Oral Exam

ORAL PEMPHIGUS
AND PEMPHIGOID
BY NANCY W. BURKHART, BSDH, EDD

Pemphigus vulgaris and mucous membrane pemphigoid

are mucous membrane disorders that, like other mucosal disorders, can be difficult to initially diagnose. For
many patients, the search for a correct diagnosis involves
months or even years of anxiety and uncertainty.
One such patient is Becky Strong. She suffered for several years before obtaining an accurate diagnosis of oral
pemphigus, even though she exhibited the oral lesions that
are often an early symptom of pemphigus vulgaris. Becky
and I are both affiliated with the International Pemphigus
& Pemphigoid Foundation. Becky is a pemphigus vulgaris
patient, and I am on the Dental Advisory Council.
If youre not aware of it, you may be wondering, What
is the International Pemphigus & Pemphigoid Foundation
(IPPF)? The IPPF is a global organization dedicated to
improving the quality of life of all people diagnosed with,
or affected by, pemphigus or pemphigoid (P/P). The IPPF
provides direct access to innovative and effective support,
and it is a reliable source of information on P/P. I am proud
to be associated with the IPPF as a member of its Dental
Advisory Council, which includes an impressive list of
hygienists, dentists, pathologists, oral medicine practitioners, and faculty and students from various medical and
dental schools.
The IPPF recently launched an awareness website
(pemphigus.org/awareness), built specifically with dental
professionals in mind. The new website is the online home
to the IPPFs Awareness Campaign, which is seeking to reduce diagnosis times of P/P. Kate Frantz, Awareness Program Manager, has organized the campaign around the
slogan, Put It on Your Radar.
The website has valuable information for both patients
and dental providers, including access to peer health
coaches, diagnostic resources, and clinical photos. There
are opportunities to get involved with the organization, as
well as a section for continuing education credits for dental professionals.
UNDERSTANDING PEMPHIGUS AND PEMPHIGOID

What are pemphigus and pemphigoid (P/P)? What makes

a diagnosis so difficult, and why are they confused with
other disorders?
Pemphigus is a general term for a group of rare, autoimmune mucocutaneous diseases, with an established
immunological basis, but unknown etiology. Pemphigus

42 | rdhmag.com

Why are
Deck
these
conditions
goes
so hard to
here
diagnose?

can be life-threatening. The two

main types of pemphigus are
pemphigus vulgaris (PV) and pemphigus foliaceus (PF). A key characteristic of PV is blistering that may occur orally. Oral practitioners may classify
the appearance of PV and mucous membrane pemphigoid (MMP) as desquamative gingivitis or common types
of oral ulcers, effectively misdiagnosing the patient. MMP
may have the appearance of gingivitis and exhibit painful
ulcerations and blisters.
Desquamative gingivitis is a descriptive term and not a
diagnostic term. Fewer than 50,000 people in the United
States are affected, qualifying P/P as rare, ultra-orphan
diseases. As with most autoimmune type diseases, women
are affected more than men, with a mean onset of 50 to
60 years old. But both may occur in younger age groups as
well (see Figure 1).
Several forms of pemphigus exist:
Drug-induced pemphigus
Endemic pemphigus
Pemphigus erythematosus
Pemphigus foliaceus
Pemphigus vegetans
Pemphigus vulgaris
Paraneoplastic pemphigus
Benign familial pemphigus (Hailey-Hailey disease)
Pemphigus features a separation of the epithelial cells
caused by autoantibodies that attack a protein component
of the desmosomes, which is the adhesion site between the
epithelial cells. This protein component binds epithelial

NANCY W. BURKHART, BSDH, EdD, is an adjunct

associate professor in the department of
periodontics, Baylor College of Dentistry and
the Texas A & M Health Science Center, Dallas. Dr. Burkhart is founder and cohost of the
International Oral Lichen Planus Support Group
(http://bcdwp.web.tamhsc.edu/iolpdallas/)
and coauthor of General and Oral Pathology
for the Dental Hygienist. She was a 2006 Crest/
ADHA award winner. She is a 2012 Mentor of Distinction through
Philips Oral Healthcare and PennWell Corp. Her website for seminars on mucosal diseases, oral cancer, and oral pathology topics
is www.nancywburkhart.com.

RDH | March 2015

Oral Exam

cells together in stratified squamous epithelium. Bulla formation and ulceration occur due to this separation.
MUCOUS MEMBRANE PEMPHIGOID (MMP)

Mucous membrane pemphigoid (MMP) is a chronic, autoimmune, vesiculobullous disease. No contagion factors exist, it is rare in children, and our resources
point to it generally affecting women more than men. Occurrences most often are diagnosed in the fifth decade of life with an increased incidence after
age 70. The overall incidence is approximately seven to 10 new cases per million inhabitants per year. Antibodies that target the basement membrane and
weaken the underlying attachment to the connective tissue cause MMP. MMP
targets the gingiva and has the appearance of generalized gingivitis. When
affecting the gingiva, MMP may be referred to using the descriptive term of
desquamative gingivitis. As stated previously, the term is descriptive but not
diagnostic (see Figure 2).
The gingiva may slough and exhibit a Nikolskys sign. The skin, genitalia,
anterior nares, eyes, rectum, and esophagus may be involved. Diagnostically,
immunofluorescence will clearly identify MMP and PV, as well as rule out erosive lichen planus, erythema multiforme, discoid lupus erythematosus, and additional allergy type responses. The histological appearance viewed by using
immunofluorescence and histological staining, will indicate sub-basal separation of the epithelium from the underlying connective tissue.
BECKYS SEARCH FOR A DIAGNOSIS

I interviewed Becky Strong about her difficult journey to obtain a diagnosis and

Figure 1: Oral pemphigus vulgaris

Figure 2: Mucous membrane pemphigoid (courtesy of Dr. T.D. Rees)

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Oral Exam

subsequent treatment. Becky is a nurse, and she also speaks to

various groups of patients, dental students, and health-care
providers about her diagnosis and treatment.
She found the process of obtaining a diagnosis to be timeconsuming and exhausting, both physically and emotionally.
Becky commented that, as a nurse, she had a difficult time
finding the correct health-care provider to establish a diagnosis. She believes that, if she could not make sense of what was
occurring, the average patient would be in a far worse situation.
She began to have oral ulcers in 2008, and could not explain
them away. Her most memorable ordeal began in 2009. She had
bouts of no detectable oral lesions, followed by bouts of painful oral lesions. Becky was examined by dentists, physicians,
gastroenterologists, oral surgeons, and other health-care professionals, but didnt receive a definitive diagnosis until 2010.
During this time, while trying to establish a diagnosis, she was
given various medications and forms of treatment.
The one phrase that she heard many times was, I dont know
if this will help, but it certainly wont hurt. She describes the
process of a diagnosis as hit or miss during the entire ordeal.
Becky commented that, at times, she felt as though she may
be dying, and that the medical/dental community had failed to
find what was causing her illness. She felt badly that her husband had been on this roller-coaster ride with her.
He did not initially sign up for all of these issues, she

said. She commented that he told me later he thought he was

watching his new wife as she was dying. Becky also said that
her parents were worried for her, and she felt badly for them as
well. Becky and her husband wanted to start a family, but her
condition caused her much anxiety about potential problems
for her baby. When she successfully became pregnant in 2014,
her doctors followed her very closely for any complications to
her and her unborn child.
The years of anxiety, frustration, fear, and total perplexity in
the inability to obtain a diagnosis continue to amaze her. Becky
feels that a more methodical approach by both dental and medical providers would have rendered a more timely diagnosis.
CONSIDERATIONS FOR YOUR RADAR

Frantz (2014) lists some key recognition messages for dental hygienists. Practitioners should think of PV/MMP when there is a
combination of multiple oral lesions, chronic lesions, primary
lesions, and lesions that also occur outside of the mouth (skin
continued on pg. 95
REFERENCES
1. Burkhart NW. Mucous membrane pemphigoid. RDH. 2007; 27(3):
66,67,114.
2. Burkhart NW. Oral pemphigus vulgaris. RDH. 2007; 27(4): 84,85,94.
3. Frantz K. Oral pemphigus and pemphigoid: The role of the dental
hygienist. Access April 2014.

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References: 1. Earl JS and Langford RM. Am J Dent 2013; 26 (Spec Issue): 19A-24A. 2. Parkinson C et al. Am J Dent 2013, 26
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Circle 18 on card

Public Health
continued from pg. 18
comfort in coming to the elementary
school for their dental care. Being a part
of a program that continues to provide assistance to the community is very rewarding.
WHAT TYPE OF ADVICE WOULD
YOU GIVE TO A PRACTICING
HYGIENIST WHO IS THINKING OF
DOING SOMETHING DIFFERENT?

I would ask what area(s) do you enjoy

most about dental hygiene and encourage you to continue to pursue those
area(s) within your career in dental hygiene. There are many other opportunities for dental hygienists to include sales,
ownership, management, public health,
military, and more that you could experience and excel in throughout a career
in dental hygiene.
For those dental hygienists who are
feeling trapped in a certain setting,
please realize there are many opportunities. I have been fortunate to be in this
profession, and I have to admit, times
are changing gone are the days that
dental hygienists had only one career
option. Make changes if you feel that you
have other skills to offer! RDH

Evolving Hygienist
continued from pg. 33
differences should always be the foundation of dealing with the office drama
queen.
Whatever personality type rubs you
the wrong way, hopefully, after learning
a little bit about the different traits, youll
be able to understand people better and
realize you can react differently. Only we
control our emotions and reactions and
whether or not we allow someone to get
on our nerves. Its easy to forget that fact
and let someones behavior bother us. We
have to take back control and realize that
were the only ones who can give some-

RDH | March 2015

one the power to ruin our day. Have the

emotional maturity you need to take back
control and keep it. I know this is often
easier said than done, but with practice
it should get easier. I hope youve learned
about yourself and your coworkers and
that the information has helped you. Dont
let the office drama queen get to you any
longer! Happy scaling! RDH
REFERENCES
1. DiSC Preview, 1994 Inscape Publishing Inc.
Minneapolis, Minnesota. Hayes Ratledge,
Success Seminars Institute PO Box 18201,
Greensboro, NC 27419 Transformational Learning Opportunities hayes@hayesratledge.com
website: www.hayesratledge.com
2. DiSC Personality Test | DiSC Profile website:
https://www.corexcel.com/html/disc-personality-test.htm
3. Myers IB. Introduction to Type 6th Ed. https://
www.corexcel.com/html/disc-personality-test.
htmde to understanding your results on the
Myers-Briggs Type Indicator. Consulting Psychologists Press, Inc.: Pala Alto, California.
4. Shouse D. Communicating like a pro: Professional pointers for boosting your communication skills. 2008, SkillPath Publications Mission,
Kansas. p 62-64.

Oral Exam
continued from pg. 45
surfaces). The lesions may also occur
with minor trauma.
As the new slogan, Put It on Your
Radar, is being promoted, perhaps the
consideration of both pemphigus and
pemphigoid will be on your differential
diagnosis list, if these relevant factors
appear visible for your patient.
As always, listen to your patients and
continue to ask good questions! RDH

O R A L C A N C E R V. 2 . 0 1 5
continued from pg. 52
salivary transcriptome markers that
have been shown to be discriminatory
for oral squamous cell carcinoma, and
can assess the likelihood that the indi-

vidual in your chair is presenting with

oral squamous cell carcinoma. The test
was originally designed to provide confidence when assessing if an early stage
suspicious lesion detected during the
head and neck examination should be
evaluated histologically through biopsy.
By utilizing information regarding certain gene expression in the presence of
cancer, specifically the oral squamous
cell carcinoma that accounts for approximately 90% of all OHNC, the test can
detect the presence of early stage squamous cell carcinoma even carcinoma
in situ and hopefully avoid unnecessary biopsies; even better, prompt an
urgent biopsy of a lesion that previously
might have been approached with a waitand-see attitude.
Knowing that a highly accurate, noninvasive, easy-to-use chairside test now
exists to provide information about the
presence of oral and head and neck cancer, hopefully even more dentists and
hygienists will have the confidence to
provide the level of care that patients are
demanding as health care moves into a
new era of personalization.
So, make a belated resolution for 2015
to be the year your practice finally takes a
bold stand against oral cancer. Get serious
about your screening. After all, its only
March. The best is yet to come! RDH
DENNIS M. ABBOTT, DDS, is the founder and
CEO of Dental Oncology Professionals (DOP),
an oral medicine practice based in Dallas,
Texas, dedicated to care for the unique dental
and oral health needs of individuals battling
cancer. Dr. Abbott holds a bachelor of arts
in biology from Rice University in Houston,
and is an honor graduate of Baylor College
of Dentistry in Dallas. After dental school, Dr.
Abbott studied immunology, microbiology,
and oral medicine at the School of Dental
Medicine, State University of New York at Buffalo. In addition to private practice, he is a
member of the dental oncology medical staff
at Baylor Charles A. Sammons Cancer Center
and Baylor University Medical Center in Dallas
and serves as a consultant to the national
American Cancer Society in the development
of oral monitoring guidelines for post-treatment
cancer survivors.

rdhmag.com | 95

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