Have you ever heard of pemphigus and pemphigoid? Not many people have, since they only affect fewer than 50,000 people in the United States. These chronic, autoimmune diseases usually begin with painful lesions in the mouth. Typically, patients see 5 different doctors during 10 months before they get a correct diagnosis.
“We’re trying to reduce diagnostic delays,” said Kate Frantz, MPH, awareness program manager at the International Pemphigus & Pemphigoid Foundation (IPPF), which recently launched the educational website, putitonyourradar.org. “And because about two thirds of patients present symptoms orally first, we’re reaching the dental community to spread awareness.”
Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) target proteins that hold together skin and mucosal epithelial cells. When these proteins are attacked, the tissue blisters. This destruction results in multiple, chronic ulcerations and erosions of the skin and/or mucosa anywhere on the body, including inside the mouth.
“Patients describe eating a piece of lettuce like eating a shard of glass. It can be that painful,” said Frantz. “So for patients that are presenting orally, many times they end up being limited to eating very, very soft foods, or all they can tolerate are maybe smoothies.”
Both diseases usually begin in patients between the ages of 40 and 70, though they can occur at any age. Practitioners should consider PV and MMP when they see multiple oral lesions; ulcerations preceded by bullae; non-healing or slow to heal chronic lesions; primary lesions that run their course until resolved; or lesions outside the mouth.
Dentists who suspect PV or MMP should refer the patient to an oral surgeon for a biopsy, which is the only method for confirming the diagnosis, according to the IPPF. Neither disease is contagious, nor are they curable. But they are treatable, and patients and providers can manage their symptoms through high doses of oral or topical steroids.
Yet these diseases also can impact other aspects of dental care. For example, patients may find brushing painful and rely on soft foods to eat, increasing dental caries and periodontal disease. They also may experience more pain and bleeding during procedures than other patients. The IPPF offers dentists a management plan for treatment as well as other resources, including information about continuing education, at pemphigus.org.
“This is our dental website where anyone in the dental field can go and get more information about pemphigus and pemphigoid, biopsy information, and clinical presentation,” said Frantz, who also noted the important role that education plays in improving diagnosis.
“We’re sending trained patient speakers out to dental schools across the United States to share their story. We’ve already done this at 12 schools and have reached more than 1,700 people. The patients share their emotional story toward receiving a diagnosis. We usually do that during oral pathology lecture hours,” Frantz said. “We’re trying to connect that scientific piece with that emotional patient story.”
The IPPF offers patients resources as well. In addition to information about diagnosis and care available on the website, it provides advice on speaking with dentists, conference calls led by noted physicians and scientists, seminars and meetings, an annual conference, a quarterly magazine, and peer health coaches.
“If they call our foundation, we can connect them with a peer health coach, someone who has pemphigus or pemphigoid, and they can walk them through and educate them on various treatments that are available,” said Frantz. “We also have a physician referral list, so if they’re looking for an expert in the area, we can try to point them in the right direction.”
The emphasis on early diagnosis and support is vital, the organization believes, because delays in treatment can lead to greater pain and suffering, greater risk for progression to other lesions, more difficulties in achieving remission, greater risk for poor treatment response, reduced productivity and quality of life, weight loss, fear and anxiety, and increased healthcare expenses.
“Dentists are one of the first clinicians that patients go to see because of its oral presentation, and so we really want to make sure that dentists are aware of it and recognize it,” said Frantz.
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