Ear

Relapsing Polychondritis

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Relapsing Polychondritis

  • See Also
  1. Auricular Perichondritis
  2. Otitis Externa
  • Epidemiology
  1. Incidence: Rare
  2. Age: Peaks age 40 to 50 years
  • Pathophysiology
  1. Not infectious
  2. Rare, multisystem, autoimmune, Collagen vascular disease
  3. Affects at least 2 cartilaginous sites
  • Findings
  • Symptoms and signs
  1. Characteristics: Ear
    1. Cartilaginous Auricle is primary site of ear involvement
      1. Pain and swelling
      2. Inflammation and local destruction
    2. Ear lobule is spared
      1. Ear lobule is the non-cartilaginous, lower External Ear
  2. Characteristics: Other cartilaginous sites involved
    1. Nasal chondritis (often accompanies ear involvement)
    2. Migrating nonerosive Oligoarthritis
    3. Other tissues involved may include skin, eye, renal and neurologic
  3. Characteristics: Serious other cartilaginous involvement (associated with increased morbidity and mortality)
    1. Tracheobronchial tree
    2. Cardiac connective tissue
  4. Associated findings
    1. Sensorineural Hearing Loss may be associated
    2. Vertigo in 20% of cases
  5. Course
    1. Recurrent self-limited flares
  • Associated Conditions
  1. Other autoimmune disorders
  • Differential Diagnosis
  1. Otitis Externa
  2. Ramsay Hunt Syndrome (Herpes Zoster Oticus)
  3. Auricular Perichondritis
  4. Squamous Cell Carcinoma of the Skin
  5. Granulomatosis with Polyangiitis
  6. Chondrodermatitis nodularis helicis
    1. Auricular helix with painful, swollen Nodule (which later ulcerates)
    2. Benign, skin and cartilage inflammation of the auricular helix
    3. Typically localizes to the patient's preferred sleeping side (ear down)
  • Diagnosis
  1. Biopsy
  • Management
  1. Systemic Corticosteroids (and other Immunotherapy)
  2. Refer to Otolaryngology (ENT) or Rheumatology
  • References
  1. Duran (2024) Am Fam Physician 110(5): 529-30 [PubMed]