Rheumatology – Lesson 6

A 55-year-old woman was referred by the local oncologist for evaluation of joint pain. The patient was diagnosed with Lymphoma before and is currently in remission after her treatment. She reports that in addition to her joint pain, she has developed recurrent swelling and redness of her ears as well. She saw a dermatologist who felt she may have dermatitis. On the review system, she does admit to some hearing loss, recurrent eye irritation, as well as a chronic cough with dyspnea upon exertion.

On examination, she was noted to have tenderness and swelling of the nasal bridge. Her PFT’s showed fixed airflow limitation with both inspiration and expiration. A picture of her ears are shown below:

What is the most likely diagnosis here?

  1. Recurrence of Lymphoma
  2. Atopic dermatitis
  3. Relapsing Polychondritis
  4. Cogan Syndrome

The correct answer is Relapsing Polychondritis or RPC. This is a very rare disease but it is seen 1-2 times per year in a busy rheumatology practice. Hallmark of this disease is the painful ear swelling and swelling of the nasal cartilage.

Relapsing Polychondritis is characterized by:

Ear inflammation

Swollen joints

  1. Recurrence of Lymphoma. This would be very unusual. Ears are usually not the site of recurrence for lymphoma.
  2. Atopic dermatitis. This can cause a diagnostic dilemma at times. With atopic dermatitis, the whole ear is swollen as shown here. Obviously, with RPC the cartilage swelling and inflammation spares the lobule, the lower part of the auricle is the only area of the outer ear that contains no cartilage and thus is not involved in Relapsing Polychondritis
  3. Cogan Syndrome: This may manifest as recurrent dizziness, fatigue, fever, weight loss, uveitis, corneal inflammation, episcleritisVitritis, choroiditis, scleritis, or Papillitis. Vasculitis including aortitis can also occur. Although these patients can develop inner ear problems with sensorineural hearing loss, tinnitus, and vertigo, there is no actual outer ear swelling in these patients.