Granulomatous diseases of nose

A class on granulomatous lesions of nose and PNS

1. Granulomatous Diseases
of Nose
Dr Krishna Koirala
MS (ENT-HNS)
2019-12-03

2. Focalareaofchronic
inflammationproducedby
circulatingmonocytesas
partofanimmunologic
process
Granuloma

3. Classification

4. 1. Infective
•Bacterial
–Tuberculosis,Lupusvulgaris,Leprosy,Scleroma,
Syphilis,Actinomycosis
•Fungal
–Rhinosporidiosis,Aspergillosis,Mucormycosis,
Candidiasis,Histoplasmosis,Blastomycosis
•Protozoal-Leishmaniasis
•Viral-AIDS

5. 2.Inflammatory
–Sarcoidosis
–Wegener’sgranulomatosis
–Eosinophilicgranuloma,ChrugStrausssyndrome
(Allergicgranulomatosis),Cholesterolgranuloma,
Foreignbodygranuloma
3.Neoplastic
–Tcelllymphoma,Stewart’sGranuloma

6. Tuberculosis
•Associatedwithprimarypulmonarytuberculosis
•Affectsanteriorseptumandanteriorpartofinferior
turbinate
•C/F:
–Nasalobstruction,discharge,pain
–Red,nodularthickening,ulceration+/-
–Perforation/scarringofthecartilagenousseptum

7. Lupus vulgaris (Cutaneous tuberculosis)
•Indolentandchronicformof
tuberculouslesionsoftheskinof
nose
•Maycauseepitheliomatous
reactionofsquamousepithelium
•Applejellynodulesondiascopy
•Scarringmoresevere

8. Diagnosis
•History of previous TB or active pulmonary TB
•Mantoux test : Skin test for delayed hypersensitivity
by injection of purified protein derivative
•Microscopy : Ziehl-Neelsen stain
•Culture (6-8 weeks) : Lowenstein -Jensen medium,
Middlebrook's medium
•Polymerase chain reaction (PCR)
•Histopathological examination of biopsy specimen

10. Treatment
•2 monthscourse of therapy with
–Isoniazid (5 mg/kg)
–Rifampin (10 mg/kg)
–Streptomycin (15 mg/kg ) / Ethambutol (15 mg/kg)
•6 more months of isoniazid & rifampin
•2 HRE+6 HR
•Surgical reconstruction

11. Leprosy (Hansen’s Disease)

12. •Organism: Mycobacterium leprae
•Nasal Findings:
–Excessive nasal discharge, red swollen mucosa,
crusting & bleeding
–Nodular thickening of mucus membrane, ulcer,
perforation of cartilaginous septum
–Leonine facies: (characteristic of lepromatous )
nasal destruction, lack of eye brow & eyelashes,
altered pigmentation & ocular alterations

14. •Late sequelae:
–Atrophic rhinitis
–Saddle nose deformity
–Destruction of anterior nasal spine
–Retraction of columella
•Diagnosis: scraping of mucosa and biopsy, acid fast
bacilli in foamy appearing histiocytes (lepra cells)
•Treatment:
–Dapsone, Rifampicin, Clofazimine

15. Syphilis

16. •Organism:Treponema
pallidum
•Endarteritisofsmall
bloodvesselswith
secondaryhypertrophic
changesinendothelium
leadstoendarteritis
obliteransandluminal
obliteration

17. Congenital syphilis
•Nosealwaysinvolved
•InInfants:
–‘Snuffles’3weeks-3months
–Simplecatarrhalrhinitisbecomespurulentwith
secondaryfissuringandexcoriationofnasal
vestibule,upperlip
–Nasalobstructioninterfereswithsucklingand
nutrition

18. Snuffles

19. Intraoral mucous patches and facial skin lesions
in congenital syphilis

20. •Atpuberty
–Gummatous&destructive
lesionsinthemucous
membrane,periosteum&
boneofnasalseptum
–Ulceration&destruction
leadstosecondary
atrophicrhinitisand
saddlenosedeformity

21. Tertiary syphilis
•Mostcommonlyinvolvesthe
nose
•Pathologicallesion:Gumma
•Beginsasasubcutaneous
nodule,progressestoinvolve
overlyingskinandbreaks
downtoformpunchedout
destructiveulcer

22. •Posteriorbonyseptalperforationandextensive
necrosisofnasalandfacialtissuesSaddlenose
deformity
•Earlysymptoms:
–Painoverthenasalbridge(worseatnight),swelling
andobstruction
–Offensivedischarge,bleeding,crusting,anosmia
(secondaryatrophicrhinitis)
•Severescarringofnose

23. Saddle nose deformity Hard palate perforation

24. Treatment
•Parenteralpenicillin:drugofchoiceforallstages
–BenzathinepenicillinG(2.4IUIM,singledose)
•Localtreatment
–Clearanceofcrustsandregularcleansingby
copiousalkalinedouches(1-3timesaday)
–Yellowmercuryoxideointmentappliedlocally
•Reconstructivesurgery

25. Rhinoscleroma (Respiratory scleroma)
•Progressivegranulomatousdiseasecommencinginnoseand
extendingintothenasopharynx,oropharynx,larynx,
sometimestotracheaandbronchicausedbyKlebsiella
rhinoscleromatis(Frischbacillus)

26. Stages
•Catarrhal:Foulsmellingpurulentrhinorrheapersistingfor
weekstomonths
•Atrophic:Foulsmellinglargenasalplaquesorcrusts
(simulatesatrophicrhinitis)
•Granulomatous(proliferative/nodular):
–Granulomatousnodulesenlargeandcoalesce(Tapirnose)
–Pathologicchangesmostcharacteristic
–K.rhinoscleromatismostfrequentlyisolated
•Cicatrizing:Adhesionsandstenosisdistortnormalanatomy
(Hebranose)

27. Tapir nose Hebra nose

28. Diagnosis
•Highindexofsuspicion:Coalescent,enlargedgranulomatous
nodulesatornearthenasalvestibule,diffuseandbilateral
•Microscopy:Silverimpregnationstains,Wartin-Starryor
Giemsastain
•Cultureofinfectedtissue:K.rhinoscleromatisin98%
•Complementfixationtest
•Biopsyshowingcharacteristichistologicalfeatures

29. Histopathology
•Mikuliczcells
–Scatteredlargefoamcellswitha
centralnucleusandvacuolated
cytoplasmcontainingbacilli
(transformedmacrophagesthat
haveingestedthebacillus)
•Russelbodies
–Resembleplasmacellswithan
eccentricnucleusanddeepeosin
stainingcytoplasm

30. Treatment
•Highdoseofbactericidalantibiotics
–Minimumof4-6weeks,continueduntil2consecutive
culturesfrombiopsymaterialareprovennegative
•Streptomycin(1g/day),Tetracycline(2g/day)
•Rifampicin,Sulphamethoxazole–trimethoprim,Ciprofloxacin
•Localapplicationof2%acriflavinfor8weeks
•Irradiation:3000-3500Gyover3weeks
•Surgicaldebridement
•Reconstructivesurgery

31. Rhinosporidiosis
•Chronicgranulomatousinfectionthataffectsthenasalmucosa
(70%),ocularconjunctivaandothermucosa
•Organism:Rhinosporidiumseeberi
•CommoninEasternteraibeltofNepal(Janakpur,Rajbiraj)
•Contactedbyimmersionincontaminatedwaterofponds
containinganimaldungs

32. Clinical Features
•Insidiousonsetofpainless,gradualnasalobstruction
•Nose
–Largesessileorpedunculatedlesions
–Leafy,papillomatousorproliferativelesionsstuddedwith
whitedots
–Pink,redorpurpleincolour
•Vascularandbleedontouch

34. •Histopathology(Diagnostic)
–Pseudoepitheliomatoussquamouscellmetaplasia
overliesnumerousmultisized,microscopicglobular
cystscalledsporangiawiththickwallandan
operculum
–Largesporangiafilledwithendospores
–Granulomatousreactionoffibroustissue,
neutrophils,plasmacells,andlymphocytes

35. Treatment
•Completesurgicalexcisionwithcauterizationofthebase
•Dapsone
–100mg/dayfor6months
–Arrestmaturationofsporangiaandaccentuated
granulomatousresponsewithfibrosis
•AmphotericinB
•Medicalmanagementisnotveryeffective

36. Wegener’s granulomatosis
(Rhinogenic granulomatosis)

37. •Triadof
–Granulomatousinflammationoftheupper
andlowerrespiratorytract
–Necrotizingvasculitisaffectingsmallto
mediumsizedvessels
–Necrotizingglomerulonephritis

38. •Etiology
–Inflammatory,hypersensitivityreactionwith
animmuneresponse
–Depositionofimmunecomplexleadsto
vasculitis

39. Types
•Type1:Limitedtonose
–Chronicnonspecificrhinitis,serosanguinous
nasaldischarge,nosepain,crusting,septal
perforation
•Type2:Moresystemicsymptoms
–Hemoptysis,cavityinchest
•Type3:Widelydisseminated
–Multipleorganfailure

40. •C/F
–Progressivemalaise,pyrexia,weightloss
–Noseandsinusesinvolvedin>80%
•Mucosaledema,Nasalobstruction,sinusitis
•Crusting
•Septalperforation
•Saddlenose
–Palatalulcers/perforation

41. Palatal changes

42. •Oralcavity:hyperplasticgranularlesionofgingiva,
ulcerativestomatitis
•Ocularsymptoms:conjunctivitis,episcleritis,corneal
ulceration,opticneuritis,retinalarteryocclusion,
proptosis(20%)
•Pulmonarysymptoms:cough,hemoptysis,pleuritic
pain
•Renalsymptoms(30-90%):hematuria

43. •Otologicalsymptoms:RecurrentAOM,OME
•Laryngealandtrachealsymptoms:unusual,most
commonlysubglottic&uppertrachea
•Centralnervoussystem:(10-15%)
•Others:ulcerationofskinindistalarms&legs,
polymyalgia,polyarthritis

44. Investigations
•Erythrocytesedimentationrate(ESR):Raised
•C-reactiveprotein(CRP) :+ve
•C–ANCA :positivein95%-60%iflocalizeddisease
•Serumangiotensinconvertingenzyme(SACE):Raised
•Bloodurea,Serumcreatinine
•UrineRE/ME
•ChestX-ray/CTchest:Nodularlesionswithcavitation
•Tissuebiopsy:typicalhistologicalfeatures

46. •CT/MRI:
–Non-specificmucosalthickeninginnoseorPNS
(86%)
–Evidenceofbonedestruction(75%)
–Newboneformationinwallsofsinus(50%)
–Orbitaffected(30%)
–MRIshowsfatsignalfromscleroticsinuswall
–Chestmayshowprogressivecavitationandfibrosis

47. •Histological features
–Fibrinoid vascular necrosis
–Granulomas are epithelial cell type (large, irregular
and lined with histiocytes)
–May show fibrinoid necrosis or be non-necrotic
–Multinucleated giant cells + eosinophils

48. Criteria for the diagnosis of Wegener's granulomatosis
Criteria Definition
Nasal or oral
inflammation
Painful or painless oral ulcers or purulent or
bloody nasal discharge
Abnormal chest x-ray
Presence of nodules, fixed infiltrates or cavities
Urinary sediment
Microhematuria (over 5 RBC /HPF) or red cell
casts in urine sediment
Granulomatous
inflammation on biopsy
Histology showing granulomatous inflammation
within wall of artery or in perior extravascular
area
Any 2 or more of four criteria

49. Treatment
•Prednisone (1 mg/kg per day) + Cyclophosphamide (2 mg/kg
per day) for 1 month
–Prednisolone tapered to alternate days for 2 months 
stopped after achievement of complete response
–Cyclophosphamide continued for 6 months to 1 year 
tapered over a few months
•Trimethoprim -Sulphamethoxazole, Azathioprine (200
mg/day)
•Methotrexate, Cyclosporin, Rituximab

50. Other treatment options
•Trimethoprim-sulphamethoxazole
•Azathioprine(200mg/day)
•Methotrexate
•Cyclosporin
•Rituximab