Granulomatous diseases of nose and pns

Granulomatous diseases of nose and pns

1. Granulomatous diseases of nose & pns Dr.divya raana Ms ent pg

2. granuloma Organized collection of macrophages, known as epithelioid cells, which fuse to form multinucleated giant cells. Encountered in a number of infective, inflammatory and neoplastic conditions of the nose and sinuses. Also seen in various conditions in which vasculitis plays a role.

3. INFECTIVE CONDITIONS

4. BACTERIAL

5. TUBERCULOSIS OF THE NOSE

6. Lupus vulgaris Cutaneous tuberculosis associated with painful skin lesions  face around the nose, eyelids, lips, cheeks, ears & neck. Macroscopic appearance: 1. Nodular form (Most Common) 2. Ulcerative form 3. Sinus granuloma Nodular form: Indolent & Chronic form. Affects skin & mucous membrane of nose. Young Female : Male = 2:1 Temperate climate > In Tropics.

7. Usually begins in the Vestibule (M.C. site = Mucocutaneous Junction of the nasal septum) & then extends to the adjoining skin & mucosa. HISTOPATHOLOGY: Tuberculous granuloma In the center these lesions show collection of reticuloendothelial cells. These cells soon necrose & coalesce. Necrotic center is surrounded by a ring of living RE cells which further are surrounded by ring of Lymphocytes, Plasma Cells & Fibroblasts. Giant cells are found scattered throughout the tubercle & are usually multinucleated.

8. SYMPTOMS: Nasal discharge Nasal obstruction Presence of non-foul smelling crusts Epistaxis Evening rise of temperature The typical early lesion in the nose is a reddish firm nodule at the mucocutaneous junction of the nasal septum. These nodules are known as “Apple Jelly Nodules” The nodules may coalesce & break down to form characteristic ulcers with a pale granular base & undermined edges

9. DIAGNOSTIC FEATURES OF LUPUS NODULES: 1. Blanching 2. Bacterial examination 3. Biopsy Blanching – To highlight the apple jelly nodules, blood should be expressed from adjoining tissues by applying pressure with a glass slide, this will make the pinkish lupus nodules to stand out in contrast. Bacteriological examination may demonstrate Acid Fast Bacilli. Culture of the organism – Lowenstein-Jensen slope, Culture can now be accelerated by BACTEC. Biopsy is always diagnostic.

10. COMPLICATIONS: Pulmonary tuberculosis, Dacryocystitis , Corneal Ulceration, Nasopharyngeal Lupus, Lupus Of Face, Atrophic Rhinitis ULCERATIVE FORM: Ulcers are commonly found on the anterior part of the nasal septum, inferior turbinate & anterior choanae. This usually involves the cartilaginous nasal septum or the inferior turbinate. Ulceration of nasal mucosa is usually followed by fibrosis & contraction causing distortion of alae nasi.

11. If turbinates are involved  lining ciliated columnar epithelium is not renewed  lead to formation of secondary atrophic rhinitis. Septum may also undergo perforation, but in these cases classically the cartilaginous portion alone is involved & hence there is no sinking of the nasal bridge.

12. SINUS GRANULOMA Isolated sinus involvement may occur without any signs or symptoms in the nose. Diffuse soft tissue swelling & multiple discharging sinuses in the supraorbital region secondary to osteomyelitic involvement of the frontal bone is seen. Involvement of the orbit & its nerves may occur. CT & MRI are nonspecific – demonstrate a soft tissue mass with or without bone destruction The diagnosis is established by an External or Endoscopic Biopsy.

13. SPECIFIC INVESTIGATIONS: Montaux test, AMTD (Amplified Mycobacterium Tuberculosis Detection), PCR, Fluorescence microscopy AMTD - uses principles of transcription mediated amplification of rRNA of Mycobacterium TB. Detection of tRNA indicates the presence of actively multiplying MTB. TREATMENT: Responds well to antituberculous drugs.

14. LEPROSY Causative Organisms – M. Leprae. M. Leprae  cannot be cultured on an artificial medium, it can nevertheless be inoculated into experimental animals, particularly immunologically deficient mice, to produce a disease similar to that in man. Based on the Host's Immunological Status & Clinical, Histological & Microbiological features leprosy divided into types.

15. Tuberculoid leprosy: Immunocompetent patients Solitary lesions(anesthetic patches) Involvement of one / more related sensory or motor nerves. Nasal Vestibule involved without mucosa involvement Isolated cranial nerve palsies (V and VIIth ) have also been documented

16. Lepromatous Leprosy : Least immunological resistance to the organism. Diffuse infiltration of skin, nerves and mucosal surfaces. Cutaneous infiltration is more common over the edges of pinna, chin, nose and eyebrows. Highly infectious nasal discharge.

17. Signs and Symptoms : Earliest sign - nodular thickening of nasal mucosa Crust formation Nasal obstruction (out of proportion) Sero sanguineous discharge Nodules are paler than the surrounding mucosa with a yellowish tinge. Commonly begin at the anterior end of inferior turbinate, progresses to gross inflammation of nasal mucosa.

18. Both the bony and cartilaginous portions of nasal septum are destroyed due to perichondritis and periosteitis (nasal bridge collapse) Anterior nasal spine destruction seen Radiographs showing the absence / erosion of anterior nasal spine are virtually diagnostic of Lepromatous leprosy Hyposmia is seen in 40% Nasal mucosa scraping microscopy shows typical cigar pattern Lepra bacilli

19. Borderline leprosy Poor immunological resistance May progress to lepromatous leprosy Skin lesions are more numerous and are seen around eyes, nose and mouth In pure borderline lesions - nasal mucosa involvement is not seen Nasal mucosal involvement suggests progression to LL.

20. Diagnosis Early diagnosis is essential since nasal discharge is principle route of transmission of the disease Clinical findings of anaesthesia on the skin, thickened peripheral nerves and skin lesions, & supplemented by bacteriological examination. Confirmed by demonstration of M. leprae on microscopy of the nasal discharge or scrapings of nasal mucosa, or histopathology of nasal mucosa. Skin biopsy in tuberculoid leprosy

21. Treatment Dapsone reduces the bacilli count of nasal discharge to zero or near zero within a couple of months BUT increased resistance. Modern drugs like Rifampicin and Clofazimine can reduce the bacilli count to zero within 10 days Triple therapy: Rifampicin – 600 mg on first two days of a month taken before breakfast Clofazimine – 100 mg on alternate days for three times a week Dapsone – 100 mg a day

22. Rifampicin stopped after 3 months Rest 2 continued till 9 months Intranasal Rifampicin much faster than oral route Nasal douching for removal of crusts

23. syphilis Nasal syphilis can occur at any age Uncommon as signs symptoms difficult to elicit in early stages especially if antibiotics are given Causative org. – Treponema Pallidum(Spirochete) Pathogenesis - The organisms usually reside and multiply in the perivascular lymphatics of the blood vessel wall. Local host reaction results in an infiltration by polymorphs, activated lymphocytes (CD4+ and CD8+), plasma cells and histiocytes which are typical of syphilis.

24. The resultant endarteritis of small blood vessels with secondary hypertrophic changes in the endothelium, may lead to endarteritis obliterans and luminal obliteration. HPE – Diagnosis is purely histopathological Characterized by oedema , stromal infiltration with lymphocytes, plasma cells and endothelial cells Perivascular cuffing by these cells and endarteritis will cause a reduction in the lumen of blood vessels causing necrosis and ulceration.

25. Nasal syphilis can be classified into: Primary syphilis Secondary syphilis Tertiary syphilis Primary syphilis : Also known as Chancre, seen at external nose or inside the vestibule. Appears as a hard, non painful ulcerated papule always associated with enlarged, rubbery, and non tender lymphadenopathy

26. A sore or chancre develops at the site of inoculation 10-90 days(average21 days) following infection These lesions undergo spontaneous resolution within 6-10 weeks Should be differentiated from malignant neoplasms and furunculosis Malignant neoplasms – affect older ages, relentless progression Furunculosis – Painful condition, progresses to suppuration. Treatment : Anti syphilitic Rx - i.m. penicillin. The chancre may be cleansed with 1:2000 solution of perchloride of mercury and the surface smeared with 2% yellow mercuric oxideointment . The following points should be borne in mind : Cultures from the surface of the lesion will always be negative.

27. Smears when examined under dark ground illumination will show the spirochete Treponema palladium Serological tests for syphilis may be positive– VDRL, TPHA, FTA-ABS. ( TheVDRL is a non treponemal test and is relatively nonspecific but becomes positive early (four to fiveweeks ) and correlates well with disease activity.) A biopsy from suspicious lesion may confirm the diagnosis

28. Secondary Syphilis Most infectious of all the three stages Symptoms6-10 weeksafter inoculation Secondary syphilis is rarely recognized in the nose, as mucous patches hardly ever occur on such a thin, attenuated mucous membrane. The symptoms include: Simple catarrhal rhinitis(persistent) ,Crusting & fissuring of nasal vestibule Other secondary lesions like mucous patches in the pharynx are also seen Roseolar / papular skin rashes ,enlarged non tender lymph nodes. The scar of the primary lesion in the genitals or elsewhere may be visible.

29. Serological tests for syphilis are positive. Dark-field examination of moist or intentionally a braided dry lesions may demonstrate the organism. Lymph node aspirates or tissue specimens (lymph nodes, liver, skin or mucous membrane) may also be examined by silver stains or immunofluorescence for detection of spirochaetes These patients respond to antisyphilitic drugs.

30. Tertiary Syphilis : Only 1/3rd of secondary syphilis cases progress to show clinical manifestations of tertiary syphilis. This stage is commonly encountered in the nose. Lesion is also known as gumma . This lesion invades the mucous membrane, periosteum and bone. Thebony portion of the nasal septum is frequently affected causing septal perforation.

31. Rarely the following portions of then nose can also be involved : Lateral nasal wall Frontal sinus Nasal bones Floor of the nose Symptoms include: 1. Pain / headache( worseduring night) 2. Swelling / obstruction of nose- swelling may bediffuse / localized associated with offensive discharge, bleeding and crusting of the nose 3. Olfactory acuity diminishes 4. Perforation of bony portion of nasal septum associated with collapseof the bridge of the nose can cause structural damage to the nasal architecture 5. There may also be associated secondary atrophic rhinitis.

32. Nasal complications of gumma : 1. Secondary infections with pyogenic organisms 2. Sequestration 3. Perforation of bony portion of nasal septum, palate or nasal walls 4. Collapse of bridge of nose with deformity of nose 5. Scarring / stenosis of nasal passages 6. Atrophic rhinitis 7.Intracranial complications due to involvement of meninges

33. Treatment : 1.DOC for all stages– parentral Penicillin 2.Alkaline nasal douches one to three times a day. 3.Local yellow mercury oxide ointment. Diluted mercuric nitrate ointment should be applied freely to the nasal vestibules. 4.Atrophic rhinitis and deformity may persist after the disease is cured and this may need further surgery.

34. Congenital syphilis “Snuffles”: Any of the lesions of secondary and tertiary forms of syphilis of nose can occur. Classically begin during the3rd week of life. May appear around 3rd month also At First – simple catarrhal rhinitis. Later becomes purulent with fissuring and excoriation of the nasal vestibule and upper lip.

35. Gummatous and destructive lesions occur most commonly at puberty. Mucous membrane, periosteum and bonemay all beaffected . Other stigmata of syphilis, particularly Hutchinson's incisors and Moon’s molars, interstitial keratitis, corneal opacitiesand sensorineural deafnessmay bepresent , and there may be a family history of syphilis, miscarriages and still births.  Serologic tests for syphilis will invariably be negative

36. Treatment : In snuffles, the airway must be restored for suckling The nasal discharge is removed by gentle suction and irrigation and the use of local drops of 0.5 percent ephedrine solution or simple normal saline, and by hyper extending the head before feeding In the tertiary forms, simple nasal toilet by syringing with isotonic alkaline douche solution will remove the crusts and discharge, and yellow mercuric oxide ointment may be applied frequently to the nasal vestibules

37. rhinoscleroma Progressive granulomatous disease commencing in the nose and later extending into the nasopharynx and oropharynx, the larynx and sometimes the trachea and bronchi. Laryngeal involvement may occur in almost half the cases and the disease is perhaps better designated as respiratory scleroma , rather than rhinoscleroma . Aetiology is multifactorial and usually seen in low socio economic status and poor domestic hygiene.

38. Pathology : Caused by gram negative bacilli Klebsiella rhinoscleromatis / Frisch bacilli / diplo bacillus. F>M, This organism could be a secondary invader following a viral infection. Resides intracellularly and can be difficult to isolate in the laboratory. The characteristic histological features include granulomatous tissue infiltrates in the submucosa, characterized by the presence of plasma cells, lymphocytes and eosinophils.

39. Scattered large foam cells (Mikulicz cells) which have a central nucleus and a vacuolated cytoplasm containing frisch bacilli and Russel bodies. The Russel bodies resemble plasma cells with an eccentric nucleus and deep eosin staining cytoplasm. Histochemical studies have indicated a high content of mucopolysaccharides around the walls of the bacillus It is hypothesized that this may be responsible for the protection afforded to the organism from antibiotics and the hosts antibodies

40. Clinical features : Three stages– 1.The atrophic stage: The features resemble that of atrophic rhinitis, including crust formation and a foul smelling discharge (carpenter’s glue) 2. Granulomatous or proliferative (or nodular) stage: Nonulcerative nodules develop which are at first bluish red and rubbery and later become paler and indurated. These nodules never break down but fibrose and progressively decrease in size.

41. 3. Cicatrizing stage: Adhesions and stenosis distort the normal anatomy. The disease may extend to involve the lacrimal sac, maxillary sinus, nasopharynx, hard palate, trachea and main bronchi Bone involvement has also been reported The shape and contour of the nose changes causing a condition known as“Tapir’s nose”(woody feeling on palpation of nose) , hebra nose Lymphatic spread is uncommon because of extensive fibrous tissue deposition This deposition blocks the lymphatics. Occasionally, malignant change has been reported Gothic Sign – because of fibrosis the pharyngeal wall is pulled up and resembles the top of a church.

42. Complications :   1. External nosedeformity 2. Vestibular stenosis 3. Cicatrization of soft palate 4. Nasal regurgitation 5. Tracheal stenosis

43. Diagnosis : Levin test (complement fixation test) : High titres of antibodies against K. Rhinoscleromatis has been demonstrated. Diagnosis in the atrophic stage is extremely difficult and the disease is usually recognized only in the granulomatous or cicatrizing stage MRI in the hypertrophic stage shows a soft tissue mass with mild to marked high signal intensity in both Tl- and T2-weighted images. Microbiological examination and a confirmatory Biopsy

44. Treatment : Usually self limiting course of its own accord ending in the cicatrizing stage, the organism can nevertheless be extremely difficult to eradicate by antimicrobials. Bactericidal antibiotics in large doses are given for a minimum of four to six weeks and are continued until two consecutive cultures from biopsy material are proven negative. Traditionally Used : streptomycin ( im 1gm for 4-6wks) and tetracyclines. Recently used : Oral rifampicin (450mg for a period of 6 weeks), sulphamethoxazole -trimethoprim combination, and ciprofloxacin.

45. Local application of 2 % acriflavin for a period of 8 weeks has been noted to be both efficacious and nontoxic. Rifampicin : Nasal Instillation - 1 Amp of rifampicin 125mg diluted in 20mL saline solution and 2mL of the solution is applied twice daily locally for a period of 8 weeks. Nasal Infiltration - 1 Amp of rifampicin 125mg infiltrated into granulomas every other day Irradiation to a total dose of 3000-3500 Gy over three weeks destroys scleroma but, with the currently available antimicrobials, is unlikely to be required

46. Fungal conditions

47. RhinospoRidiosis Chronic granulomatous infection that affects the nasal mucosa, ocular conjunctiva and other mucosa. Aetiological Agent : Rhinosporidium seeberi 15-40 yrs age group, M>F It has long been unclear whether this organism is a fungus Natural habitat - stagnant pools of fresh water The disease is most prevalent in rural districts, among persons bathing in public ponds or working in stagnant water, such as rice fields

48. Classification : Benign : Nasal - Septal Mucous Membrane, Floor of Nose/ Spur Nasopharyngeal - Nasopharyngeal surface of soft palate. Mixed - Nasolacrimal Bizzare - Conjuctival / Cutaneous Malignant : Generalised

49. Clinical Manifestations : Nose is the commonest site(70% cases) The fungus causes the production of large sessile or pedunculated lesions that affect one or both nostrils Goes unnoticed till nasal obstruction develops, Nasal discharge& epistaxis A/R – reveals leafy, papular or nodular, smooth-surfaced lesions that become pedunculated and acquire a papillomatous or proliferative appearance The lesions are pink, red or purple in colour , vascular (bleeds on touch) studded with white dots

50. Spontaneous remission is unusual and, left untreated, the mass will continue to enlarge Diagnosis : HPE of tissue sections.  These contain large, round or oval sporangia up to 350 µm in diameter, with a thick wall and an operculum Management Options : The treatment of choice is surgical excision of lesions, with cauterization of base No drug treatment has proved effective Dapsone is said to prevent maturation of sporangia & promotes fibrosis, 50mg BD, 3 months preop and 6 months postop.

51. INFLAMMATORY CONDITIONS

52. SARCOIDOSIS Systemic granulomatous condition of unknown aetiology . May affect any part of the body, but most frequently involves the lungs and intra-thoracic lymph nodes, in over 90% of cases. 6–16 per 1,00,000 population per annum. Young adults with a peak onset between the third and fourth decades. F > M

53. AETIOLOGY: The aetiology of sarcoidosis remains unknown, but theories suggest an immunological response to an unidentified antigen in genetically predisposed individuals. The antigens may be infectious or environmental, and possibilities include mycobacteria, fungi, chemicals such as beryllium and zirconium, pine pollen and peanut dust. There are abnormalities of both the cell-mediated and humoral immune systems.

54. Clinical features: Multisystem disease It involves the upper respiratory tract in up to 18% of cases. Nasal symptoms commonly include obstruction, crusting, bleeding or facial pain A staging system for sinonasal involvement has been described to help treatment: Stage I : Reversible involvement of the nose alone. Stage II : Moderate but potentially reversible disease of the nose and/or limited sinus involvement Stage III : Severe, irreversible and extensive sinonasal disease.

55. The nasal mucosa often has a characteristic granular appearance, sometimes referred to as a ‘strawberry skin’ because of the tiny pale granulomas against hypertrophic erythematous mucosa. Friable ,ulceration, crusting and adhesions. The nasal bones may be involved; patients may present with a soft-tissue mass or expansion of the nasal bridge. This may be associated with thickening and purplish discoloration of the overlying skin known as lupus pernio, which is a cutaneous manifestation of chronic systemic sarcoid.

56. Salivary gland enlargement is seen in 5–10% of cases. More rarely associated with facial palsy and uveitis when it is termed Uveoparotid fever or Heerfordt’s syndrome. The larynx is involved in 1–5% of cases, most commonly the supraglottis (85%), with symptoms of cough, hoarseness, dysphagia and more rarely stridor. Tracheal involvement is rare but bronchial sarcoid is common. Chronic progressive pulmonary fibrosis occurs in 25% of cases of chronic pulmonary sarcoid.

57. DIAGNOSIS: No test is pathognomonic. Diagnosis relies on a combination of clinical features, imaging, histology, biochemical and serological testing and the exclusion of other granulomatous diseases. Biopsy should target potentially affected tissue such as the lung, skin and lymph nodes; lip biopsy of minor salivary glands can be useful. Serum angiotensin converting enzyme (ACE) is elevated in up to 85% during active disease, but this is non-specific Imaging of the lower respiratory tract shows varying degrees of pulmonary involvement, which allow staging of the disease Plain X-rays of the nasal bones may show rarefaction or punctate osteolysis CT will also demonstrate secondary involvement of the sinuses though will not distinguish between active disease and secondary infection.

58. TREATMENT: Variable clinical course that often correlates with the mode of presentation; Acute onset with arthritis and erythema nodosum is usually self-limiting, more insidious onset with lupus pernio. Sinonasal involvement tends to be more chronic. Two-thirds of patients will spontaneously remit. 10–30% follow a chronic course with relapses despite systemic therapy. Steroids remain the mainstay of treatment for severe disease (lack of controlled studies), with the addition of agents such as methotrexate to reduce the steroid dose if required. Hydroxychloroquine  cutaneous and sinonasal sarcoidosis, response rate is less than 50% and there is a risk of ocular toxicity. TNF-alpha inhibitors such as infliximab and etanercept

59. Nasal symptoms  intra-nasal steroids, glucose and glycerine drops, and nasal douching. Surgery has a limited role, particularly in the presence of active disease, although endoscopic sinus surgery can be undertaken to remove obstructing lesions or for secondary bacterial infection. Septal surgery should be avoided if possible as an increased rate of septal perforation. Symptomatic laryngeal disease that fails to respond to systemic treatment may be treated with transoral laser and intra- lesional steroid injections

60. GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S) Systemic condition characterized by granulomatous inflammation of the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels with focal or proliferative glomerulonephritis. Triad of upper airway, lung and renal disease 40–50 years. Men and women are equally affected

61. Aetiology Autoimmune disease, given its strong association with antineutrophil cytoplasmic antibodies (ANCA). ANCA binds to and activates neutrophils  release oxygen radicals, inflammatory cytokines and lytic enzymes and adhere to and kill vascular endothelial cells. ANCA may also induce immune complex formation. In GPA, cytoplasmic ANCA (c-ANCA) are specific. There is evidence that a mimic peptide may be introduced via an exogenous infective agent such as Staphylococcus aureus . This theory is supported by the higher rate of chronic nasal S. aureus carriage in patients with GPA, in whom it is associated with more frequent relapses.

62. Clinical features Necrotizing granulomas with vasculitis of the upper and lower respiratory tracts. Focal necrotizing glomerulonephritis. Nose and sinuses involved in 80% cases There may be intra nasal destruction of bone and cartilage leading to collapse of nasal bridge Rapid diagnosis remains of great importance since a fulminating course with a fatal outcome can occur in as little as 48 hours Nasal symptoms : blood stained nasal discharge, crusting, granulation and can even lead to septal perforations

63. Minor nasal surgery and/or repeated biopsies during this period may add to the problem Many patients may complain of significant facial pain There can be destruction of the eye, orbit, palate, oral cavity,oropharynx and sometimes middle ear Oral Symptoms : Most common - hyperplastic granular lesion of the gingiva If a tooth is lost, the socket may fail to heal Extensive ulcerative stomatitis has also been reported Otological Symptoms : AOM , SOM There may be deafness, pain and suppuration. Facial nerve paralysis has also been recorded.

64. Laryngeal and Tracheal symptoms : Laryngeal involvement is unusual, but when present the subglottis and upper trachea are most commonly affected May lead to laryngotracheal obstruction Biopsies nonspecific and are contraindicated leading to further inflammation and scarring. positive c-ANCA test seen in idiopathic subglottic stenosis

65. Diagnosis : The cANCA test is positive in 95 percent of patients with generalized active disease. ESR is found to be elevated Serum angiotensin enzyme levels are increased Presenceof vasculitis Granulomasareof epitheloid cell type Multinucleated giant cells have also been demonstrated

66. Treatment : The following drugs can be used in combination. 1. Steroids – Prednisolone60 – 80mg/day 2. Cyclophosphamide – 2mg/kg/day (side effects including alopecia, haemorrhagic cystitis, opportunistic infections and malignancies; leukaemia and lymphoma are 11 times more likely, and there is a 33-fold increase in the risk of bladder cancer. ) 3. Azathioprine – can be used instead of Cyclo phosphamide in doses of 200mg /day. The multicentre randomized double-blind RAVE (Rituximab for ANCA-Associated Vasculitis) trial also suggested that rituximab may be superior in relapsing disease. Topical nasal treatment such as douching, intra-nasal steroids and nasal lubricants may be helpful for symptomatic relief of crusting and bleeding.

67. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG STRAUSS) ‘Eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium-sized vessels, associated with asthma and eosinophilia.’ Rarest of the ANCA-associated vasculitis Annual incidence of 0.5–4 per million. In asthmatics this incidence rises to 67 per million. The mean age at diagnosis is 50 years (range 4–75 years). M=F

68. AETIOLOGY: 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. Autoimmune disease Th2-cells Activate eosinophils and neutrophils. Tissue injury release of cationic proteins and cytokines

69. Clinical features : Asthma occurs in approximately 99% of patients Sinonasal symptoms are present in up to 93% . Allergic rhinitis and chronic rhinosinusitis with nasal polyps occur in up to 75% of cases. Nasal obstruction, rhinorrhoea , anosmia and sneezing, as well as crusting and epistaxis Neurological symptoms : mononeuritis multiplex, peripheral polyneuropathy Cutaneous symptoms : Papules and nodules Cardiac : granulomatous myocarditis.

70. Phase one: prodromal  persist for several years and is characterized by adult-onset asthma and upper respiratory tract inflammation. Phase two : characterized by peripheral eosinophilia, usually with pulmonary infiltrates and eosinophilic gastroenteritis. Phase three : systemic vasculitis, including constitutional symptoms, neurological and cutaneous involvement.

71. Treatment : The following drugs can be used in combination. 1. Steroids – Prednisolone60 – 80mg/day 2. Cyclophosphamide – 2mg/kg/day (side effects including alopecia, haemorrhagic cystitis, opportunistic infections and malignancies; leukaemia and lymphoma are 11 times more likely, and there is a 33-fold increase in the risk of bladder cancer. ) 3. Azathioprine – can be used instead of Cyclo phosphamide in doses of 200mg /day.

72. GIANT CELL GRANULOMA Granuloma-like aggregates of giant cells in a fibrovascular stroma characterize this benign condition otherwise referred to as ‘giant cell reparative granuloma’ or ‘giant cell reaction of bone’. children and young adults Female to male preponderance of 2:1.

73. Clinical features : Pain and swelling over the affected bone  common symptom Diplopia, hearing loss, vertigo and tinnitus have also been reported. The maxilla and mandible are most affected followed by the sphenoid and temporal bones. Bilateral symmetrical involvement of the jaws  cherubism , a rare inherited childhood condition Diagnosis : CT shows expansile lytic lesions with a ‘soap bubble’ centre and well-demarcated edges.

74. Histologically, the granuloma has a cellular fibroblastic stroma containing aggregates of giant cells. These cells are smaller and have fewer nuclei than in a true giant cell tumour Treatment Curettage alone is associated with recurrence in 15% of cases and excision should be undertaken where possible.

75. neoplastic

76. EXTRANODAL NK/T-CELL LYMPHOMA Previously  Midline lethal granuloma Destruction of the midface Most common in south-east Asia and South America most common in the fifth or sixth decades. M>F Aetiology ENKTCL appears to be caused by the Epstein-Barr virus.

77. Clinical features : Arises in the nasal cavity Spreads to involve adjacent structures including the orbits, oral cavity, skin and paranasal sinuses Initially causes symptoms of obstruction, discharge and bleeding. Progressive destruction of the nasal skeleton and overlying skin occurs Diagnosis : Early diagnosis is important, as the prognosis for wide- spread disease is much worse. Biopsy material from tissue beneath the slough and crust is essential.

78. Histologically, the infiltrates are polymorphic and atypical cells may to be arranged in a necrotizing angiocentric growth pattern. The tumour consists of neoplastic T-lymphocytes with a significant inflammatory infiltrate. Immunohistochemistry is usually positive for CD56, CD2 and cytoplasmic CD3. Treatment Very good response to radiotherapy. Local recurrence rates range from 31–67%, with an over all 5-year survival rate of 38–45%.

79. Thank you