Granulomatous lesions of nose

Dr T. Balasubramanian

Introduction

Presence of granuloma

Macrophages, granulocytes and multinucleated giant cells

Presence of vasculitis

Systemic involvement is common

Types of granulomas

Infective

Inflammatory

Neoplastix

Infective granulomas

Bacterial Tuberculosis, leprosy, Rhinoscleroma, syphilis and actinomycosis

Fungal Aspergillus, zygomycosis, Dermatocietes, Blastomycosis, Histoplasmosis, Sporotrichiasis and coccididomycosis

Protozoa Leishmaniais

Miscellaneous - Rhinosporidiosis

Neoplastic

Non healing granulomas

Midline granulomas

T cell lymphomas

Inflammatory granulomas

Wegner's granulomatosis

Sarcoidosis

Churg-Strauss syndrome

Cholesterol granuloma

Eosinophilic granuloma

Sarcoidosis

Etiology unkonwn

Multisystem disorder

Commonly involved organs include: Lymph nodes, skin, lungs, eye, nose, liver and spleen

Cutaneous lesions are known as lupus pernio

Nasal involvement of sarcoidosis almost always indicate multisystem involvement of the disease

Sarcoidosis (features)

Commonly affects adults

Affected age group 2nd - 5th decades

Females outnumber males 2:1

Sarcoidosis (Etiology)

Unknown

Infective agents

Chemicals (Beryllium, Zirconium etc)

Pine pollen

Peanut dust

Immunological

Sarcoidosis (Immunology)

Type IV hypersensitivity reaction reduced in these patients

No depression of CMI

Humoral immunity normal

Sarcoidosis (current hypothesis)

Causes for persistent granuloma (sarcoidosis)

Increased production of calcitriol by monocytes

Continued antigenic stimulation

Failure of supressor mechanism

Abormalities in the regulation of cytokine network

Sarcoidosis (Histology)

Fromation of epitheloid cell tubercles

There is no evidence of caseation

Fibrinoid necrosis may be seen sometimes

Fibrinoid necrosis may be converted to hyaline fibrosis

Tubercles are surrounded by lymphocytes and fibroblasts

These histological features are not diagnostic

Nasal sarcoidosis (clinical features)

Nasal stuffiness & obstruction

Crusting of nasal mucosa is commonly seen

Blood stained nasal discharge

Purulent nasal discharge

Facial pain

Sometimes mucoid discharge

Anosmia (rare) due to mechanical obstruction of the olfactory cleft by crusts

Nasal sarcoid clinical features (contd)

Perforation of anterior portion of nasal septum

Nasal bridge collapse

Nasal bones may be thickened due to osteitis

Secondary infections of sinuses common

Adenoid / tonsillar enlargement

Secretory otitis media

Diagnostic features of nasal sarcoidosis

Clinical acumen

Histology

Imaging

Hematology

Kveim test (cutaneous hypersensitivity test for sarcoidosis) withdrawn now

Angiotensin converting enzyme elevated during acute phase

ESR raised

Sarcoidosis (Radiology)

X-ray nasal bones show rarefaction & osteolytic reaction

Punctate osteolysis of nasal bones may be seen

CT scan of nose and PNS will also reveal soft tissue changes over the nasal bone area

MRI brain is a must to rule of CNS involvement

X-ray chest would show pulmonary infiltrates

CT nasal bone area

X-ray chest in sarcoidosis

Sarcoidosis (treatment)

Spontaneous remission (possible)

Oral steroids

Methotrexate

Hydroxychloroquine

Role of surgery in nasal sarcoidosis

Contraindicated

It could cause extensive nasal deformity

Contraindication is absolute in active nasal sarcoidosis

FESS can be resorted to in order to releive obstruction and to facilitate drainage of the involved paranasal sinuses

Wegner's granulomatosis

Granulomatous inflammation involving nasal mucosa and upper respiratory tract

Necrotizing vasculitis of of small & medium sized blood vessels

Classic triad airway, lung and renal disease

ANCA test diagnostic (Antineutrophil cytoplasmic antibody test)

Wegner's granuloma (age of presentation)

Seen in the age group 15 70)

Younger patients are more prone for multisystem damage

Renal damage is rather common in the young age group

Wegner's granuloma (etiology)

Unknown

Immune response to an unknown stimulus ??

Hypersensitivity reaction ??

Antigen from inhaled bacteria could be the stimulus ?? a reason why it commonly begins as an URI

Vasculitis is caused by deposition of immune complexes

ANCA

Two types of ANCA are present

PANCA (Perinuclear)

CANCA (Cytoplasmic)

Patients with Wegener's have elevated levels of cANCA

These tests are performed using immunoflorescence / radioimmunoassay

Clincial features of Wegners

Involvement of upper and lower respiratory tract

Glomerulonephritis

Nasal block / crusting of nasal mucosa / epistaxis

Sinus imaging in wegners

CT scan shows evidence of mucosal thickening

Bone destruction

New bone formation

Wegner granulomatosis diagnostic criteria

Nasal mucosa /oral mucosal inflammation

Abnormal x ray chest showing the presence of nodules. Cavities andd fixed infiltrates

Microhematuria and urinary sediments

Biopsy from the lesions showing granulomatous inflammation

Treatment

Prednisalone

Azathioprine

Cyclophosphamide (discontinued because of extensive side effects)

Patients on long term steroids should undergo bone densitometry in order to pick up early osteoporotic changes

Mycophenolate mofetil is currently being used instead of azathioprine

Nasal wegeners managment

Saline alkaline douching

Intranasal steroid drops

Glucose glycerine drops

Nasal surgeries like rhinoplasty / augmentation rhinoplasty should be avoided during active stages of the disease

Churg-Strauss syndrome

Characterised by systemic vasculitis and asthma

Granulomas of churg strauss are richly infiltrated by eosinophils

Vasculitis is histologically giant cell necrotizing type

Nasal manifestations include: Mucosal crusting, septal destruction, epistaxis etc

Eosinophilic granuloma

Characterized by clonal proliferation of Langerhan's giant cells associated with a heterogenous inflammatory infiltrates of eosinophils, lymphocytes, plasma cells and neutrophils

Currently this condition is regarded as a neoplastic disorder

It can also be considered as a variant of Histiocytosis X

Eosinophilic granuloma cllinical features

Bones are predominantly affected

Skull is the common site of involvement

Males are affected twice as often as females

Painful swelling involving facial bones associated with cervical adenopathy

Mandibular lesions could cause tooth ache, gum bleeding etc

Eosinophilic granuloma (Histology)

Langerhan's giant cells predominate

These cells are found to be mixed with infiltrates of histiocytes, neutrophils and plama cells

Cytoplasm of Langerhan's giant cells may be eosinophilic with the presence of charcot leyden crystals

Intense osteoclastic activity can be seen in the periphery due to the presence of cytokines and prostaglandins by Langerhan's cells

Eosinophilic granuloma (treatment)

Depends whether it is mono ostototic / polyostotic

Spontaneous regression have also been documented

For managing mono ostotic variety a combination of curettage / excision and radiotherapy have been attempted with varying degrees of success

Combination of etoposide and steroids administered for a period of 12 months is beneficial

Alpha interferon and bone marrow transplant have been found to be useful in polyostotic variety

Giant cell granuloma

Reparative granuloma

Aggregates of giant cells (uninucleate) in a fibrovascular stroma characterizes this condition

Commonly involves the jaw. Cranio facial bones are also commonly involved

Commonly seen in children

Bilateral involvement causes cherubism in children. This is caused by symmetrical involvement of jaws

Imaginng in giant cell granuloma

Expansile lytic lesion with a soap bubble appearance in the middle

Edges are well demarcated

Treatment of giant cell granuloma

Curettage may help

Total excision is advisable when possible

Recurrence rate is very high

Cholesterol granuloma

This is due to granulomatous reaction to the cholesterol crystals precipitated in the tissues

This precipitation is seen in hemorrhage / injuries

Commonly involves maxilla / frontal sinuses

Adjacent normal structures like the orbit may also be displaced

Can be treated by excision

Granulomatous neoplasia

Non healing granuloma

Midline destructive granuloma

T cell / NK cell lymphoma

Stewart granuloma

This lesion classically causes extensive destruction of the middle third of face

Granulomatous neoplasia (features)

Common in males

Common during 5th - 6th decades of life

Currently these granulomas are considered to be T cell lymphoma / NK cell lymphoma

Granulomatous neoplasia (stages)

Prodromal

Period of activity

Terminal stage

Prodromal stage

This stage could last for many years

These patients may c/o persistent nasal obstruction with rhinorrhoea

These patients would also be offered surgery for the same

Period of activity

Purulent nasal discharge

Crusting / necrosis / tissue loss

Progressive destruction of nasal framework

Destruction of palate and upper lip

Fever due to secondary infection

Terminal stage

Gross mutilation of face

Exhaution

Distant metastasis would have already occured

Eventual death

Diagnosis

Necrotic areas can be seen in the biopsy

Atypical cellular infiltrates can be seen in the midst of necrotic tissue

Good deep biopsy of the tissue is a must to obtain a representative specimen

Immunohistochemistry using monoclonal antibodies against T cell differentiation antigen can be used

Treatment

Low dose radiation was used previously to manage these cases

Full course of RT is advised these days in order to manage these cases adequately

Nasal tuberculosis

Nasal infections are caused by direct inoculation

Nose picking has been commonly attributed as a cause

Dissemination from cavity in the lung

Hematogenous spread to the nasal cavity mucosa

Nasal tuberculosis (types)

Nodular type

Ulcerative type

Granulomatous type involving the sinuses

Nodular form

Also known as Lupus vulgaris

Usually begins in the vestibule and extends to involve the nasal mucosa

Commonly caused due to direct inoculation

These lesions appear are glistening reddish brown papules / nodules (apple jelly nodules)

These nodules are usually painless

These papules may coalese to form ulcers with pale granular base and undermined edges

Blanching tests

Pressure by using glass slide will cause blanching of surrounding area making these papules more prominent. For mucosal lesions adrenaline / cocaine can be used to cause blanching

Ulcerative type

This type usually involves the cartilagenous portion of nasal septum and inferior turbinate

Mucosal crusting, epistaxis

This lesion can lead to septal perforation and saddle nose deformity

The bony portion of the nasal septum is not involved this differentiates it from syphilis

Sinus granuloma

Isolated involvement of sinus without involvement of nasal cavity

Soft tissue swelling over the involved sinus.

Multiple discharging sinuses from the swelling

Bony destruction of the sinus wall could also be evident in these patients

Diagnosis

Tissue biopsy would show epitheloid cell granulomas

Evidence of caseation +

Presence of AFB is virtually diagnostic if present

PCR has increased the sensitivity

Treatment

Since it is extrapulmonary initially the treatment is started with 4 drug regimen (INH, Rifampicin, ethambutol and pyrazinamide) for 2 months

Two drugs INH and Rifampicin 4 months

Syphilis

Primay, secondary and tertiary syphilis

Can involve patients of any age group

The organims usually reside and multiply in the perivascular lymphatics of the blood vessel

Primary syphilis

Sore or chancre develops at the site of inoculation

Begins during the third week after inoculation

Regional lymphadenitis is common

These sores are painless nodules

These lesions usually disappears within 3 months

Smears from the lesion usually demonstrate T pallidum

Secondary syphilis

This is the most infectious stage

Simple catarrhal rhinitis is seen

Crusting / fissuring of nasal vestibular skin is seen

Patches could be seen over the nasal mucosa

Best confirmed by serological tests

Tertiary syphilis

This is the commonest lesion involving the nose

Bony portion of the nasal septum is commonly destroyed causing septal perforation

Nocturnal pain is one of the features

Secondary atrophic rhinitis can also be caused

Collapse of nasal bridge

Congenital syphilis

Also known as snuffles

Occurs during the 3rd week - 3rd month after birth

Begins as simple catharral rhinitis which later turns purulent

Child has difficulty sucking milk due to excoriation involving the lips

Rhinoscleroma

Caused by Klebsiella rhinoscleromatis

Progressive granulomatous disease involving the nose and later extending to oro and nasopharynx

The causative organisms are usually intracellular and hence are difficult to isolate

Rhinoscleroma (Histology)

Granulomatous tissue infiltrates in the submucosa

Plama cells, lymphocytes and eosinophils are seen in these infiltrates

There are scattered large foam cells known as Miculicz cells. These cells have a central nucleus with vacuolated cytoplasm which contain the bacilli.

Rhinoscleroma (stages)

Atrophic stage

Granulomatous / proliferative stage

Cicatrizing stage

Atrophic stage

This stage resembles atrophic rhinitis

Crusting of nasal mucosa

Epistaxis

Foul smelling discharge

Granulomatous stage

Non ulcerative nodules develop

They are bluish red and rubbery later becomes paler and harder

These nodules dont break down but fibrose

Regional nodal involvment is rare

Cicatrizing stage

Adhesions and stenosis are seen

Distortion of normal nasal anatomy

Spread to nasopharynx

Bone involement could be seen

Leprosy

Chronic granulomatous lesion caused by M leprae

Two types are seen Tuberculoid and lepromatous

Tuberculoid leprosy

Anesthetic cutaneous patches

Nasal mucosa is uninvolved

Vestibular skin is involved

Isolated cranial nerve palsies 5th and 7th cranial nerves are commonly involved

Lepromatous leprosy

Nasal mucosal involvement is seen

Nasal mucosal crust formation / epistaxis

Secondary atrophic rhinitis

Destruction of both bony and cartilagenous portions of nasal septum

Collapse of nasal bridge

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