Granulomatous Diseases

of the Nose

Moderator : Dr Shraddha Pai/Dr Santosh G

Presenter: Dr. Sathvika B.
 Granulomatous Diseases
• A granuloma is an organized collection of macrophages, often
referred to as epithelioid cells, which tend to fuse to form
multinucleated giant cells.
• Granulomatous conditions of the nose and paranasal sinuses
may be secondary to infection, inflammation, trauma or
substance abuse, or may be due to an autoimmune or
neoplastic condition.
• The disease process may be confined to the nose or be part of
a systemic condition involving multiple organ systems.
Granulomatous Diseases of the Nose
 Infective
Granulomatous Diseases
- Fungal
 Rhinosporidiosis
• Tropical fungal disease caused by Rhinosporidium seeberi
• Characterized by the occurrence of granulomata in the upper
airway
• First described by Seeber (1900) - Buenos Aires
• Ashworth adopted the name R. seeberi
• More than 95% of cases reported from India & Srilanka
• Also from South America, Europe & N. America
 Rhinosporidiosis
• Rhinosporidium seeberi - previously considered to be a fungus
• Now considered to be a protist classified under Mesomycetozoea
• Strawberry like appearance - multiple yellowish pin head-sized
spots representing underlying mature sporangia
• Bleeds easily upon manipulation
• In India, the disease is endemic in the states of Tamil Nadu,
Chhattisgarh, Kerala, Orissa and West Bengal
 Rhinosporidiosis
• Papular or nodular, pink, red or purple smooth-surfaced lesions
that become pedunculated and acquire a papillomatous or
proliferative appearance
 Life Cycle of Rhinosporidum
seeberi

• Trophic Stage – Development of trophocyte with single nucleus.

• Development of sporangium- Nuclear + cytoplasmic division of trophocyte results in intermediate
sporangium. Enlarges to form mature sporangium which contains 16000 endospores
• Production of endospores- Rupture of mature sporangium releases infective endospores.
 Modes of Transmission
• Stagnant pools of water - source of human infection
• DNA sample from infected tissues & from free living
spores in infected ponds match (show similar pattern in
Random Primer Analysis of molecular DNA)

• Bathing in ponds
• Traumatic inoculation from one site to others
• Laryngeal rhinosporidiosis - may be due to inoculation from the
nose during endotracheal intubation
 Rhinosporidiosis - Sites
• Nasal • Maxillary antrum
• Nasopharyngeal • Nasolacrimal
• Oral, Oropharyngeal • Conjunctival
• Laryngeal • EAC
• Nasal-nasopharyngeal • Scalp
• Trachea, Bronchi, • Tarsal
Lung • Parotid duct
• Cutaneous • Bone
• Disseminated
 Rhinosporidiosis
• Nose - 70%
• Palpebral conjunctiva - 15%
• The disease affects mostly males (70–90%)
• Incidence – greater in patients aged 20 - 40 years
 Clinical Features
• Nasal obstruction • Oral mass
• Rhinorrhea • Airway obstruction
• Sneezing • Ocular mass
• Postnasal discharge • Photophobia
• Pruritus • Epiphora
• Epistaxis • Cutaneous, bony
• Anosmia lesions
• Cough
 Rhinosporidiosis - Manifestations
• Painless, friable, pink or purple-red polypoidal growth studded
with minute white dots, which are sporangia containing the
spores

Nasal (> 70%) Oropharyngeal

 Rhinosporidiosis - Manifestations
Nasopharyngeal Rhinosporidiosis Supraglottic

Lacrimal
Combined
Rhinosporidiosis - carina
 Rhinosporidiosis
Nasolacrimal Cutaneous

Conjunctival
 Disseminated Infections
• Involving limbs, trunks and viscera
• Brain involvement may lead to fatality
• Disseminated infection of limbs - often associated with
destruction of underlying bones
 Histopathology - Diagnostics
• Several round or oval sporangia and spores which may be seen
bursting through its chitinous wall
• The sporangia are 50-1000 μm in diameter, containing
numerous endospores of diameter 5-10 μm
• Overlying epithelium is usually hyperplastic and loose
fibrovascular stroma infiltrated with lymphocytes,
macrophages, plasma cells and even polymorphonuclear
leucocytes
• Rupture of sporangia can cause giant cell reaction

Hematoxylin & Eosin stain

 Rhinosporidiosis
COMPLICATIONS
• Life-threatening dissemination
• Local secondary bacterial infection
• Hemorrhage
• Recurrence

MANAGEMENT
• Surgical excision with cauterization of the base
• Laser excision
• Medical treatment - not been proven effective
• Dapsone, Itraconazole, Amphotericin etc
 Rhinosporidiosis

Epiglottic Rhinosporidiosis Subglottic Rhinosporidiosis

Post-op Post-op
 Rhinoentomophthoramycosis
• A rare tropical mycotic infection affecting the nasal cavities and
the subcutaneous tissues
• Most common causative organism – Conidiobolus coronatus
followed by basidiobolus
• Presentation - nasal discharge, nasal obstruction, pain, nasal
polyps or subcutaneous swellings of the lip or face.
• Common in diabetes & immuno-compromised
• Acquired via inhalation of spores or a minor trauma such as an
insect bite.
 Rhinoentomophthoramycosis
Right rhinofacial entomophthoramycosis

Subcutaneous swellings

Granulomatous inflammation with

multinucleated giant cells with well
defined tubular structures inside the
giant cells
 Rhinoentomophthoramycosis
DIAGNOSIS
• Clinical, histopathological and mycological examination
• Direct microscopy of tissue in KOH – broad nonseptate or
irregularly branching, infrequently septate filamentous hyphae

MYCOLOGICAL DIAGNOSIS
• Grocott's methenamine silver (GMS) and Periodic acid–Schiff
(PAS) stains - help identify the causative organism
• Culture from biopsy tissue on Sabouraud glucose agar to
identify the fungus - large fluffy white, gray, or brownish
colonies
 Rhinoentomophthoramycosis
BIOPSY
• Granulomatous infiltrate of histiocytes, eosinophils and multi-
nucleated giant cells and polymorphonuclear cells
• Broad thin walled infrequently septate hyphae surrounded by
Splendore-Hoeppli phenomenon (periodic acid–Schiff stain–
positive eosinophilic material surrounding fungal hyphae) and
absence of vascular invasion and necrosis is pathognomonic of
entomophthoramycosis
 Rhinoentomophthoramycosis
MANAGEMENT
Antifungal drugs
• Potassium iodide (40 mg/kg/day)- till lesions subside
• Amphotericin b (1 to 1.5 mg/kg/day) max of 2.5 gms
• Imidazole derivatives (fluconazole 300 mg/day), ketoconazole
• (400 mg/day)
• Triazoles (itraconazole 300 mg/day)
• Or a combination of two of these drugs till lesions abate
 Rhinoentomophthoramycosis
MANAGEMENT
• Posaconazole - salvage therapy in some patients who are
refractory to, or intolerant to amphotericin B and its lipid
derivatives (oral suspension, in a dosage of 800 mg divided in
two to four doses daily)

• Hyperbaric oxygen and itraconazole or miconazole – may be

used after failure to produce clinical cure by amphotericin
 Histoplasmosis – H. capsulatum
• Dimorphic fungus lives in soil with bird droppings generally
commensal in humans
• Spores inhaled especially after tilling the soil
• Initial infection – silent or self limiting flu like
• Reactivation later even after years.
• Mainly occurs in parts of the US, Central & S.America, Africa &
S.E. Asia & Australia
 Histoplasmosis – H. capsulatum
CLINICAL PRESENTATION
• Main target organ – Lung
• ENT manifestation : solitary, ulcerated & indurated mimicking
cancer.
• Occurs in Tonsil, buccal mucosa, tongue, pharynx, Larynx &
rarely Nasal mucosa
• Treatment : Itraconazole, IV Amphotericin
 Histoplasmosis – H. capsulatum
• Histoplasmosis of the Tonsil & Ary-epiglottic fold
 Paracoccidioidomycosis
• SOUTH AMERICAN BLASTOMYCOSIS
• Fungal infn caused by Paracoccidioides brasiliensis & P.lutzii
• Endemic in Central & S. America
• Acute & sub acute form in children mainly affects lymph nodes,
hepato-splenomegaly & bones – occasionally URT mucosa
• Chronic form affects adult men affects lungs & mucosa of Nose,
mouth, oropharynx, larynx & palate
 Paracoccidioidomycosis
• Infection through inhalation of conidia from soil
• Animal carrier – Armadillo
• Human to human transmission not known
• Diagnosis: demonstration of fungus in sputum – GMS stain
• Antibody detection by gel diffusion – 95% specificity
• Treatment: antifungals – itraconazole, Amphotericin
 Paracoccidioidomycosis

Culture in the yeast phase Histopathology

Acute phase
 Infective
Granulomatous Diseases
- Bacteria
 Nasal Tuberculosis
• Bacterial Infective granulomatous disease. Anterior part of
nasal septum and anterior end of inferior turbinate are
commonest sites of involvement
• Primary, or secondary to pulmonary tuberculosis or facial
lupus
• Although rare, it is one of the differential diagnosis of chronic
nasal granulomas
• Etiology:
• Consequent to direct
inoculation by nose picking, finger nail trauma,
open pulmonary TB or hematogenous
dissemination

Diagnosis: 1) DNE- nodular infiltration followed by

ulceration and perforation of cartilaginous part of
nasal septum
 Nasal Tuberculosis
• CT – Paranasal sinuses
• Soft tissue mass seen attached to the inferior turbinate on the
left side
 Nasal Tuberculosis
• Diagnosis – smear, histopathology, culture & polymerase chain
reaction
• Treatment: ATT is curative
 Hansen’s Disease (Leprosy)
• Chronic granulomatous infection caused by Mycobacterium
leprae (Bacteria)
 Hansen’s Disease (Leprosy)
• Portals of entry – skin & upper respiratory tract
• Predilection for skin, peripheral nerves and upper aerodigestive
tract
• Affects mucous membrane of the nose, nasopharynx,
oropharynx, oral cavity, larynx, pinna, facial & cervical
skin, facial nerve, cervical nerves e.g. greater auricular
nerve
 Hansen’s Disease (Leprosy)
• 2.2 million cases worldwide
• 1.66 million in India - WHO estimate (1994)
• Increased incidence in Africa & South Asia
• Anterior end of inferior turbinate - most likely site for a positive
biopsy
 Hansen’s Disease (Leprosy)
• Nose is the earliest involved site in lepromatous leprosy (95%)
• Pinna, palate, larynx & epiglottis may be involved
STAGES
• Catarrhal stage
• Nodular stage - nodules, ulcers, cartilagenous septal
destruction, saddle nose & alar deformity, atrophic rhinitis
• Spread of lesions to skin, pinna, other parts of the body,
invasion of nerves causing anesthesia
 Hansen’s Disease (Leprosy)
• M.Lepra in the nasal mucosa
 Lepromatous Leprosy
• Nasal crusts, serosanguinous highly infectious nasal discharge,
Severe nasal obstruction
• Earliest feature – nodular thickening of nasal mucosa seen in
the anterior end of inferior turbinate
• Collapse of anterior bridge of the nose
• Destruction of the anterior nasal spine
• Destruction of bony & cartIlagenous nasal septum
 Tuberculoid Leprosy
TUBERCULOID LEPROSY
• Lesions may extend upto nasal vestibule
• Nasal mucosa is not involved
• Cutaneous anesthesia is a feature

BORDERLINE LEPROSY
• Poor immunologic tolerance
• Skin around nose & eyes are involved
• Nasal mucosa is free of disease
 RHINOSCLEROMA
• A progressive granulomatous disease caused by the Frisch
bacillus (Klebsiella rhinoscleromatis) bacteria
• First described by Von Hebra (1870)
• Affects primarily the nose
• Other sites: nasopharynx, oropharynx, larynx, trachea, bronchi
& rarely the paranasal sinuses

• A diagnostic and therapeutic challenge due to its chronic

course, need for prolonged treatment and relapses
• Early detection is essential for proper treatment of this disease
 RHINOSCLEROMA - Stages
• Usually presents in the second and third decades of life, with a
female predominance
• Three sequential but overlapping clinicopathological phases:
• catarrhal/rhinitic
• non-specific rhinitis
• Foul smelling purulent rhinorrhea persisting for weeks to months
• granulomatous/florid
• Granulomatous nodules enlarge and coalesce – tapir nose
• Pathologic changes most characteristic
• K. rhinoscleromatis most frequently isolated
• clinical features are most prominent, incl epistaxis
• sclerotic/cicatricial
• - nasal deformities (tapir nose), anosmia, oral anesthesia, dysphonia,
dysphagia and stridor
• Adhesions and stenosis distort normal anatomy (Hebra nose)
Rhinoscleroma
 RHINOSCLEROMA - Histology
• Submucosal infiltration with granulation tissue
• Mikulicz cells (foam cells), Russell bodies
 RHINOSCLEROMA - Histopathology
• Catarrhal stage - nonspecific sub-epithelial infiltrate of
polymorphonuclear leukocytes with granulation tissue
• Granulomatous phase - diagnostic histological changes of RS
seen
• Dense infiltration by lymphocytes, plasma cells, Russell bodies
and the pathognomonic large Mikulicz cells; foamy
macrophages with numerous cytoplasmic vacuoles containing
viable and nonviable Klebsiella bacilli
• Fibrotic phase - Extensive fibrosis and less inflammatory cell
infiltration
 RHINOSCLEROMA - Diagnosis
• Biopsy - Dominance of plasma cells and absence of eosinophils,
Russell bodies, Mikulicz cells
• Culture of biopsy tissue in blood agar
• Periodic acid Schiff, Giemsa, Gram & silver stains
• Serology: Complement Fixation Test
• Imaging (In advanced cases, palatal sign - V shaped soft palate )
• Type III Klebsiella antigen - detected by immunohistochemistry
 RHINOSCLEROMA - Treatment
• Bactericidal Antibiotics - 4 - 6 weeks
• Streptomycin- 1g OD IM + Tetracycline 500mg QID orally
• Rifampicin- 450mg OD orally
• Surgery – during 4th stage. Reepthelization
• Use of Laser
• Plastic reconstructive surgery- correct nasal deformity
 SYPHILIS
• A STD caused by the spirochaete Treponema pallidum.
• It is classified into primary, secondary, tertiary and congenital
forms.
• Primary chancre of the nose is rare but may be seen on the skin
or within the vestibule.
• Secondary syphilis is an infectious systemic condition, with
rhinitis the most common nasal symptom.
• Tertiary syphilis may cause a perforation of the bony septum or
present as a subcutaneous nodule (gumma) which then
ulcerates.
• Congenital syphilis most commonly presents with ‘snuffles’, a
purulent rhinitis with eventual excoriation of the upper lip.
 SYPHILIS
• Usually diagnosed with serological tests.
• Smears from a lesion may show the organism
• Parenteral penicillin is the antibiotic of choice for all stages.
 YAWS
• A granulomatous disease involving skin & bones caused by
Treponema pertenue
Treatment
• Medical line : Benzathine penicillin 2.4 millon units
IM every week for 3 weeks with total dose of 7.2
millon units
• A. nasal alkaline wash
• B. Removal of nasal crusts
• Surgical Line: Removal of bony and cartilaginous
sequestra and correction of deformities are done
when disease becomes inactive
 Infective
Granulomatous Diseases
- Protozoa
 LEISHMANIASIS
• Caused by protozoa and conveyed to humans by female
Phlebotomine sandfly
• Genus Leishmania is named after Sir William Leishman, who
discovered the flagellate protozoa causing Kala-azar, the Indian
visceral leishmaniasis.
 LEISHMANIASIS
3 main types : Visceral (Kal-azar):VL, cutaneous (CL) &
mucocutaneous (affects the nose): MCL

Association with malnutrition, population displacement, poor

housing & weak immunity
Estimated 2 million cases annually – WHO
Mucocutaneous form leads to partial or total destruction of
nose, mouth & throat
Transmission : Bite of infected female phlebotomine sandflies
Co-exists with HIV
 LEISHMANIASIS - Diagnosis
• Clinical Features
• VL: Fever, Spenomegaly, Hepatomegaly, Anaemia, Weightloss,
Darkening of skin of feet, face and hand.
• CL: Painful ulcers, mostly restricted to skin
• MCL: Similar to CL but appears around margin of mouth and
nose.
 Diagnosis of Leishmaniasis

Parasitological diagnosis – Demonstration in the aspirations of spleen, liver,

lymph nodes or skin.
Aldehyde test of Napier:
Used for diagnosis of VL.
1-2 ml of serum from a patient of VL and a drop or 2 drops of 40% formalin is
added.
+ve test - Jellification to milk
It becomes positive 2-3 months after on set of disease and reverse to negative
6months after cure.
Serological diagnosis - DAT, ELISA,IFAT ELISA used for diagnosis as well as
epidemiological field survey
• Leishmanin(montenigro)
test:
• It’s a skin test.
• Induration of 5mm or
more is positive.
• Usually positive after 4-6
weeks after onset of case
of C.L and M.C.L.
• -ve in active phase of V.L
and becomes +ve in 75%
of people after recovery.
• Hematological findings:
• Progressive leucopenia,
anaemia,
• reversed Ab/Gb ratio.
• E.S.R is increased.
 Prevention And Treatment of Leishmaniasis

Control of reservoir: Active and passive case detection and

treatment. House to house visits and mass surveys.
Treatment: Pentavalent antimony compounds are used.
Recommended schedule: Sodium stibogluconate 20mg/KG daily for
20 days.
Second line drug: Pentamidine isethionate 3mg/KG for 10 days.
Amphoterecin B 1mg/KG for 20 days.
Milteforsine 2.5mg/KG for 4 weeks.
Animal reservoir: Dogs & rodent control programme
Sand fly control: insecticide.
Sanitation measures: Elimination of breeding places and
location of cattle shed at a far distance.
Personal prophylaxis - Health education, Individual protect
measures like Avoiding sleeping on the floor, Using fine mesh
nets, Keeping environment clean.
 Inflammatory
Granulomatous Diseases
 SARCOIDOSIS
• Inflammatory bacterial granulomatous disease.
• Systemic granulomatous condition of unknown aetiology
• Most frequently involves the lungs and intra-thoracic lymph
nodes, in over 90% of cases
• Sinonasal sarcoid - 1–4%
• Aetiology - unknown, immunological response to an
unidentified antigen in genetically predisposed individuals
(infectious or environmental)
 SARCOIDOSIS
• Facial pain - in one in five patients, hyposmia
• Granular appearance of nasal mucosa - ‘strawberry skin’
• Ulceration, crusting and adhesions
• Perforation of anterior nasal septum
• Involvement of nasal bones - soft-tissue mass or expansion of
the nasal bridge
• Thickening and purplish discoloration of the overlying skin -
lupus pernio
 SARCOIDOSIS
• CT scan - sclerosis and osteolysis of the nasal bones, soft tissue
mass

Septal perforation
 SARCOIDOSIS
MANAGEMENT
• Intra-nasal steroids, glucose and glycerine drops, nasal
douching
• FESS - limited role, only to remove obstructing lesions or for
secondary bacterial infection
• Septal surgery - avoided (increased rate of septal perforation)
 WEGENER’S GRANULOMATOSIS
• GRANULOMATOSIS WITH POLYANGIITIS
• Inflammatory bacterial granulomatous disease.
• Systemic condition characterized by granulomatous
inflammation of the respiratory tract and necrotizing vasculitis
affecting small- to medium-sized vessels with focal or
proliferative glomerulonephritis
• Autoimmune disease - strong association with antineutrophil
cytoplasmic antibodies (ANCA)
 WEGENER’S GRANULOMATOSIS
• Nose and sinuses - involved in more than 80%
• Symptoms - nasal obstruction, crusting, discharge and bleeding, facial pain
• Destruction of septum, turbinates and sinuses with formation of a single
large cavity
• CT scans - septal destruction, hyperostosis and opacification of sinuses
 WEGENER’S GRANULOMATOSIS
MANAGEMENT
• Corticosteroids, cytotoxic drugs
• Topical nasal treatment - douching, intra-nasal steroids, nasal
lubricants
• Endoscopic sinus surgery – only if refractory to medical
treatment, or for complications
• Endoscopic dacryocystorhinostomy – for epiphora, but avoided
in active disease
• Septal perforation - silastic septal button
 Churg Strauss Syndrome
• EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA)
• A syndrome of allergic angiitis and granulomatosis.
• ‘Eosinophil-rich and granulomatous inflammation involving the
respiratory tract, and necrotizing vasculitis affecting small to
medium-sized vessels, associated with asthma and
eosinophilia.
• It is the rarest of the ANCA-associated vasculitides
• The aetiology remains unknown but it may be an autoimmune
disease mediated by Th2-cells which activate both eosinophils
and neutrophils
• High levels of eosinophils and immunoglobulin E (IgE) in
vessels and tissues also suggests a direct role in the
development of the vasculitis, possibly an allergic response to
an unknown allergen.
 Churg Strauss Syndrome
• Other suggested precipitants include vaccination,
desensitization therapy and inhalation of various aeroallergens
• Asthma occurs in approximately 99% of patients, and is
characteristically late-onset
• In 83% of cases it precedes the vasculitis by an average of 8
years.
• Sinonasal symptoms are present in up to 93%.
• Allergic rhinitis and chronic rhinosinusitis with nasal polyps
occur in up to 75% of cases.
• Patients frequently complain of nasal obstruction, rhinorrhoea,
anosmia and sneezing.
• Nearly half may have undergone previous nasal surgery before
the diagnosis is made.
 Churg Strauss Syndrome
• Central nervous system involvement and Cardiac involvement
is less common but is the major cause of mortality.
• Three clinical phases of EGPA, although they do not necessarily
follow each other consecutively, are
• Prodromal phase – It may persist for several years, and is
characterized by adult-onset asthma and upper respiratory
tract inflammation.
• Phase two - peripheral eosinophilia, usually with pulmonary
infiltrates and eosinophilic gastroenteritis.
• The third phase is that of the systemic vasculitis, including
constitutional symptoms, neurological and cutaneous
involvement.
 Churg Strauss Syndrome
• A patient shall be said to have Churg Strauss syndrome if
he/she has satisfied any four or more of these six criteria.
 Churg Strauss Syndrome
• Diagnosis is based on the typical clinical features with a
peripheral eosinophilia of greater than 10% of the white
cell differential, plus tissue biopsy.
• ANCA is positive in 40–75% of cases, typically p-ANCA specific
for MPO but occasionally c-ANCA is seen.
• Treatment is primarily with high-dose systemic steroids.
• Those with organ or life threatening disease, or those requiring
persistently high doses of steroids may require treatment with
cyclophosphamide to induce remission
• Sinonasal symptoms are treated with topical nasal steroids,
douching and endoscopic sinus surgery as required.
 Cocaine-induced midline destruction
lesion
• Cocaine use via intra-nasal inhalation is known to cause
mucosal inflammation.
• cocaine abuse can cause granulomatous inflammation and
destruction of the nose, sinuses and palate that may be
clinically indistinguishable from GPA
• Due to the marked vasoconstrictive effect of cocaine
• Septal perforation seen in 5% of chronic users
• Cocaine-induced midline destructive lesions (CIMDL) can mimic
localized or systemic GPA as well as neoplasia.
• Nearly 90% have a positive p-ANCA against human neutrophil
elastase (HNE).
• ANCA is often positive in this condition, with PR3 reactivity in
more than 50 per cent
 Cocaine-induced midline destruction
lesion
• Symptoms - chronic nasal obstruction and bleeding, with
change in shape of the nose and nasal regurgitation as the
lesion progresses to destroy the nasal framework

• Examination confirms a variable degree of destruction of the

septum, turbinates, lateral nasal wall and floor
• Histology is very similar to that of GPA, with vasculitis and
fibrinoid necrosis
 Cocaine-induced midline destruction
lesion
• Cessation of cocaine abuse is most important.
• Patients must stop using cocaine to prevent further
progression.
• Topical nasal treatments may provide symptomatic relief
• 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.
 Cocaine-induced midline destruction
lesion
• Oral endoscopy showing a 1cm hard palate perforation with regular margins;
• Nasal endoscopy showing extensive destruction of the nasal septum and inferior
turbinate as well as the hard palate perforation;
• Coronal and axial CT showing extensive destruction of the septum and turbinates
 Giant Cell Granuloma
• Benign condition with Granuloma-like aggregates of giant cells
in a fibrovascular stroma
• Also known as ‘giant cell reparative granuloma’ or ‘giant cell
reaction of bone’.
• It was first described by Jaffe in 1953 in the jaws.
• These lesions commonly occur in children and young
adults, with a female to male preponderance of 2:1.

• Pain and swelling over the affected bone are the most common
symptoms, but diplopia, hearing loss, vertigo and tinnitus have
also been reported.
• The maxilla and mandible are most commonly affected
followed by the sphenoid and temporal bones.
•
 Giant Cell Granuloma
DIAGNOSIS
• CT shows expansile lytic lesions with a ‘soap bubble’ centre
and well-demarcated edges.
• The lesions are benign despite the presence of mitoses.
• Biochemical investigations (serum calcium, phosphate and
alkaline phosphatase) should be undertaken to distinguish it
from the brown tumour of hyperparathyroidism.
• Curettage alone is associated with recurrence in 15% of
cases and excision should be undertaken where possible.
 Giant Cell Granuloma
• Coronal CT scans of upper and lower jaw showing typical
appearances of cherubism.
 EOSINOPHILIC GRANULOMA
• A clonal proliferation of Langerhans cells associated with a
heterogeneous inflammatory infiltrate of eosinophils,
histiocytes, lymphocytes, plasma cells and neutrophils.
• Considered as a neoplastic condition with a variable clinical
course
• Also referred to as Langerhans cell histiocytosis or Langerhans
granulomatosis.
• about 85% of cases are detected in the first three decades of
life
• 60% are young children.
• Males are affected twice as frequently as females.
 EOSINOPHILIC GRANULOMA
• Eosinophilic granuloma predominantly occurs in bones.
• The skull is a common site of involvement, in particular the
temporal, frontal and parietal bones.
• The usual presentation is a painful swelling of the involved
bone, often for many months, associated with cervical
lymphadenopathy.
• Radiological evaluation shows punched-out bony lesions
and radiolucent areas around the teeth.
 EOSINOPHILIC GRANULOMA
• Treatment depends on whether or not the eosinophilic
granuloma is localized and/or solitary.
• Solitary, or ‘type II disease’, is at least five times more common
than polyostotic disease.
• With unifocal disease a combination of curettage/excision and
radiotherapy is usually curative, provided that no new lesions
develop within the first year.
• A proportion of patients will develop a generalized disease
with hepatosplenomegaly, lymphadenopathy, skin lesions
and further osseous lesions, so called ‘type I disease’.

• Alpha interferon and bone marrow transplantation have also

been used successfully.
 Cholesterol Granuloma
• This is a rare granulomatous foreign body reaction
to cholesterol crystals precipitated in the tissues.
• Also known as a chocolate cyst or orbital cholesteatoma.
• A male preponderance of 5:1
• It is presumed to result from haemorrhage due to trauma,
but there must also be abnormal ventilation and drainage
of the involved sinus.
• Although more common in the ear, these lesions may affect
the maxillary or frontal sinuses, producing bony expansion,
cosmetic deformity and displacement of adjacent
structures, such as the globe.
• The differential diagnosis includes a mucocele and a dermoid
or epidermoid cyst.
 Cholesterol Granuloma
Diagnosis
• Typical CT appearances are of a cyst-like expansion of the sinus
that does not enhance with contrast. A very high signal is
produced on all MRI sequences.

Treatment
• Surgical drainage and marsupialization is required, by
whichever approach facilitates complete removal of the
granulation tissue to prevent recurrence
 Neoplastic
Granulomatous Diseases
 T-Cell Lymphoma
• A rare non-Hodgkin’s lymphoma (NHL) that causes destruction
of the midface.
• Most common in south-east Asia and South America.
• Extranodal NK/T-cell lymphoma (ENKTCL) appears to be caused
by the Epstein-Barr virus.
• Representative biopsy from beneath necrotic tissue is required
for diagnosis.
• Imaging shows widespread bony and soft tissue destruction.
• Early diagnosis is important, as the prognosis for widespread
disease is much worse.
• Treatment is with radiotherapy plus or minus chemotherapy.
• Long-term follow-up is required because there may be late
relapses.
 T-Cell Lymphoma
• The tumour consists of neoplastic T-lymphocytes with a
significant inflammatory infiltrate.
• Immunohistochemistry is usually positive for CD56, CD2 and
cytoplasmic CD.
• This is an aggressive tumour but tends to have a dramatic
response to radiotherapy, which is therefore the recommended
treatment for localized disease

significant
early central
ulceration destruction
 Conclusion
• Granulomatous nasal diseases represent an important and
often neglected subgroup of diseases with significant
morbidity & mortality
• Any patient with blood-stained discharge and crusting in the
nose has a granulomatous condition until proven otherwise
• Early diagnosis is crucial to avert more severe systemic
disease
 References
Scott Brown’s text book of otorhinolaryngology 8th edition
Logan Turner’s textbook of Diseases of the Nose, Throat and Ear 11 th Edition
Cummings text book of otorhinolaryngology- 6 th edition
Mohan Bansal text book of ENT – 2nd edition
Thank you..