As a dental hygienist of 19 years, I thought I had seen it all. But recently I had one of those rare moments that left me wide-eyed and mouthing "WTH" behind my mask. This is how I became familiarized with localized amyloidosis.
During a routine appointment with a new female patient, I did what I always do: extensively reviewed the patient's medical history, discussed her current oral hygiene habits, and addressed any concerns. Next, I performed my regular oral cancer screening by asking permission to lean the patient back and touch the outside of her face and neck.
After the extra-oral exam, I began my intra-oral screening, feeling around the cheeks and mouth floor, pulling the tongue out, and examining the sides for any abnormalities. Then, as I was assessing the patient's airways by checking her throat and Mallampati score, my eyes stopped dead in their tracks.
Something was off. Something unfamiliar.
As a professional, I maintained my composure and gently said, "I'm noticing something in the back of your throat…"
Before I could even finish, she casually said, "Oh yeah, that's my amyloidosis."
Come again? What word did you just throw at me? I had literally just reviewed this patient's medical history and saw nothing that mentioned amyloidosis. My mind immediately went to scenes from "House," where they throw around all these rare conditions-but this one? It was new to me.
I completed the rest of my oral assessment, probing, and hygiene diagnosis. After removing my gloves, I sat the patient up and asked a few more questions while Googling this "amyloidosis" (after asking her to spell it for me, of course). I was expecting some rare yet harmless ailment with a hard-to-pronounce name, but what she said next made my heart skip a beat: "It's localized amyloidosis. If it were systemic, I'd probably be dead."
Wait, what?
This was one of those moments where I had to keep my face looking neutral, but inside I was spinning. How could this condition be fatal? What exactly was this lesion I was seeing? She then pulled out her phone and showed me a video of her nasopharyngoscopy from her ENT, which clearly showed the lesions localized in her throat and oropharyngeal area. Needless to say, I took a deep dive later that night into PubMed and picked the brains of my smartest friends about it.
Here's what I learned.
Systemic vs. Localized Amyloidosis
Amyloidosis is a rare metabolic disorder1 in which abnormal protein deposits-known as amyloids-build up in tissues and organs. These deposits can interfere with normal tissue function. The scary part? If this happens on a systemic level (meaning in multiple organs), it can be life-threatening, affecting the heart, kidneys, liver, and nervous system. There are three main types2 of systemic amyloidosis, which are equally serious, and the prognosis often isn't great.
On the other hand, localized amyloidosis is more contained. It only affects one specific area of the body, such as the skin, bladder, or, in my patient's case, the oropharyngeal area. While localized amyloidosis is much less dangerous than its systemic counterpart, it still needs to be monitored closely. If left untreated, it can cause functional issues, depending on where the deposits are located, especially in vital areas such as the mouth or throat.3
Oral Manifestations of Amyloidosis
Now, what threw me for a loop was that amyloidosis can actually present in the oral cavity-something that had never been on my radar. Oral amyloidosis4 is a rare manifestation, but when it happens it can affect several parts of the mouth, including the tongue, lips, mouth floor, gingiva, buccal mucosa, and even the submandibular and parotid gland regions.
One of the most common signs is macroglossia5 (an enlarged tongue). In fact, up to 40% of systemic amyloidosis cases present with this condition, making it crucial for us hygienists to be informed of this potential cause. The tongue can appear swollen, with indentations from the teeth along the sides, and in severe cases it can lead to altered taste and difficulty speaking, chewing, and swallowing. It's worth mentioning that there are rare cases of localized amyloidosis of the tongue6 that mainly involve painless lesions.
Such lesions can also appear on the palate,7 tonsils, uvula, and even around the mandibular condyle, and they might look like firm, raised nodules or plaques. In the case of localized amyloidosis, these manifestations are usually isolated, as with my patient.
Interestingly, amyloid deposits can also affect salivary gland function, potentially leading to dry mouth or swelling in the affected glands. The submandibular and parotid glands are particularly vulnerable, making this condition relevant for oral health professionals like us who monitor these areas. Depending on the affected tissue, other symptoms might include hoarseness, airway obstruction, and jaw claudication.
What Will I Do Differently in the Future?
Honestly, not much will change in my approach. However, one thing this experience highlighted is how, even after years of experience, there are always opportunities to learn something new. Now that I know about oral amyloidosis, I'll be much better equipped to recognize it in the future and skip that initial moment of panic when I see similar lesions. Knowledge is power, and we only gain it by familiarizing ourselves with things that fly under our radar.
In terms of the medical history, it served as a good reminder that sometimes important details can be missed. I'll continue encouraging my patients to be open about their entire medical history-whether it's something that seems relevant or not. You never know when a rare condition like localized amyloidosis might pop up.
As dental hygienists, we often serve as the first line of defense in spotting abnormalities, keeping our patients informed, and guiding them to seek medical attention when needed. Understanding how conditions like systemic or localized amyloidosis can show up in the oral cavity is crucial, as early detection can lead to quicker treatment and better outcomes for our patients. So, stay curious. Even when you think you've seen it all, something can always surprise you in the ever-evolving field of oral health.
Michelle Strange, MSDH, RDH, CDIPC
Owner
Level Up Infection Prevention
References
1. Deng J, Chen Q, Ji P, Zeng X, Jin X. Oral amyloidosis: A strategy to differentiate systemic amyloidosis involving the oral cavity and localized amyloidosis. Oral Dis. 2019;25:670-675.
2. Types of amyloidosis. Boston University Chobanian & Avedisian School of Medicine: Amyloidosis Center. https://www.bu.edu/amyloid/what/types/. Accessed December 9, 2024.
3. Bezerra HKF, de França TRT, Prado JD, et al. Oral localized amyloidosis. Head and Neck Pathol 2022;16:818-822.
4. Greening M. A Dental Hygienist's Overview of Amyloidosis and Its Oral Implications. Today's RDH. https://www.todaysrdh.com/a-dental-hygienists-overview-of-amyloidosis-and-its-oral-implications/. Published October 28, 2023. Accessed December 9, 2024.
5. Thibault I, Vallières I. Macroglossia due to Systemic Amyloidosis: Is There a Role for Radiotherapy? Case Rep Oncol. 2011 May;4(2):392-9.
6. Fahrner KS, Black CC, Gosselin BJ. Localized amyloidosis of the tongue: a review. Am J Otolaryngol. 2004;25(3):186-189.
7. Pentenero M, Davico Bonino L, Tomasini C, Conrotto D, and Gandolfo S. Localized oral amyloidosis of the palate. Amyloid. 2006;13(1) 42-46.
Figures and Images
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Michelle Strange, MSDH, RDH
