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Fast Facts: Perio Edition - Periodontitis as a Manifestation of Systemic Disease - Papillon-Lefèvre

Fast Facts

Fast Facts: Perio Edition Episode 61

[Andrew Johnston, RDH]
Welcome back everyone! You are listening to another episode of Fast Facts- Perio Edition brought to you by A Tale of Two Hygienists in partnership with DentistRX. And now, please welcome your host, Katrina Sanders.
[Katrina Sanders, RDH]
Hello, and welcome to Fast Facts: Perio Edition. This week we are continuing the conversation around patients who present with anyone of multiple different types of rare diseases or conditions that align with Periodontitis as a Manifestation of Systemic Disease. This week we're talking about patients who present with Papillon-Lefèvre. Isn't that a fun syndrome to say? Although it might be fun to say it sounds like it's not very fun to experience. Patients who present with Papillon-Lefèvre are oftentimes characterized by patients who have hyperkeratosis of their tissue.
Most specifically, they present with hyperkeratosis of their palms and the bottoms of their feet. So no, these are just patients who need to get a pedicure more routinely. These are patients who will present with dry scaly patches of skin, and this starts early on in the process. In rare cases, we can also see patchiness associated with the eyelids, the lips, and the cheeks as well. These skin lesions also, of course, get worse if individuals are in colder climates and sometimes walking can even be painful with these individuals. As we see them early on, these are individuals who may even experience hyperhidrosis and odors associated with excessive sweating. These are also individuals, although rare, that may observe primary or deciduous teeth frequently becoming loose and falling out early on. So these might be patients that say "yeah, by the time I was five, all of my baby teeth were out" and this could be an indicator that this individual has Papillon-Lefèvre. But we're not just here to talk about palmar and plantar hyperkeratosis. The important piece is that although affected individuals will usually demonstrate appropriate form and eruption sequence of the teeth, these affected individuals will also experience chronic severe inflammatory and degenerative challenges of the tissues that surround and support the teeth.
The underlying ligaments we know are also typically involved. So early on in the primary dentition, the deciduous dentition, these young kiddos are experiencing red, swollen, and bleeding tissues. This may even move into stomatitis lymphadenopathy or swelling of the lymph nodes, and we might even start to see gum pockets forming around these gum tissues. We will also see notable halitosis in many cases, and for many of these young kiddos, chewing can even be painful. We start to see a thickening of the bony ridge, the alveolar process, which begins to cause that early breakdown or that loosening and falling out of teeth by age four or age five.
However, as we continue to see, of course, the inflammation is reduced and the gum tissues appear healthy when those primary teeth are lost. Now let's see those adult or permanent teeth move in. And when we start to see the adult or permanent teeth move in, these teeth if they are not treated efficiently and consistently, we can even see early tooth loss, even by age 16! These individuals can start to experience disease processes onset of periodontal disease that is frequent and recurring that oftentimes results in pus in the tissues and so with these individuals, we can oftentimes see that they are prematurely losing their teeth associated with the sequelae of periodontal disease. As with all of the rare diseases and conditions that we have discussed thus far, Papillon-Lefèvre is caused by a change in the CTSC gene. So of course, this, as one we may imagine, is a gene that plays an integral and critical role in the function of the body. And of course, this is where we start to see the sequelae in many different organ systems of the body associated with Papillon-Lefèvre.
Thank you for joining us this week. Join us next week as we continue the conversation around patients who may present with anyone of multiple, obscure and relatively rare diseases or conditions that may affect the periodontium. Thank you and have a great week.
This has been another episode of Fast Facts - Perio Edition with Katrina Sanders, RDH. Please feel free to reach me on Instagram @thedentalwinegenist or on my website Cheers.
[Andrew Johnston, RDH]
Thank you for listening to another episode of Fast Facts - Perio Edition, brought to you in part by DentistRX makers of the InteliSonic line of power brushes. Find out more by visiting their website at We'll see you next week for another Fast Fact!

Reference: Virulence Mechanisms of Leukotoxin from Aggregatibacter actinomycetemcomitans - Scientific Figure on ResearchGate. Available from: [accessed 27 Dec, 2021]

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