Describe the granulomatous diseases of the nose.

Approach and the Infective Granulomas

• Granulomatous diseases OF the nose are conditions producing chronic granulomatous inflammation of the nasal mucosa, with crusting, nasal obstruction, discharge and tissue destruction.
• They are important in India, where the infective causes remain common.
• The presentation IS remarkably similar whatever the cause.

The Non-infective Granulomas and Mimics

• Granulomatosis with polyangiitis (formerly wegener) is the most important non-infective cause, and the nose is frequently where it first declares itself
• It is a necrotising vasculitis with granulomas, affecting the upper airway, lungs and kidneys — the classical triad
• Nasal features — persistent crusting and blood-stained discharge that fails to respond to treatment; septal perforation and saddle deformity; obstruction; and friable, granular mucosa that bleeds on touch
• Systemic features to seek — haemoptysis and pulmonary infiltrates or cavities; glomerulonephritis with haematuria and a rising creatinine.
• Diagnosis — c-ANCA with PR3 specificity.
• Treatment is immunosuppression — corticosteroid with cyclophosphamide or rituximab for induction, then maintenance with azathioprine or methotrexate; with co-trimoxazole.
• Nasal care — douching and crust removal, and surgery only when the disease is quiescent.
• Sarcoidosis — non-caseating granulomas; nasal crusting and obstruction with submucosal nodules giving a "strawberry skin" appearance on the inferior turbinate; with lupus pernio, a violaceous plaque on the nose and cheeks. Raised serum ACE and calcium, hilar lymphadenopathy on chest imaging; treated with steroid
• NK/T-cell lymphoma, nasal type (formerly "lethal midline granuloma") — an EBV-associated malignancy causing relentless midfacial destruction. It is commoner in Asian and South American populations, and must not be treated as an infection. Diagnosed by repeated deep biopsy with immunohistochemistry (CD56 positive) and EBV testing; treated with radiotherapy and chemotherapy
• Rhinoscleroma and rhinosporidiosis are considered separately, but both belong in this differential
• The mimics that must not be forgotten — cocaine and inhaled drug abuse.
• Ask about cocaine use directly and non-judgementally in any midfacial destructive lesion; patients rarely volunteer it and the diagnosis is otherwise repeatedly missed
• They all look the same, so biopsy decides; appearance alone cannot separate infection, vasculitis, lymphoma and carcinoma
• Take a deep and generous biopsy; superficial specimens show only non-specific inflammation and waste the procedure
• Repeat a negative biopsy where suspicion remains, particularly in vasculitis and lymphoma
• Send for fungal and mycobacterial culture as well as histology, or the treatable infections will be missed
• Tuberculosis and leprosy perforate cartilage, whereas syphilis destroys the bony septum, which is a useful discriminator
• The nose is an early site in lepromatous leprosy and a major route of transmission.
• Nasal tuberculosis is secondary to pulmonary disease, so the chest must be imaged in every case
• Check urinalysis and creatinine in every suspected vasculitis; the kidney determines the prognosis and is silent early
• C-ANCA may be negative in limited disease, so a negative result does not exclude granulomatosis with polyangiitis
• Operate only when vasculitis is quiescent; surgery on active disease fails and may worsen the destruction
• Co-trimoxazole reduces relapse in limited upper airway disease, and is a cheap addition to immunosuppression
• Strawberry skin on the turbinate suggests sarcoidosis, with lupus pernio on the nose and cheeks
• Relentless midfacial destruction suggests lymphoma, which must not be treated as an infection while it advances
• NK/T-cell lymphoma is EBV-associated and commoner in Asian populations, and needs immunohistochemistry to diagnose
• Ask about cocaine directly and without judgement; it mimics vasculitis exactly and may even give a positive ANCA
• Chronic steroid spray misuse perforates the septum, and is a mimic that is corrected simply by changing the technique
• Carcinoma is the reason biopsy is mandatory, since it may present as a crusting non-healing lesion like any of these
• Mucormycosis is an angioinvasive emergency, and a black eschar in a diabetic must be acted on the same day
• Lupus vulgaris shows apple-jelly nodules on diascopy, and is the indolent cutaneous form of tuberculosis