Patient Seminar Series: Pemphigus and Pemphigoid
Imagine that you’ve just started the adventure of a lifetime, moving across the world to Australia and living a healthy life full of yoga, good food, and walks on the beach. Then, one day at brunch, you realize that your mouth feel different: You can lift a flap of your gums with your tongue.
“It was as if my tongue were a knife and my gums were like butter, and I could just slice the gum off, and I could bury the tip of my tongue in my gum,” said Ellen Levine during a Zoom presentation on April 5, 2022, entitled “The Diagnosis Journey of a Pemphigus Patient.”
The presentation was given as part of the school’s Patient Seminar Series, in which guest patients help “put a face” on oral-related diseases encountered only rarely, but which have marked consequences for those affected. The seminar series is directed by Dr. Yoshiyuki Mochida, clinical associate professor of Molecular & Cell Biology and also a Medical Advisory Council member of the International Pemphigus & Pemphigoid Foundation (IPPF).
Levine is a patient educator with the International Pemphigus & Pemphigoid Foundation’s Awareness Program. Through this program, Levine shares her diagnosis story with dental students and professionals to increase awareness about the illnesses and to improve the diagnosis process.
Pemphigus and pemphigoid are chronic autoimmune, mucocutaneous surface disorders. While there’s no cure for these illnesses, they are not contagious and can be managed with treatment. About 80 percent of patients who develop pemphigus vulgaris will develop lesions in their mouth first, which places dentists in a unique position to be able to identify and diagnose this disease.
From the moment Levine first experienced symptoms during that brunch, it took visits to at least 11 different healthcare providers to receive a diagnosis – even though pemphigus had been mentioned as an outside possibility at her first appointment, with a dentist. Levine ended up moving back to the United States after that first visit, as she was facing long waits to see specialists in Australia.
Throughout her journey to a diagnosis, Levine endured a number of inappropriate and inadequate treatments, including a deep-scale cleaning that caused even more lesions and irritation, as well as a number of misdiagnoses.
“During my quest to find the right specialist … I really did feel like Dorothy, following the yellow brick road in search of the Wizard of Oz,” Levine said.
She continued later, “I really felt trapped in a nightmare that wasn’t ending, trying to work my way through an endless maze of obstacles and medical appointments. And I really was filled with this overwhelming fear, wondering, ‘Why isn’t my mouth cleared up?’”
Finally, four months after her oral lesions first appeared, Levine received a definitive diagnosis of pemphigus and found a dermatologist who could treat her. There was still a long road ahead: As Levine’s doctor worked to identify the right treatment protocol, the lesions and blisters in Levine’s mouth eventually spread to almost every part of her body.
Eventually, they found an approach that worked, and Levine has been in and out of remission over the last 15 years. Now, she said she feels ownership over her treatment protocol, and if she feels a flare up coming on, her doctor trusts her judgment on how best to respond.
One of many things that Levine found challenging is that she was advised not to have her teeth cleaned during the first year after her diagnosis, to avoid tearing her sensitive gums and aggravating any oral lesions. Eventually she was able to find a periodontist who understood her journey with pemphigus and is able to give her gentle periodontic cleanings that do not aggravate her pemphigus.
Levine noted that it’s important for all healthcare providers to cultivate trust-based partnerships with their patients. She also emphasized that while a rare disease may be “fascinating” for the provider, it is terrifying for the patient.
Dentists may be the first point of contact for patients with a rare disease, so it’s important that they search until they can identify an answer – or recognize something that’s outside their sphere of expertise and refer the patient to someone who can help, said Levine.
“As future dental professionals, you have the unique opportunity, as well as the obligation, to help identify any pathology you may come across and steer the course of your patient’s lives,” Levine said.