Granulomatous lesions of nose

This presentation discusses the various granulomatous lesions involving nose and sinuses.

1. Granulomatous lesions of nose Balasubramanian thiagarajan Otolaryngology online

2. Introduction Fea tures 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 Otolaryngology online

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

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

5. 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 Otolaryngology online

6. Inflammatory Wegner’s granulomatosis Sarcoidosis Churg-strauss syndrome Cholestrol granuloma Eosinophilic granuloma Otolaryngology online

7. Neoplastic T-cell lymphoma (Lethal midline granuloma) Otolaryngology online

8. 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 Otolaryngology online

9. 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 Otolaryngology online

10. Otolaryngology online

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

12. 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). Otolaryngology online

13. 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 Otolaryngology online

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

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

16. 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 Otolaryngology online

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

18. 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 Otolaryngology online

19. 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 Otolaryngology online

20. 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) Otolaryngology online

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

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

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

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

25. 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 Otolaryngology online

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

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

28. 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 Otolaryngology online

29. 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. Otolaryngology online

30. 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 Otolaryngology online

31. 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 Otolaryngology online

32. 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 Otolaryngology online

33. 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 Otolaryngology online

34. 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 Otolaryngology online

35. 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 Otolaryngology online

36. 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 Otolaryngology online

37. 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 Otolaryngology online

38. 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 Otolaryngology online

39. 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 Otolaryngology online

40. 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 Otolaryngology online

41. 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 Otolaryngology online

42. Nasal tuberculosis - complications Dacryocystitis Lupus of the face Atrophic rhinitis Development of epithelioma - very rare Otolaryngology online

43. 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 Otolaryngology online

44. 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 Otolaryngology online

45. 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 Otolaryngology online

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

47. 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 Otolaryngology online

48. 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. Otolaryngology online

49. 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 Otolaryngology online

50. 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. Otolaryngology online

51. 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 Otolaryngology online

52. Rhinosporidiosis – Special characteristics More than 90% of these patients have been reported from India and S rilanka 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 Otolaryngology online

53. 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) Otolaryngology online

54. 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 Otolaryngology online

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

56. 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 Otolaryngology online

57. 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 7 th division size becomes 100 microns Fully mature sporangium – 150 microns Mature spores are found in the centre and immature ones at the periphery Otolaryngology online

58. 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 Otolaryngology online

59. 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 Otolaryngology online

60. Clinical classification Nasal Nasopharyngeal Mixed Bizarre (ocular / genital) Malignant rhinosporidiosis (cutaneous) Otolaryngology online

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

62. 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 Otolaryngology online

63. 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 Otolaryngology online

64. Nasal rhinosporidiosis - Features Chronicity Recurrence Dissemination Otolaryngology online

65. Chronicity - Reasons Antigen sequestration Antigenic variation Immune suppression Immune distraction Immune deviation Binding of host immunoglobulins Otolaryngology online

66. Treatment Surgery Dapsone 100 mg/day – 6 months Otolaryngology online

67. Otolaryngology online Thank you