Chapter 12: Granulomatous Diseases of the Head and Neck

Overview

• What is a granuloma?

  1. A granuloma is created by a chronic immunologic process resulting clinically in localized nodular inflammation.

• Histology

  1. The inflammatory response is mediated by monocytes and macrophages. Macrophages can give rise to epithelioid cells, which can secrete extracellular enzymes. Multinucleated giant cells are often found in granulomas and are thought to arise from the fusion of macrophages.

  2. These cells are surrounded by lymphocytes and eosinophils.

  3. A granuloma is complete with a fibroblastic proliferation occurring around the cells.

• What is the differential diagnosis of a granuloma in the head and neck?

  1. There is a significant range of symptoms caused by granulomatous diseases, not limited to the head and neck. A full history and physical examination should be performed for all patients with a granulomatous biopsy. Refer to Table 12-1.

Differential Diagnosis

See Table 12-1.

Autoimmune

Granulomatosis With Polyangiitis (GPA, Renamed From Wegener Granulomatosis)

• Small and medium-vessel vasculitis.

• Belongs to a larger group of necrotizing vasculopathy syndromes, all of which involve an autoimmune attack by abnormal circulating anti-neutrophil cytoplasmic antibodies (ANCA).

• Thought to be a T-cell mediated hypersensitivity reaction.

• Initially named after Friedrich Wegener, a German pathologist whose Nazi past was uncovered, leading to the eponym being abandoned and renamed in 2011.

Incidence/Epidemiology

• Cause unknown.

• 10 to 20 cases per million per year.

• 5-year survival is over 80% with adequate treatment.

• Commonly occurs in whites, in third to fifth decades.

Presentation

• Extremely variable.

• Classic triad: airway necrotizing granulomas, systemic vasculitis, and focal glomerulonephritis.

• Most common presenting symptom is rhinitis.

• Renal: rapidly progressive glomerulonephritis (75%).

• Sinonasal: nasal congestion, crusting, rhinitis, epistaxis, septal perforation, and possible saddle nose deformity.

• Otologic: conductive hearing loss from Eustachian tube dysfunction, middle ear effusion, possible sensorineural hearing loss.

• Oral cavity: gingival hypertrophy, gingivitis, tooth decay, nonspecific ulcerations.

• Ocular: pseudotumors, conjunctivitis, scleritis, episcleritis, uveitis, nasolacrimal obstruction.

• Larynx: subglottic stenosis.

• Pulmonary: cavitary lesions, nodules, infiltrates, pulmonary hemorrhage.

Histology

• Characteristic patchy necrosis surrounded by giant cells, causing necrotizing granuloma formation in nonspecific inflammatory background

• Macrophages, inflammatory cells, and giant cells

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