On a recent wintry night at a walk-in clinic in Philadelphia, a mother came in with her young teenage daughter who complained that her ears hurt. She had pain and redness on the outside of her ears that would come and go. Initial examinations tested for conditions that would cause her ears to become red:

  • Ear bud dermatitis – caused by allergens or headphone plastic, it causes redness and itching
  • Reynaud’s disease – caused by cold weather, it causes blood vessels to narrow in the fingers, toes, and ears
  • Erythromelalgia – caused by increased body temperature, it causes redness and pain in the hands, feet, and ears
  • Eczema – a condition where skin patches are rough and inflamed, with blisters that cause itching and bleeding
  • Sunburn

Tests proved negative. Ultimately, however, blood work indicated a condition known as relapsing polychondritis, or RP.
 

What Is Relapsing Polychondritis (RP)?

Relapsing polychondritis (RP) is a rare autoimmune rheumatic disorder that only affects 3-4 people per million annually and, therefore, is easy to overlook or misdiagnose. It’s characterized by intermittent pain and inflammation of the bodies’ cartilage and other connective organ tissues. RP is also referred to as red ear syndrome which is characterized by redness and inflammation of the cartilage at the tips of the ears, sparing the ear canals and lobes.

As the disease progresses, inflammation and damage to cartilage in other areas of the body can occur, including the nose, joints, inner ear, and more fatally, the windpipe (trachea). Life-threatening inflammation from RP may also affect the aorta (the largest artery in the body), the heart, and the nerves within the brain.
 

Can Doctor’s Test for RP?

Since there is no specific test for RP, doctors must rely on more general blood work to indicate inflammation throughout the body or a biopsy of affected cartilage to confirm the presence of inflammation and the absence of other causes, such as infection.
 

Treatment Options for RP

Anti-inflammatory medications are the preferred treatment option for relapsing polychondritis. If severe breathing or heart complications arise, however, surgery may be recommended. The prognosis can be variable from patient to patient, depending on which tissues are affected, how aggressive the disease is, and whether the inflammation responds rapidly to treatment.

 

References
The Intuition in Pregnancy Study. http://vas.web.arizona.edu/intuit.htm
Medical mystery: a girls’ red ears, a mother’s intuition. https://www.inquirer.com/health/medical-mystery-relapsing-polychondritis-20190913.html
Relapsing polychondritis. https://www.medicinenet.com/relapsing_polychondritis/article.htm#what_is_the_long-term_prognosis_for_patients_with_relapsing_polychondritis
A is for aphorism. https://www.racgp.org.au/afp/2012/september/a-is-for-aphorism/
Relapsing polychondritis. https://rarediseases.info.nih.gov/diseases/7417/relapsing-polychondritis
Polychondritis. https://www.health.harvard.edu/a_to_z/polychondritis-a-to-z