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