Granulomatous conditions of nose 20 marks answer for ms ent exam

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Granulomatous Conditions of the Nose

(20 Marks - MS ENT Exam Answer)

Introduction

A granuloma is a focal collection of epithelioid histiocytes, often with Langhans or foreign-body type giant cells, lymphocytes, and plasma cells. The nose and paranasal sinuses are common sites for both infectious and non-infectious granulomatous diseases. These conditions share a tendency to cause nasal obstruction, crusting, epistaxis, and eventual structural destruction.
Classification:
CategoryConditions
Infectious - BacterialRhinoscleroma, Leprosy (Hansen's disease), Tuberculosis, Syphilis, Actinomycosis
Infectious - FungalRhinosporidiosis, Histoplasmosis, Blastomycosis
Autoimmune/VasculiticGranulomatosis with Polyangiitis (GPA/Wegener's), Sarcoidosis
Foreign bodyCholesterol granuloma

1. RHINOSCLEROMA

Etiology: Caused by Klebsiella rhinoscleromatis, a gram-negative bacillus.
Epidemiology: Endemic to Central and South America, Central Africa, India, Southeast Asia, Middle East, Eastern Europe. Contracted by inhalation of droplets; crowded/poor hygienic conditions. Female predominance (13:1 in some series); 2nd-3rd decade.
Sites: Nasal cavity (most common); nasopharynx, larynx, trachea, paranasal sinuses. In the nasal cavity, the middle and inferior turbinates are most frequently affected. Begins at areas of epithelial transition (nasal vestibule).
Three Stages:
StageFeatures
1. Catarrhal (Rhinitic)Persistent purulent rhinorrhea, nasal obstruction, honeycomb-colored crusting; lasts weeks to months
2. Granulomatous (Florid/Hypertrophic)Painless granulomatous nodules in nose and upper airway; septal destruction, thickened soft palate; epistaxis
3. Sclerotic (Cicatricial)Dense fibrotic scarring; stenosis of nasal cavity, larynx or tracheobronchial tree; anosmia, dysphonia, stridor
Pathology (Key Features):
  • Mikulicz cells - large vacuolated macrophages with clear cytoplasm containing bacilli
  • Russell bodies - bloated plasma cells with birefringent eosinophilic inclusions (dilated endoplasmic reticulum with aggregated immunoglobulin)
  • Pseudoepitheliomatous hyperplasia of the overlying epithelium
Diagnosis: Tissue culture on MacConkey agar (positive in only 50-60%); histopathology from biopsy (gold standard). Clinical diagnosis suspected from honeycomb crusting.
Treatment:
  • Long-term antibiotics: ciprofloxacin (drug of choice), tetracycline, streptomycin
  • Nasal debridement for crusting and obstruction
  • Dilatation for stenosis; tracheostomy or laser excision for severe laryngeal involvement
  • Reconstructive surgery for nasal deformity after disease control
(Cummings Otolaryngology, p. 1008; KJ Lee's Essential Otolaryngology, p. 581)

2. LEPROSY (Hansen's Disease)

Etiology: Mycobacterium leprae - obligate intracellular, acid-fast bacillus; thermolabile (grows optimally at 27-33°C, hence nasal predilection).
Epidemiology: Rare in developed countries. The nose is the most frequently affected site in the head and neck.
Clinical Features:
  • Epistaxis, crusting, nasal obstruction, anosmia
  • Initial stage: friable, granulomatous intranasal lesions on endoscopy
  • Progressive: septal perforation → nasal deformity
  • Atrophy of the nasal spine and premaxillary alveolar process
  • Oroantral fistula in advanced cases
Diagnosis: Clinical exam, biopsy and culture of lesion, slit-skin smear.
Treatment: Multidrug antibiotic therapy (dapsone + rifampicin ± clofazimine per WHO regimen).
(Cummings Otolaryngology, p. 1008)

3. NASAL TUBERCULOSIS

Etiology: Mycobacterium tuberculosis; nasal involvement usually secondary to pulmonary TB. Risk groups: immunocompromised, healthcare workers, immigrants.
Site: Most commonly involves the cartilaginous septum.
Clinical Features:
  • Crusting, epistaxis, nasal obstruction
  • Septal perforation, nodular thickening of nasal mucosa
  • Lupus vulgaris (painful nodular tuberculoid lesion)
  • Purulent rhinorrhea, nasal fissures
  • Saddle nose deformity with progression
Diagnosis:
  • Histopathology: caseating granulomas with Langhans giant cells + acid-fast bacilli on ZN stain
  • Mantoux/PPD skin test or interferon-gamma release assay (IGRA)
  • HIV testing mandatory
Treatment: Prolonged multidrug antitubercular therapy (isoniazid, rifampicin, pyrazinamide, ethambutol).
(Cummings Otolaryngology, p. 1007)

4. RHINOSPORIDIOSIS

Etiology: Rhinosporidium seeberi (now classified as Mesomycetozoa, not a true fungus). Endemic to India, Sri Lanka, Africa, Pakistan.
Transmission: Transepithelial inoculation through exposure of spores in contaminated stagnant water (public bathing, wading in ponds).
Clinical Features:
  • Nasal cavity and external eye (palpebral conjunctivae) are most common sites
  • Symptoms: nasal obstruction, epistaxis, rhinorrhea, foreign body sensation
  • Examination: friable, polypoid, exophytic mass with submucosal budding; erythematous surface studded with white spores - classic "strawberry" appearance
  • Progressive lesion may fill the entire nasal cavity
Histopathology: Sporangia (thick-walled cysts containing endospores) within the submucosal tissue; pseudoepitheliomatous hyperplasia.
Treatment: Surgical excision (wide base excision to prevent recurrence) + oral dapsone therapy.
(Cummings Otolaryngology, p. 1008; KJ Lee's Essential Otolaryngology, p. 581)

5. SYPHILIS

Etiology: Treponema pallidum.
Nasal Manifestations by Stage:
  • Primary: Chancre (hard, painless ulcer) if the nose is the site of inoculation
  • Secondary: Mucous patches, condylomata
  • Tertiary: Gumma formation - a granulomatous mass that can cause nasal septal perforation and saddle nose deformity; bony destruction
Diagnosis: FTA-ABS (fluorescent treponemal antibody absorption test); VDRL/RPR for screening; dark-field microscopy or immunofluorescence staining for spirochetes.
Treatment: High-dose Penicillin G (still effective). Note increasing incidence in the HIV era.
(Cummings Otolaryngology, p. 1008)

6. GRANULOMATOSIS WITH POLYANGIITIS (GPA / Wegener's Granulomatosis)

Etiology: ANCA-associated necrotizing vasculitis involving small and medium vessels.
Classic Triad:
  1. Upper respiratory tract (nose, sinuses, larynx)
  2. Lower respiratory tract (lungs)
  3. Kidneys (necrotizing glomerulonephritis)
Nasal Features (>90% of patients):
  • Crusting, bleeding, obstruction
  • Cartilaginous inflammation → nasal septal perforation
  • Collapse of nasal bridge → "saddle nose" deformity (a hallmark)
  • Erosive sinus disease
  • Subglottic stenosis
Systemic Features: Pulmonary infiltrates/nodules, haematuria/proteinuria (renal involvement), purpuric skin lesions.
Investigations:
  • c-ANCA (anti-PR3) - positive in ~90% of active generalized disease; highly specific
  • Biopsy: necrotizing granulomatous inflammation with vasculitis
  • Urinalysis, renal function, chest X-ray/CT
Treatment:
  • Induction: rituximab or cyclophosphamide + glucocorticoids
  • Maintenance: azathioprine, methotrexate, or rituximab
  • Nasal management: irrigations, nasal corticosteroids; surgery for selected complications
(Goldman-Cecil Medicine; KJ Lee's Essential Otolaryngology)

7. SARCOIDOSIS

Etiology: Unknown; non-infectious systemic granulomatous disease. Affects Black Americans disproportionately; peak incidence 20-40 years.
Nasal Features:
  • Submucosal non-caseating granulomas in the nasal mucosa
  • Symptoms: nasal obstruction, crusting, epistaxis, anosmia
  • Lupus pernio - distinctive skin lesion: indurated violaceous plaques/nodules on the nose, nasal alae, cheeks, lips and ears; often involves nasal mucosa; associated with chronic systemic sarcoidosis
  • Nasal polyps; rarely, cartilaginous destruction causing "saddle nose" (less common than in GPA)
Histopathology: Non-caseating ("naked") epithelioid granulomas with Langhans giant cells; no central necrosis.
Systemic Associations: Bilateral hilar lymphadenopathy, pulmonary fibrosis, uveitis, hypercalcaemia, erythema nodosum (non-granulomatous).
Investigations:
  • Elevated serum ACE (angiotensin-converting enzyme)
  • Chest X-ray/HRCT
  • Biopsy for confirmation
  • Serum calcium, 24-hour urine calcium
Treatment: Systemic steroids (mainstay); immunosuppressants including anti-TNF agents (infliximab) for refractory cases. Nasal: irrigations, nasal steroids; surgery in select cases.
(KJ Lee's Essential Otolaryngology, p. 581; Fishman's Pulmonary Diseases)

8. FUNGAL GRANULOMAS

Histoplasmosis

  • Histoplasma capsulatum; endemic to Mississippi/Ohio/Missouri River valleys.
  • Inhalation of spores from soil; nasal involvement presents as nodules or ulcers.
  • Diagnosis: biopsy showing granuloma; PAS or GMS fungal stains.
  • Treatment: IV amphotericin B for systemic disease, then oral itraconazole.

Blastomycosis

  • Blastomyces dermatitidis; thermally dimorphic fungus; nasal cavity involved in ~9% of extrapulmonary cases.
  • KOH prep or PAS/GMS stain; culture (takes 2-4 weeks).
  • Treatment: itraconazole (mild-moderate); amphotericin B induction followed by itraconazole (severe).
(Cummings Otolaryngology, p. 1009)

9. CHOLESTEROL GRANULOMA

Pathogenesis: Hypoventilation of air cells (e.g. from nasal obstruction/CRS) → hemosiderin deposition in enclosed space → cholesterol crystal formation → macrophage-mediated granulomatous reaction → expansile mass with bony erosion.
  • Most common in frontal and maxillary sinuses.
  • Symptoms: headache, nasal obstruction, diplopia, proptosis.
  • Imaging: CT shows expansile cystic lesion with bony erosion; MRI shows hyperintense on both T1 and T2 (due to cholesterol/blood products - a characteristic finding).
  • Treatment: marsupialization or complete resection (endoscopic or external approach).
(Cummings Otolaryngology, p. 1007)

Summary Table

ConditionCausative AgentKey HistologySpecific Treatment
RhinoscleromaKlebsiella rhinoscleromatisMikulicz cells, Russell bodiesCiprofloxacin (long-term)
LeprosyM. lepraeLepra cells (macrophages with bacilli)MDT (dapsone + rifampicin)
TBM. tuberculosisCaseating granuloma, AFBAnti-TB drugs (HRZE)
RhinosporidiosisRhinosporidium seeberiSporangia with endosporesExcision + Dapsone
SyphilisTreponema pallidumGumma (tertiary)High-dose Penicillin G
GPA/Wegener'sANCA vasculitisNecrotizing granuloma + vasculitisRituximab/cyclophosphamide
SarcoidosisUnknownNon-caseating granulomaSystemic steroids
HistoplasmosisH. capsulatumGranuloma with yeast formsItraconazole/Amphotericin B
BlastomycosisB. dermatitidisSuppurative granuloma, broad-based budding yeastItraconazole
Cholesterol granulomaNon-infectiousCholesterol clefts, giant cells, hemosiderinEndoscopic marsupialization

Examination-Ready Key Points

  • Rhinoscleroma: honeycomb crusting + Mikulicz cells + Russell bodies = pathognomonic triad; 3 stages (catarrhal, granulomatous, cicatricial).
  • Rhinosporidiosis: "strawberry" polyp; sporangia on biopsy; treatment = excision + dapsone.
  • GPA (Wegener's): saddle nose + c-ANCA + renal + pulmonary triad; septal perforation is the nasal hallmark.
  • Sarcoidosis: lupus pernio on nose; non-caseating granuloma; elevated ACE; treat with steroids.
  • Cholesterol granuloma: T1 and T2 bright on MRI; endoscopic marsupialization.
  • TB: caseating granuloma + AFB; septal cartilage predilection; saddle nose possible.
  • Syphilis (tertiary): gumma = granulomatous; treat with Penicillin G.

Sources: Cummings Otolaryngology Head and Neck Surgery (6th ed.); KJ Lee's Essential Otolaryngology (11th ed.); Goldman-Cecil Medicine; Fishman's Pulmonary Diseases
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