A 25-year-old presents with a saddle nose deformity, septal perforation, and positive c-ANCA (PR3-ANCA). Nasal biopsy shows necrotizing granulomatous vasculitis. What is the diagnosis and the MOST specific histological finding?

• A Sarcoidosis; non-caseating granulomas without vasculitis
• B Relapsing polychondritis; chondrolysis and cartilage inflammation
• C NK/T-cell lymphoma (lethal midline granuloma); atypical lymphoid infiltrate
• D Granulomatosis with polyangiitis (Wegener's); necrotizing granulomatous vasculitis of small vessels ✓

Explanation

Granulomatosis with polyangiitis (formerly Wegener's) presents with upper respiratory tract (saddle nose, septal perforation, epistaxis), lower respiratory, and renal involvement. c-ANCA (anti-PR3) is 95% specific. Histology shows necrotizing granulomatous inflammation with vasculitis of small vessels (classically described as 'geographic necrosis' with palisading histiocytes and vasculitis). Sarcoidosis has non-caseating granulomas without vasculitis; NK/T-cell lymphoma shows CD56+ atypical lymphocytes with angiodestruction.

Reference: Dhingra Diseases of Ear, Nose and Throat, 7th ed.

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Written and medically reviewed by the StethoPrep medical team.