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Key Features

  • Inflammatory destructive lesions of cartilaginous structures, principally the ears, nose, trachea, larynx, and chest wall cartilage

  • Associated conditions include

    • Systemic lupus erythematosus

    • Rheumatoid arthritis

    • Hashimoto thyroiditis

    • Cancer (especially plasma cell myeloma)

    • Hematologic disorders (myelodysplastic syndrome)

Clinical Findings

  • The cartilage is painful, swollen, and tender during an attack

  • Subsequently becomes atrophic, resulting in permanent deformity

  • Noncartilaginous manifestations

    • Fever

    • Episcleritis, uveitis

    • Deafness

    • Aortic regurgitation

    • Glomerulonephritis (rare)

  • In 85% of patients, a migratory, asymmetric, and seronegative arthropathy occurs, affecting both large and small joints and the costochondral junctions

  • Laryngotracheal and bronchial chondritis can lead to life-threatening airway narrowing and collapse

Diagnosis

  • Clinical

  • Differential diagnosis

    • Other causes of ear deformity (skin cancer, cauliflower ear [cartilage dissolution posttrauma], chondritis or perichondritis, chondrodermatitis nodularis helicis)

    • Other causes of nose deformity (syphilis, rhinophyma [rosacea], Hansen disease [leprosy], granulomatosis with polyangiitis [formerly Wegener granulomatosis])

Treatment

  • Prednisone, 0.5–1.0 mg/kg/day orally, is often effective

  • Dapsone, 100–200 mg/day orally, or methotrexate (7.5–20 mg/week orally) may have efficacy, sparing the need for long-term high-dose corticosteroid treatment

  • Involvement of the tracheobronchial tree may respond to TNF inhibitors

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