Putting an end to the desperation

Becky Strong first noticed something wasn’t right in fall 2008. That’s when the nosebleeds started, and the tiny water blisters started to crop up in her mouth. She blamed it on stress. After all, she was simultaneously planning her wedding, serving as maid of honor in a friend’s nuptials, and helping another friend in the wake of heart surgery.

When the water blisters surfaced again, this time during her honeymoon to Antigua in early 2009, she figured it was because of the acidic food and umbrella drinks synonymous with the tropical locale. Then the blisters wouldn’t go away. Through the course of the next several months, those blisters turned to large, painful canker sores. By this point, Strong, a registered nurse, had bounced back and forth between her dentist and a GI doctor, trying prescription mouthwash with ingredients like Decadron and lidocaine. It helped, but when she stopped using it, the sores in her mouth came back with a vengeance. Come Thanksgiving, a few bites of mashed potatoes turned pink with the blood from the open sores. Meat was swallowed whole; she avoided her electric toothbrush at all costs, and opening her mouth all the way to talk — forget it.

“I was desperate,” recounted Strong, outreach manager for the International Pemphigus and Pemphigoid Foundation, to an audience of students, faculty and staff during a fall 2016 presentation at Texas A&M College of Dentistry. ‘’The greatest time of fear and worry is when a patient hasn’t been diagnosed.”

One unfortunate part of it all is that Strong saw her family dentist throughout the entire ordeal. But the dentist didn’t recognize the disease for what it was. It took an appointment with an internal medicine physician and subsequent referrals to an oral surgeon and dermatologist to pinpoint and treat the source of her pain: pemphigus vulgaris.

Because pemphigus vulgaris manifests itself in blisters, itching, stinging, burning and severe pain, it is often confused with other conditions. This means the average patient with this disease sees five health providers and waits an agonizing 10 months before receiving a correct diagnosis. It does have one telltale feature, however: In most patients with pemphigus, lesions will first form in the mouth. The same is true of one of its counterparts, mucus membrane pemphigoid, which also can affect the eyes, the upper respiratory tract and other mucosal surfaces of the body.

Dr. Terry Rees, professor emeritus in periodontics, who established the college’s Stomatology Center in 1984, invited Strong to speak at the dental school. He is quite familiar with these rare yet devastating autoimmune disorders that attack the skin and mucous membranes. His first memory of treating patients with pemphigus and pemphigoid traces back to 1968, when he devoted the majority of an extra year of periodontics training to oral medicine.

“The patients we see with these conditions often report that they have been unsuccessfully treated for yeast infections using antifungal medications, for viral infections using antivirals, and for bacterial infections using antibiotics,” says Rees. “It is also fairly easy to mistake either of these for toothpaste allergies or allergies to mouth rinses or other oral hygiene products. This, of course, is usually true quite early in the disease process.”

There’s one rule of thumb — an American Dental Association standard — Rees references that can shorten the arduous journey toward diagnosis. If a patient has dealt with an unexplained oral lesion for more than two weeks, either biopsy or referral to a specialist is indicated, such as an oral medicine provider, a clinical oral pathologist or an oral surgeon. Because of the disease’s divergent symptoms, pemphigus patients often consult professionals outside the dental spectrum, such as dermatologists, ophthalmologists, and ear, nose and throat doctors.

“I think the primary goal should be recognition that something unusual is going on,” Rees says. “Certainly dental colleges often have specialists on their faculty who are at least somewhat familiar with these diseases and who can help with the diagnosis and treatment.”

Dr. Nancy Burkhart, adjunct associate professor in periodontics and a registered dental hygienist, reiterates that getting patients to a dental school with a stomatology center or oral pathology department is key. It’s something she understands well, as she and Rees have counseled, supported and provided resources to thousands of patients through the college’s web-based Oral Lichen Planus Support Group, which they founded in 1997. Their ongoing dedication to early diagnosis and treatment of patients with oral mucosal diseases, including those with pemphigus and pemphigoid, their participation in patient seminars, and their efforts to expand the knowledge of these diseases among dental professionals recently earned them the 2017 Professional of the Year Award from the International Pemphigus and Pemphigoid Foundation. Among other initiatives, the organization offers an annual meeting for patients and caregivers and even free continuing education programs for dental professionals.

Rees and Burkhart hope that the more knowledgeable dentists are of the autoimmune condition, the more apt they’ll be to refer patients to the specialists who can help them. The faster patients can get to these professionals, the sooner they can complete necessary testing — in most all cases, a biopsy with histopathological evaluation and special stain plus immunofluorescence studies will be performed — and the sooner a diagnosis can be reached; in some cases, as early as two weeks.

Then healing can begin on multiple fronts.

“The medications that are used are key in stabilizing and controlling these diseases, but providing emotional support for any mucosal disease is also very crucial,” Burkhart says. “Patients often become discouraged because they may have seen multiple practitioners without an accurate diagnosis. When they get a complete diagnosis, they become much more involved in their own health care.”

Strong, like many patients, responded to a mix of immunosuppressants, but a vast array of treatment options is available. She no longer needs active treatment on an ongoing basis but still monitors lesion flare-ups just in case. Strong avoids acidic or alcohol-containing mouth rinses like the plague, and she sticks only to bland toothpaste and soft-bristled toothbrushes, such as the UltraSuave Red Brush, less likely to create lesions. Since avoiding dry mouth is crucial for these patients, Rees and Burkhart often recommend gel products such as MighTeaFlow, with green tea extract as the key ingredient, and Xerostom, which utilizes olive oil extract.

Strong lives with the reality that relapse is a possibility, but with a diagnosis and medical team now in place, feelings of desperation are kept at bay.

“I’m left with the hope that sharing my story with you could one day help one of your patients,” Strong said during her presentation.

“Dentists can help reduce fear and anxiety by giving clear, concise information. “There is a whole patient wrapped around the mouth. Compassion is key.”