“Raccoon Eyes” and a Large Tongue — Could It Be AL Amyloidosis?

AL Amyloidosis is a rare disease that occurs when certain blood cells produce abnormal proteins called amyloid.  The amyloid proteins are deposited into bodily organs, interfering with their ability to function properly.  Various organs can be affected, including (but not limited to) the heart, kidneys, and digestive tract.  

Symptoms of AL Amyloidosis may be different in each person, depending on which organs are impacted.  For instance, patients with amyloid deposits in their hearts may experience shortness of breath and fatigue, while patients with more amyloid build up in their digestive tracts may experience weight loss and diarrhea.  

But did you know that AL Amyloidosis can cause changes to the skin and tongue too?  Approximately 10% of AL Amyloidosis patients experience purpura, which happens when small blood vessels leak blood under the skin’s surface causing dark patches or bruising.  This often occurs around their eyes, giving them a “raccoon-like” appearance.  Depending on their skin color, the spots may appear reddish-purple (on lighter complexions) or brownish-black (on darker complexions).  Additionally, about 15% of patients experience amyloid build up in their tongues.  This causes their tongues to become enlarged, which can make it difficult to eat, breathe, and talk.

AL Amyloidosis can present itself in many ways.  From gasping for air to raccoon eyes to a swollen tongue — symptoms are wide-ranging and can vary from person to person.  Knowing what physical signs to look out for may help patients and their healthcare providers better recognize the disease and avoid a misdiagnosis.

References:

1. AL Amyloidosis. Amyloidosis Foundation. 2022. http://amyloidosis.org/facts/al/#symptoms 
2. Amyloidosis. Mayo Clinic. 2020. https://www.mayoclinic.org/diseases-conditions/amyloidosis/symptoms-causes/syc-20353178
3. Merlini et al.  Amyloidosis: Pathogenesis and New Therapeutic Treatment Options.  J Clin Oncol. 2011; 29:1924-1933
4. Mantha et al. Systemic Light Chain Amyloidosis with Mucocutaneous and Cardiac Manifestations in a Black Woman: A Case Highlighting Healthcare Disparities in Patients of Color. J Am Coll Cardiol. 2021; 17(18 Suppl 1):2181
5. Matsuura et al. Raccoon Eye Appearance: Amyloidosis. Am J Med. 2018; 131(7):E305
6. Purpura. Cleveland Clinic. 2022. https://my.clevelandclinic.org/health/symptoms/22695-purpura 
7. Macroglossia. Cleveland Clinic. 2022. https://my.clevelandclinic.org/health/diseases/22544-macroglossia