heart
Kidneys
Larynx
Joints
Uterus
Lacrimal glands

Sarcoidosis – Diagnostic tests
Kveim test – Skin test Risk of virus / Prion transfer
Elevated angiotensin converting enzyme
X-ray chest – pulmonary infiltrates
Biopsy of nasal mucosa will help only if found abnormal
X-ray nasal bones – rarefaction / osteolysis if they are involved

Sarcoidosis - Management
Spontaneous remission – probable
Oral steroids / methotrexate / hydroxycholoquine
Nasal steroid spray
Nasal saline douching to remove crusts
Surgery is contraindicated

Wegner’s granuloma
Granulomatous inflammation of respiratory tract and kidneys
Necrotizing vasculitis involving small – medium sized vessels
Triad of airway, lung and renal disease common
Can occur in any age

Wegner’s granuloma - Etiology
Unknown
Inflammatory – hypersensitivity reaction with immune response to unknown stimuli (bacteria)
Deposition of immune complexes could cause vasculitis
cANCA elevated in Wagner's

Wegner’s granuloma-clinical features
Symptoms disproportionate to clinical findings
Malaise, pyrexia and sense of ill well
This disease is lethal. Pts usually die within 6 months due to renal failure
Nasal block
Epistaxis
Nasal septal destruction, collapse of bridge of nose
Facial pain
Nasal surgeries cause destruction of nasal septum and nasal bridge collapse
Cough, hemoptysis and pleural pain
Glomerulonephritis – renal casts and RBC in urine (mid urine sampling)

Wegner’s granuloma – Ocular symptoms
Conjunctivitis
Dacryocystitis
Episcleritis
Corneal ulceration
Proptosis due to orbital granuloma
Blindness

Wegner’s granuloma – oral symptoms
Hyperplastic granular lesion involving gingiva of interdental area
Loosening of tooth
Cavities fail to heal
Extensive ulcerative stomatitis

Wegner’s granuloma – otologial
symptoms
AOM
Otitis media with effusion
Facial nerve paralysis
Middle ear filled with necrotizing granulation tissue
Conductive / sensorineural hearing loss

Wegner’s granuloma-Miscellaneous
symptoms
Skin ulceration over distal arms and legs
Polymyalgia / polyarthritis
Nervous system involvement

Wegner’s granuloma - Diagnosis
cANCA test positive
ESR elevated
Elevated CRP level
Serum urea, creatinine and serum angiotensin converting enzyme levels to be assessed
Biopsy
CT imaging – nasal mucosal thickening. Pt with h/o previous nasal surgery showing bone
destruction and new bone formation is virtually diagnostic

Wegner’s granuloma - histology
Vasculitis is mandatory
Fibrinoid vascular necrosis is common
Presence of giant cell granuloma

Wegner’s granuloma - Treatment
Steroids
Cytotoxic drugs – cyclophosphamide, azathioprime, mycophenolate mofetil
Treatment to be initiated before renal damage sets in

Churg Strauss syndrome
Systemic vasculitis, bronchial asthma
Eosinophilic rich granuloma
Small and medium sized vasculitis
Nasal polyposis, nasal crusting, septal perforation
Can be managed with oral steroids

Eosinophilic granuloma
Involves clonal proliferation of Langerhans cells associated with heterogenous inflammatory
infiltrate of eosinophils, histiocytes, lymphocytes, plasma cells and neutrophils. This condition
may also be considered as a manifestation of histiocytosis x.

Eosionophilic granuloma - features
All organs may be affected
Predominantly involves bones (temporal, frontal and parietal bones)
Males are commonly affected, twice as common as females
Commonly affects individuals in the first three decades of life
Painful swelling over involved bone followed by cervical adenopathy
Mandibular lesions tooth ache, gum ulceration and loosening of teeth
Involvement of temporal bone will simulate acute mastoiditis

Eosinophilic granuloma - imaging
Punched out bony lesions in the jaw with
radiolucent areas around teeth
Skull lesions show beveled margins due to
angular destruction of bone

Eosinophilic granuloma - Management
Localized disease can be managed by curettage and irradiation if need be.
Solitary disease is more common
Regimen of etoposide & steroids for 12 months has proved promising
Recent regimen also include alpha interferon and bone marrow transplantation

Cholesterol granuloma
This condition results from hemorrhage / trauma
Granulomatous reaction is directed against cholesterol crystals
Shows male preponderance, without any age preponderance
This lesion is known to affect frontal / maxillary sinus causing swelling and cosmetic deformity
Proptosis could also be evident
Lesion produces a cyst like expansion of the affected bone. The sinus cavity appears opaque and
does not show contrast enhancement
Histology shows giant cell granuloma
Surgery and curettage of the lesion helps

Lethal midline granuloma
Also known as “T cell lymphoma” stewart
granuloma
Extensive destruction of mid face
Can involve patients of any age group
Male preponderance has been observed
EB virus infections have been implicated

Midline granuloma – clinical features
Prodromal stage – May last for many years. Nasal obstruction and rhinorrhea are the classic
features
Period of activity – Necrosis develops around nasal cavity. Purulent discharge + crusting+ tissue
loss+ There is progressive destruction of nasal framework, palate, upper lip, orbit and skull
base. Pyrexia may be present due to super added infection
Terminal stage – Bleeding, gross disfigurement and ultimately death

Midline granuloma - Diagnosis
It is a challenge because atypical cells are dispersed in necrotic areas
Biopsy material should be taken from under the slough / crust
Immunohistochemistry is a clincher
Absence of granulomas / giant cells is a feature
There is always associated thrombosis and necrosis

Midline granuloma - Management
Initially low dose radiotherapy was preferred
Now a days full course of radiotherapy is administered covering the entire area
Development of disseminated lymphoma is a problem
Chemotherapy is indicated only for high grade lesions

Nasal tuberculosis
Rare
Always associated with primary PT
Features include – ulcers, polypoidal lesions and nodules
Ulcers if present are typically seen in the anterior portion of nasal septum and inferior turbinate
Lupus vulgaris – is an indolent infection involving the skin and mucosal lining of nasal cavity
Nose picking commonly causes this condition

TB Nose – Clinical features
Nasal discharge / obstruction
Presence of non foul smelling crusts
Epistaxis
Presence of ulceration can cause mild fetor
Ulceration of nasal mucosa is followed by fibrosis, and contraction of nares
Extensive involvement of turbinates can lead to secondary atrophic rhinitis
Apple jelly nodules – Early lesion. Reddish brown nodule at the mucocutaneous junction
Cartilagenous portion of nasal septum undergoes destruction

Lupus - Features
Can cause extensive scarring of vestibule and may extend up to the skin of face. These lesions
are nodular in nature.
Features of lupus nodules:
Blanching on pressure
Bacterial examination
Biopsy
When pressure is applied to adjoining nasal mucosa using glass slide, pinkish nodules will stand
out because the adjacent areas undergo blanching
Biopsy of the lesion is always diagnostic

Nasal tuberculosis - Histopathology
Presence of tuberculous granuloma
In the center of the lesion collection of RE cells +
Giant cells are seen throughout the tubercle
Coagulation necrosis is a feature

Nasal tuberculosis - complications
Dacryocystitis
Lupus of the face
Atrophic rhinitis
Development of epithelioma - very rare

Nasal leprosy
Tuberculoid leprosy – skin lesions may extend up to nasal vestibule. Nasal mucosa is not
involved. Cutaneous anesthesia is a feature.
Lepromatous leprosy – Highly infectious discharge from nasal cavity. Nasal crust formation,
serosanguinous nasal discharge. Presence of nodular thickening of nasal mucosa is the earliest
feature. These nodules are commonly seen in the anterior end of inferior turbinate. Nasal
obstruction is classically out of proportion to mucosal oedema visualized. 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. Radiological evidence of destruction of
anterior nasal spine is virtually diagnostic of lepromatous leprosy.
Borderline leprosy – poor immunological tolerance. Skin around nose and eyes are involved.
Nasal mucosa is free of disease

Nasal syphilis
Primary syphilis - commonly seen in the vestibule of the nose. Usually appears as hard, non
painful ulcerated papule. Always associated with enlarged rubbery non tender
lymphadenopathy. Spontaneous resolution is seen in 6 weeks. Smears from the lesion are
positive. VDRL +
Secondary syphilis – infective stage. Catarrhal rhinitis, crusting, fissuring of nasal vestibule, and
enlarged non tender lymph nodes.
Tertiary syphilis – Bony portion of nasal septum involved. Septal perforation involving bony
portion of nasal septum is seen

Nasal syphilis - symptoms
Pain & headache – worse during night
Swelling / obstruction of nose
Diminished olfaction
Perforation of bony nasal septum with collapse of bridge of nose
Secondary atrophic rhinitis
Lesion is usually unilateral
Tenderness over the bridge of the nose is a characteristic sign
Swollen nasal mucosa that does not shrink with decongestants

Congenital syphilis
Also known as snuffles
Usually begins classically during 3rd week of life
Begins as serous rhinitis, which later becomes purulent
Excoriation of upper lip and vestibule of nose

Rhinoscleroma
Progressive granulomatous lesion begins at the nose and extends up to nasopharynx
Larynx, trachea and lower airway can rarely be involved
Can occur at any age, and can affect all age groups
Causative organism – Klebsiella rhinoscleromatis
Granulomatous infiltrates in the submucosa is a feature. Accumulated inflammatory cells
include: Plasma cells, lymphocytes, eosinophils and scattered large foam cells (Mikulicz cells).
These foam cells have a central nucleus and a vacuolated cytoplasm containing bacillli

Rhinoscleroma – Clinical features
Atrophic stage – Changes appear in the nasal mucous membrane. This stage clinically resembles
atrophic rhinitis
Granulation / nodular stage – Nodules are non ulcerative in nature. These nodes are initially
bluish red and rubbery. Later these nodes become a little paler and harder
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. Lymphatic
spread is uncommon because of extensive scarring. This disease may involve nasopharynx,
maxillary sinus and lower airways too.

Rhinoscleroma - 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

Rhinosporidiosis
It is a chronic granulomatous disease
characterised by production of nasal polypi
and other manifestations of hyperplastic nasal
mucosa. Etiologic agent has been
hypothesized to be Rhinosporidium seeberi. It
has also been described as a strawberry like
mulberry mass.

Rhinosporidiosis - History
1892 – Malbran observed the organism in nasal polyp
1900 – Seeber described the organism
1903 – O’kineley described the histology of the lesion
1905 – Minchin & Fantham restudied O’Kineley’s slide and named the organism as
Rhinosporidium Kineley
1913 – Zschokke reported a similar lesion in horses and named the organism Rhinosporidium
equi
1923 – Ashworth described for the first time life cycle of rhinosporidium seeberi
1924 – Forsyth described a skin lesion for the first time
1953 – Demellow described mode of transmission of the disease

Rhinosporidiosis – Special characteristics
More than 90% of these patients have been reported from India and Srilanka
Madurai, Thirunelveli, Ramnad and Kanyakumari districts of Tamilnadu are considered to be
endemic zones
Disease transmission is probably due to taking bath in common ponds where cattle also bathe

Rhinosporidiosis – Theories of spread
Demellow’s theory of direct transmission
Autoinoculation theory of Karunarathne (responsible for satellite lesions)
Hematogenous spread (spread to distant sites)
Lymphatic spread (rare)

Karunarathne’s theory
Rhinosporidium seeberi existed in dimorphic state.
It existed in saprophyte form in soil and water.
Inside tissues it took yeast form
According to karunarathne, this dimorphic existance helped organism to survive hostile
environmental conditions

Rhinosporidiosis – Reasons for
endemicity
Physical characteristics of water in the ponds
Presence of synergistic aquatic organism
Genetic predisposition of affected patients
Host immunity status

Life cycle (Ashworth)
Spore is the basic infecting unit
It is about 7 microns in size
It is also known as spherule
It has clear cytoplasm filled with 15-20 vacuoles containing food matter
It is enclosed in a chitinous membrane
These spores are commonly found in connective tissue spaces and is very rarely intracellular

Life cycle Ashworth (contd)
Spores begin to increase in size
At 50-60 microns size granules begin to appear
Nucleus prepare for cell division
Mitosis begins – 4,8,16, 32 and 64 nuclei are
formed
At 7th division size becomes 100 microns
Fully mature sporangium – 150 microns
Mature spores are found in the centre and
immature ones at the periphery

Life cycle - Recent
Juvenile sporangium also known as trophozoite – size ranging from 6-100 microns
It has a single nucleus at 7 micron stage and multiple nuclei at 100 microns stage
Lipid granules begin to appear within the cytoplasm
Intermediate sporangium – 100-150 microns. Has bilamellar cell wall, outer chitinous and inner
cellulose. Only immature spores are seen within the cytoplasm. No mature spores are seen

Mature sporangium
Size – 100 – 400 microns
Cell wall is thin and bilamellar with one weak spot known as operculum
Inside cytoplasm mature and immature spores are seen
Spores are embedded in mucoid matrix
Mature spores are covered with mucoid material resembling a comet (comet of Beattee)
Mature spores give rise to electron dense granules which are the ultimate infecting unit

Clinical classification
Nasal
Nasopharyngeal
Mixed
Bizarre (ocular / genital)
Malignant rhinosporidiosis (cutaneous)

Common sites affected
Nose – 78%
Nasopharynx – 68%
Tonsil – 3%
Eye – 1%
Skin – very rare

Nasal rhinosporidiosis - Features
Lesions are polypoidal reddish and granular
Lesions may be multiple, pedunculated and friable
Surface studded with whitish dots (sporangia)
Nasal lesions highly vascular and bleeds on touch
Entire mass could be clothed with mucous secretion
Lesion is restricted to nasal mucous membrane and does not cross the mucocutaneous junction

Rhinosporidiosis - Histology
Papillomatous hyperplasia of mucous
membrane with rugae formation
Epithelium over sporangia thinned out and
giant cells could be seen in this area
Accumulation of mucous + in crypts
Increased vascularity due to angiogenesis
factor
Spores stain with sudan black and Bromphenol
blue

Nasal rhinosporidiosis - Features
Chronicity
Recurrence
Dissemination

Chronicity - Reasons
Antigen sequestration
Antigenic variation
Immune suppression
Immune distraction
Immune deviation
Binding of host immunoglobulins

Treatment
Surgery
Dapsone 100 mg/day – 6 months

Granulomatous lesions of nose

Recomendados

Recomendados

Mais conteúdo relacionado

Mais procurados

Mais procurados (20)

Destaque

Destaque (20)

Semelhante a Granulomatous lesions of nose

Semelhante a Granulomatous lesions of nose (20)

Mais de Balasubramanian Thiagarajan

Mais de Balasubramanian Thiagarajan (20)

Último

Último (20)

Granulomatous lesions of nose

Notas do Editor