Necrotizing lesions of the nose and upper airway
- Lesion
Three different diseases can destroy the nose and palate: an invasive fungus, an ANCA vasculitis, and an EBV-driven lymphoma.
What it is
Ulcerating, necrotising lesions of the nasal cavity and midface have three main causes, which the biopsy must separate: invasive fungal infection (especially Mucorales), granulomatosis with polyangiitis, and extranodal NK/T-cell lymphoma, nasal type. All can perforate the septum or palate; treatment is completely different for each.
Structure
- Mucormycosis: infarcted, necrotic tissue with hyphae in vessel walls and thrombi.
- GPA: geographic necrosis with basophilic debris, scattered giant cells, poorly formed granulomas and necrotising vasculitis.
- NK/T-cell lymphoma: atypical lymphoid cells around and inside vessel walls with extensive necrosis; CD56, cytotoxic granules, EBER positive.
- Biopsies are often mostly necrotic; repeat or deep biopsies may be needed.
Step by step
- Mucorales spores germinate in the sinuses when neutrophils and acidosis fail to stop them; hyphae invade arteries and cause infarction.
- In GPA, ANCA activate primed neutrophils that injure vessel walls and provoke granulomas.
- In NK/T-cell lymphoma, EBV-infected NK or T cells proliferate around vessels and destroy them.
- All three lead to ischaemic necrosis of mucosa, cartilage and bone.
Destructive midline lesions
| Disease | Cause | Key biopsy finding | Test |
|---|---|---|---|
| Mucormycosis | Mucorales fungi | Broad non-septate hyphae in vessels | Silver stain, culture |
| Granulomatosis with polyangiitis | ANCA vasculitis | Geographic necrosis, granulomas, vasculitis | PR3-ANCA |
| NK/T-cell lymphoma, nasal type | EBV-driven lymphoma | Angiocentric atypical cells, necrosis | CD56, EBER |
Markers
- PR3-ANCA
- EBER
- CD56
- Grocott methenamine silver
Clinical notes
- A diabetic with facial pain, black nasal eschar and cranial nerve palsies needs emergency treatment for mucormycosis.
- Chronic sinusitis, epistaxis and nasal crusting with hemoptysis or kidney disease suggest GPA.
- Nasal-type NK/T-cell lymphoma is aggressive and fatal without treatment; localised disease responds to radiotherapy.
Test yourself
Think of the answer, then tap the question to see it.
What are the three main causes of necrotising lesions of the nose?
Invasive fungal infection (mucormycosis), granulomatosis with polyangiitis and extranodal NK/T-cell lymphoma, nasal type.
Which virus is associated with extranodal NK/T-cell lymphoma, nasal type?
Epstein–Barr virus, shown in tumour cells by EBER in situ hybridisation.
What antibody is typical of granulomatosis with polyangiitis?
PR3-ANCA (c-ANCA).
Connections
Microorganisms2
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Sinusitis (MedlinePlus, US National Library of Medicine, 2025).
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Representative model, for educational purposes only.