Yearly Archives: 2013

Just as life is a journey, so is living with chronic illnesses like pemphigus or pemphigoid. In the last newsletter, I emphasized transitions along my personal journey. Chronic illness is an adventure no one signs up for, but once the diseases are triggered, life is never again exactly the same.

For those newly diagnosed, or their family, friends or caregivers, it can all seem unreal at first. There is a vague beginning to the new normal, as it takes time to get educated and to process the diagnosis and information. Even after there is an acceptance of the situation, it is overwhelming.

Everyday life becomes uncharted territory. One thing is for sure: now challenges seem to pop up a lot more. How the challenges are met will make the difference between function and dysfunction.

The pemphigus and pemphigoid (P/P) community is not a static one. Everyone goes through different stages in different ways, but with more similarities than differences. This is why the community continues to grow. People who have had very similar situations – and have made it through to the other side – are there to help you. Eventually, once your own life and illness have stabilized, you will have the opportunity to be there for others.

Some people will get more ill than others, and some people will respond more quickly to treatment. For everyone this is an adjustment — and again the degree of difficulty differs between people. Having an IPPF community, with a discussion group, a social media presence, award-winning newsletter and website, peer health coaches and volunteers (and more!), has helped many people along this journey in so many positive ways. Maybe YOU have been helped by one or more of our resources?

The IPPF works hard to ensure that no one has to go through this alone; this is not just helpful, but a bond, and one that strengthens everyone involved. It is a lifelong bond – one that keeps gaining strength. No one has to navigate this road alone any more.

This is the time of the year to give; but in our community, there is a need 365 days a year – not just one “giving” season. If you are not currently participating in some way – ANY way – there are many who would love to see you get involved.

ou do not have to dive into the deep end.

Start small, get your feet wet and move forward at a pace that is comfortable for you. Ask for help, guidance and advice along the way.

Consider sharing part of your journey with others in the P/P community. Maybe you…

  • know of an informative article we can use in the Quarterly?
  • would like to contribute a personal story that inspires others?
  • are available for an interview for an article?
  • can reach out to a newly diagnosed patient to lend an ear and encouragement?
  • have advice for the newly diagnosed we can share on a broader scale?

Because of the support in our growing community, health coaches, webinars and annual patient meetings, you will meet people who can help you learn new positive coping strategies. These new tools will allow you to continue your own movement, becoming more and more resilient along the way. You will internalize the newer and more positive coping mechanisms, and hopefully leave behind older, strategies that are no longer working.

The point is that you WILL find yourself feeling and presenting yourself differently in this world. When you calmly and rationally respond to someone in a new way (rather than reacting), your response may even change how the other person responds to you – and others – in the future. You will gain momentum and move forward in your personal journey.

As I write this column, it is a typical colorful Pittsburgh Fall. Some trees are still green, some have lost their leaves and are bare, and others are continuing to change colors and are absolutely glorious.

Practicing mindfulness and being “in the moment” allows one to just watch and enjoy nature with a combination of awe and appreciation. I’m not a personal fan of cold weather and bare trees, I know we will have winter wonderlands soon, with freshly fallen glistening snow – on the ground and on the trees. Everyone’s personal journey moves forward and changes just as the seasons change and cycle.

During your life journeys there are choices to make and different roads which can be taken. You can take the road most often taken or take the one not usually taken — neither is correct or incorrect. Just remember that there are always choices. Also try to remember that road that seems most “safe” may not be and may not help move you forward. Everyone makes mistakes, because humans are not perfect. Mistakes help people learn. Moving out of your comfort zone is not easy; it is a choice, and if you choose to stay on the same seemingly “safe” road, that is also a choice. Please let this last point sink in. DOING NOTHING IS A CHOICE.

Yes, life is a journey, but as you travel through, remember this quote: “What lies behind us and what lies before us are tiny matters compared to what lies within us” (Ralph Waldo Emerson). If you are reading this article and have gotten this far, look within yourself for that extra strength that may seem to be eluding you; it doesn’t always come from others. Maybe you just need to look a bit harder within. And, do not be afraid to ask for help.

Happy holidays. Try to start a new tradition this year. Tis the season to give – not just money, but of yourself. It will make your own personal journey more interesting and also sweeter, and by reaching out to others you will be making a far more important and satisfying contribution.

People often have a difficult time understanding what hypnosis and hypnotherapy are and what their purpose might be. If you’ve ever been to a fair and a hypnotist asks someone to “cluck like a chicken,” and they do, the individual clucking knows exactly what he/she is doing. They may not care that they look silly because they are so relaxed from the hypnosis.

If you notice, there is usually always someone who won’t perform – these actions indicate that a person cannot do anything against their nature. In a less public setting, the practice of hypnotherapy is a valuable and positive method of helping people cope with many different and difficult issues.

Hypnosis is the process a hypnotherapist uses to help a patient find answers to issues that they are having trouble controlling. But what is hypnosis?

Most agree that it is a naturally altered state of consciousness. As defined by Gil Boyne, one of the leaders in hypnotherapy, it is “an extraordinary quality of mental, physical and emotional relaxation.” Many studies have shown that a person in hypnosis may show psychological and physiological changes that can be beneficial.

We all experience a form of hypnosis when we find ourselves “lost in the moment.” If you’re driving down the road listening to the radio and you notice that you’ve gone three exits without even realizing it – that is a form of hypnosis. Or, if you’re on your computer and in such a deep state of concentration that you don’t even hear the noises around you – that, too, is a form of hypnosis. What a hypnotherapist does is take you into that natural state of intense concentration and relaxation.

What is Stress? Stress is something we all deal with on a daily basis. Stress can be a good for alerting us to dangers. A rush of adrenaline can give you amazing strength and can help get you through physical and emotional challenges. If you diagnosed with a life-threatening disease like pemphigus or pemphigoid, stress levels may increase substantially, and for a sustained period of time. The issues that confront us can be overwhelming. Not only are we dealing with the illness itself, but the issues that come with it.

How can I live successfully with the drugs which can in themselves increase my stress levels? How is this affecting my family? Will I have the financial resources needed?.

All these issues elevate our stress levels substantially. But what does that mean physically? Stress over a lengthy period can raise blood pressure, cause irritability, cause our thoughts to race, and many other problems. Many of us turn to drug therapy – both prescription or non-prescription to reduce the symptoms, while some of us look for alternative methods to augment drug therapy — these alternative methods include acupuncture, yoga, exercise, meditation and hypnosis.

What does hypnosis do that the other alternative stress reducing methods don’t? If you find the right hypnotherapist, someone you trust, you use your mind, your imagination, and your trust that the person you are working with can help you alter any negative thoughts or habits that plague you. The techniques the hypnotherapist uses are proactive approaches, meaning that you follow the suggestions of the therapist and use your subconscious to perceive your issues in a different way.

There are definitely good and bad hypnotherapists, and there are places to go to find one — the American Society of Clinical Hypnosis ( is an example of one. But often as with a traditional therapist, you use your instincts in your initial interview to know whether that person is right for you.

I became interested in hypnosis many years ago. Since pemphigus and pemphigoid are autoimmune diseases (diseases of the self), I thought if I could learn all that I could about pemphigus and how the disease worked, maybe I could “talk myself out of it.” Unfortunately, my situation precluded me from furthering my quest. However, I learned to meditate, which helped with the side effects of prednisone. A 30mg dose every day for 3 years worked well and put me in remission..

At this point, I was able to put the theory that I could control my own body on hold. Some years later, when the disease returned, I again started thinking whether hypnosis could help. I searched the Internet looking for any literature on the subject and came across a small study that was performed by Dr. Francisco Tausch at Johns Hopkins University, on hypnosis and psoriasis..

I invited Dr. Tausch to speak at the 2005 IPPF Annual Meeting in Arlington, Virginia, on this subject because his research indicated that hypnosis might be helpful in treating psoriasis.

Regrettably, his work on the possible connection was not yet complete. Could hypnosis help with pemphigus and pemphigoid? That is an unanswered question. However, from my two years preparing for my certification, and from my practice of hypnotherapy, I have learned for myself and from several people I’ve worked with that it reduces stress levels. This can open up one’s ability to look at life in a different way. I’ve been in remission — no drugs – from PV for 12 years but I have a high anti-Dsg3 titer count, making me very susceptible to lesions. Hypnosis has helped me reduce my stress so that I can notice my triggers if I get an oral lesion (which I do from time to time). As with any case study, it is not clear if the hypnosis has helped me to be successful in remission and to have a minimal, manageable number of lesions, but I believe that the power of hypnosis has allowed me to take some control over my body.

Because doctors often cannot spend a lot of time with an individual patient, the emotional component to treatment (their bedside manner) is often minimal. Hynotherapy can be an extremely helpful factor in the emotional recovery of people with illnesses. When we are under stress because of an illness, our perspectives change. We tend to view things differently — whether positive or negative. We notice changes in our bodies we might not have noticed otherwise. What hypnosis can do with stress (and also pain) is to reduce its intensity and often change our perceptions of our feelings.
We often tend to ignore our emotional needs when faced with disease. We hide our feelings making them less important than our physical state. As humans, we are all physical, emotional and spiritual beings. The only way to really bring health and well-being in a time of crisis is to acknowledge when dealing with illness, we must deal with the whole person.

664715_11160870-pillRare diseases, including several autoimmune disorders, are getting more attention from drug-makers, according to a new report by the Pharmaceutical Research and Manufacturers of America (PhRMA), a consortium of 36 US-based pharmaceutical and biotechnology companies. In 2012 alone, 13 drugs for orphan diseases (“orphan drugs”) were approved by the Food and Drug Administration (FDA). Approximately 452 medicines and vaccines are in development for the nearly 7,000 orphan diseases worldwide.

orphan diseases are defined as diseases with fewer than 200,000 patients. In total, however, across the nearly 7,000 orphan diseases, 30 million people in the US, or about 10% of the population, are affected by an orphan disease. The pemphigus and pemphigoid (P/P) diseases are considered “ultra-orphan” diseases because they are extremely rare. It is estimated there are only about 50,000 new P/P cases each year worldwide, with only a few thousand of those being in the US.

Rare diseases tend to be more complex than common diseases, meaning that there are a number of factors that combine to cause disease. In the case of P/P, while there seem to be genetic risk factors, how these contribute, singularly or in combination, and to what extent the environment (like diet and other conditions that are present) also contributes is not well understood.

Somewhat fortuitously, complex diseases represent the next great frontier for drug developers. Having tapped into the ‘simpler’ diseases, making great strides in treatment of conditions like high cholesterol, these ‘low-hanging fruit’, as drug-makers like to call them, have been consumed. It is truly a time of paradigm-shifting mentality among drug makers.

That said, the costs of developing new medicines is extremely high, so companies must make their choices wisely. If we were to calculate the amount that pharmaceutical and biotechnology companies spend on research and development yearly and compare that to the number of drugs that are approved for clinical use by the FDA each year, the cost per successful drug is a staggering $1.2 billion. It’s not difficult to imagine, then, why companies aimed at developing new drugs are most interested in those that can recoup these huge costs — for instance, by developing drugs for very common conditions and risk factors such as diabetes and high cholesterol. As well, given the complex nature of rare diseases, they are not necessarily among the ‘low-hanging fruit’ that some diseases represent.

To incentivize companies to prioritize new drugs for rare conditions, they may apply for orphan drug status through the FDA, a result of passage of the Orphan Drug Act (ODA) of 1983. With this status, a drug receives seven years of market exclusivity. Market exclusivity is particularly appealing to companies developing drugs because the seven-year exclusivity period differs from laws applicable to other drugs in that it does not begin until the drug is approved by the FDA approval.

The ODA is considered a resounding success. Since its inception, there have been more than 400 medicines approved for a total of 447 orphan diseases. As well, there are hundreds of new medicines in development, including an impressive list available in the PhRMA 2013 report (

While not all of the 452 orphan drugs in development will be approved for patient use, this is certainly a lot of activity. A search of the list included within the PhRMA report, as well as a search of (that lists all clinical trials in progress), shows a handful of drugs in testing for conditions related to or directed at P/P.

There are 18 new orphan drugs in phase I-III trials (there are three phases of clinical trials and drugs must pass all of them, indicating reasonable levels of safety and meaningful efficacy-effectiveness in treating the condition) that are indicated for autoimmune disorders.

New drugs are not the only source of treatment for disease. Another source is to use an existing drug, developed for another condition, for a different indication. Such is the case with Rituxan® (rituximab), which was originally developed for Non-Hodgkin’s lymphoma. In that disease, B cells of the immune system bearing a marker called CD20 (thus the name CD20+ B cells) have gone awry.

Since P/P shares this hallmark, Rituxan® has been successfully used ‘off-label’ for P/P. It is an antibody-based drug, which requires it to be injected into the patient. In general, any drug that acts as a suppressor of the immune system (immunosuppressant) is a potential candidate for treating a range of auto-immune conditions, including P/P. CellCept® (mycophenolate mofetil), another immune system suppressor that was developed for transplant patients to help prevent the body’s rejection of the ‘foreign’ organ, has recently been approved for use in P/P.

Besides the high cost of developing new drugs, companies that seek treatments for orphan diseases face difficulty in finding enough patients to participate. Indeed, patients tend to be dispersed geographically and may include small children. Physicians and patients who are interested in participating in trials or gaining more information should visit

Within the P/P community, the IPPF is also a great resource for learning about clinical trials. Members of our medical advisory board serve as investigators on trials and being in our patient database could lead to a company reaching out to you about participating in a trial.

For instance, among the new drugs aimed at treating P/P, drug-maker Novartis is studying VAY736, an antibody-based drug aimed at another B cell marker called BAFF-R. The study is in a very early stage and should be recruiting patients soon.

The time is ripe for development of new drugs for complex orphan diseases. The surge in new medicines in the first 30 years since the ODA should accelerate as less ‘low-hanging fruit’ exist for companies developing new drugs.

Season’s Greetings! It’s that time of the year again and here at the office we are getting ready for 2014. But we still have another month to go, and that means finishing up 2013 with a bang!

Kate Frantz is settling in and getting the Awareness Campaign up and running; Monique Rivera has been busy processing donations and keeping the information flowing; Kevin Cruz has been working on patient and caregiver handbooks; Isaac Silva has been doing some web site work while learning more about the IPPF; Marc Yale and the Peer Health Coaches have been helping patients at an astonishing rate; and the Board of Directors and our Medical Advisory Board continue to be instrumental in shaping our future.

2013 Holiday Fundraiser. This fundraising season is shorter than usual with Thanksgiving falling where it did, but so far this has been one of our best efforts in recent years. Along with first-time supporters like Rana, Hartmut, Chris, Patricia, and Bob it has been great to see the names of long-time supporters like Lillie, Joan, Alice, Yvette, Carol, Ellen, Jay, Sonia, Ed, Therese, and dozens of others who continue to support our important work. If you have not donated there is still time. And make sure to date your check accordingly so you get your 2013 tax-deduction!

Awareness and Education. If you missed the August buzz on Facebook, Rebecca Oling and MAB member Dr. Animesh Sinha were guests on Good Morning New Haven (Connecticut) talking about pemphigus, pemphigoid, and the IPPF. She followed that up with a trip to Houston, Texas, to participate in a 4-hour long video shoot for IPPF that will be added to the Giving Library ( Add to that scouting the globe for patient educators, helping people on Facebook, traveling to sit with patients and their families, working full time, and being “Mom, wife, daughter, and sister” and you can see why Rebecca is my hero!

What do you get someone turning 20? How about a party in Chicago! The IPPF celebrates its 20th Anniversary in 2014 and we are planning an evening of food, friends, and fun at the 2014 Patient Conference. On Saturday evening (April 26, 2014) join us for dinner, our Annual Awards, and an Anniversary tribute followed by a Casino Night fundraising event. More information will be available soon! Proceeds go directly to our Patient Support programs. If you’re in Chicago, I hope to see you there!

2014 is now a couple minutes closer than it was before you read this letter. With Autoimmune Disease Awareness Month, Rare Disease Day, Pemphigus & Pemphigoid Education and Awareness Month (New Jersey…come on NJ SJR77!), 17th Annual Patient Conference, and more on the horizon – we need your help! If you’re interested in spreading the word and mission of the IPPF, call or email me and let’s do this together!

Thank you, Happy Holidays, and from my family to yours – Happy New Year!

The IPPF is looking for volunteers to help us with many of our education and support projects and programs, including in several newly-created roles. The three main types of positions we are looking to fill are Executive Board (CEO’s senior staff), Special Projects Contributors, and General Volunteers.
The Executive Board heads up a “department” or initiative under the CEO. This could include efforts like managing our patient support programs, planning and running our annual Patient Conference, running fundraising events, volunteer management, and research and development. Other areas include accounting, collaborations, and project management.

  • The commitment would be for one year or the duration of the initiative, whichever is shorter. And we are open to ideas for new “departments” and initiatives, and will even tailor a position to an individual’s talents and interests. This role is ideal for people that are operationally-oriented and want a significant role within the foundation.

Special Projects Contributors (SPC) have special expertise, resources, and access to give the IPPF a short-time commitment revolving around a specific area. This allows individuals to make a substantive contribution that fits their interests and lifestyle. Example projects include:

  • competitive landscape analysis
    review of clinical guidelines
    website and/or graphic design
    exploration of a specific clinical care question
    legal analysis of a specific issue
    accounting and investments
    technical writing and reporting
    building a data management platform for our patient registry
    development strategy
    helping set up and negotiate an external collaboration
    developing a market access/reimbursement advocacy strategy

The SPC role is great for people that have a special expertise they would like to use for a short-term project. Volunteers are encouraged to propose their own projects as well.
And equally important are the General Volunteers. General volunteers contribute across a wide range of initiatives and events. Example include newsletter and website contributions, print and graphic design, Patient Conference preparation and on-site help, event planning, peer support, and community fundraisers (e.g., 5K run, bake sales, wine and cheese tasting, golf outings, etc.). This role provides an opportunity for anyone to contribute with as much or as little time as they can.
We encourage those interested to tell us about their talents and interests, and we are happy to find a way for them to contribute.
These are great opportunities for anyone that feels strongly about our community, wants to give back to the Foundation with their time and expertise, and wants to help grow our organization to deliver greater impact.
All volunteers receive acknowledgment in our newsletters and website, and they can list the experience or project in CVs, resumes, and related documents.
If you or someone you know would like to get involved, please contact me at or by calling the office at 855-4PEMPHIGUS (855.473.6744) extension 103.
Helping the IPPF through volunteering is a great way to help us help others. That’s the Power of Giving Back!

In the days after being told that you have one of the diseases in the Pemphigus/Pemphigoid family, when you’re running around filling prescriptions for your new, huge, seven-day pill container, it’s hard to feel hopeful.

Maybe you’ve been looking for answers for months – and getting the wrong ones. Maybe you’re finding it a challenge to take care of ordinary everyday things, like brushing your teeth because of painful oral lesions. Or you don’t know how to explain to friends that you’re miserable and not feeling up to your usual activities.

That’s how it was for me. By the time I was diagnosed with PV nearly three years ago, I’d consulted five different doctors over five months and had a medicine cabinet filled with failed ointments, pills and rinses.

Because my symptoms were so widespread, and because the doctors I saw were not familiar with Pemphigus, I’d been told I had everything from allergies to cold sores to possibly cancer.

It was weird hearing doctors, whom I instinctively trusted, tell me that this or that was going on with me, and turn out to be wrong, time after time. Each doctor focused on his or her special area of expertise, ignoring the symptoms that didn’t fit.

I remember calling one doctor to complain the medicine she’d prescribed wasn’t working, and she told me I was using it incorrectly.

Another doctor simply increased the dose of what he’d given me, and a third told me that if a certain cream didn’t work in two weeks, I should come back and be biopsied for cancer.


Finally I saw a doctor who said the magic words, “I don’t know what it is,” and referred me to a great dermatologist, who biopsied me and put a name to my condition. Under his care I began the slow uphill journey back to health.

For me, the hardest part was not the discomfort of eating or washing my hair or the harsh side effects from medications, unpleasant as they were. It was the sad sense that I’d lost something precious and my life would never be the same.

As I learned how to cope with PV over time I found out that, like other major life events, this experience offered a unique opportunity to grow and learn more about myself.

Some encouraging words, for those new to this journey:

1. You will feel better, a little at a time. Celebrate the small steps as your health improves each day, each week and each month.
2. Remember P/P is just a small part of who you are. While it may loom large right now, it will command less of your attention as time goes on.
3. Keep a health journal. I found it extremely helpful – especially in those foggy prednisone days when I got lost in the middle of sentences – to record everything health-related in a notebook.
I wrote down the details of every doctor visit, the questions I wanted to remember to ask, the answers I got, symptoms and feelings, medication doses and so on. I’ve now had my notebook for three years, and it’s so handy for keeping track of lab work, bone density scans and other treatments that occur at regular intervals.
Writing things down also keeps them from swirling endlessly around in your mind and is helpful when talking to your doctor.
4. De-stress any way you can. At the 2012 IPPF Patient Meeting in San Francisco, we learned about the chemicals released by stress that aggravate autoimmune disorders.
My favorite way to get rid of excess stress is by doing yoga. I also enjoy walking, and when I don’t have time for either of those, a few long, deep breaths do wonders.
5. Count on your friends at the IPPF. There’s a wealth of help at the IPPF. You can get one-on-one support from a trained Peer Health Coach, ask questions on the discussion forum or join the active email group.
Online resources, dial-in Town Hall meetings with IPPF’s doctor-researchers, and annual Patient Conferences are other options. I made the mistake of waiting too long before getting involved with this fantastic organization.
6. Give back. Share a tip that worked for you or just lend an ear to help someone else who’s newer to P/P than you. See #5 for places you can jump in.

GlassesLiving in the northeast (actually mid-Atlantic), it is still very much summer – with humid heat and lush greenery and flowers surrounding me outside – as I write this. Nature is very much alive and in all its glory. In another month or so, I know that the leaves will be changing colors and dying while retaining their extraordinary seasonal beauty. It is really quite remarkable how beautiful nature is when dying. It doesn’t feel like a death, but more like a segue into a new and different way of being in the geographical areas where we experience the seasons and annual changes. Changes and transitions are always with us; some are just more noticeable and harder hitting. It is a cycle that continues – even with global warming and human intervention. As always, the only constant IS change – and so transitions are part of our lives whether we fight them every step of the way or accept and welcome them, learning from our experiences. Changes of any kind can take us out of our comfort zones, but are necessary for our individual journeys. People are complaining about the very hot and humid summer we are experiencing, but it brings back memories of the old “dog days of summer” which I fondly remember growing up with in the 50’s & early 60’s – without the comfort of air conditioning or even fans. I have no idea how we did it, but we did – and had fun even while sweltering. Running through a “sprinkler” attached to a hose on someone’s grass was like a piece of heaven in those days, but nights were more challenging! This made the season change even more eagerly anticipated; it always felt like summer lingered a bit too long. Of course, with age, the days, weeks, months and seasons seem to go more and more quickly, keeping us on our toes. No two years (or days) are exactly the same, which keep us guessing just as these rare chronic illnesses do. There are less challenging days and more challenging days with tears of both joy and sadness/loss along the way. The days merge into weeks and then months. Seasons change and other transitions – some smooth and expected. A lot of people become conditioned to dealing with crises, and encountering and overcoming problems and difficulties usually leads to more resilience – a very positive quality to possess. Unfortunately, a growing number of people seem very at ease with crises, seeming to live from crisis to crisis while not dealing with everyday life. This is not a natural way to go through life and leads to very real daily difficulties and unhealthy stress levels. The studies on stress continue to highlight the negative aspects of the mind-body connection. No, not every ache, pain or serious illness is caused by stress; but stress-relief strategies that work can make everyday life “easier” (or less difficult) and make crises smoother to navigate. This can be thought of as a navigation system of sorts in our life’s journeys. Unfortunately the navigation system must be learned through training, experiences and successes and failures. Often we do learn the most from our failures. We all go through stages and often with some back-peddling from time to time. These are NOT negative times. Think about an EKG; a flat line is not a good thing. The small ups and downs are totally “normal”, and it is only when there are extreme changes that they are harmful. That is when people need extra help and more and more often seek it out – or go back to a therapist or psychologist (or family member, friend, colleague) for a “tune up”. Sometimes the positive coping tools are misplaced, lost, forgotten, rusty or no longer enough. Frequently, one of the most common initial diagnoses for patients is “Adjustment Disorder”, which usually passes once new tools and strategies have been learned, especially with a mindfulness based cognitive behavioral intervention. Psychotropic medication may or may not be prescribed, and some are short-term, while others may be more long-term in more complicated cases, or when the person has deeper underlying emotional or psychological problems (e.g., bipolar disorder, recurrent depression, a personality or panic or other anxiety disorder). Sometimes previously repressed underlying issues emerge and take longer to resolve. Financial, insurance, work, primary support (or lack thereof), physical illness and other social and environmental concerns can work toward or against the eventual resolution of problems. Being aware of these and of the larger situation always helps. For me, I have spent 10 years on boards devoted to pemphigus and related diseases and have watched and participated in many changes over this decade. It has been an amazing and exciting time. I went from being a healthy and high-energy person with one long-term autoimmune condition that was under control (for decades) to developing new and life-threatening conditions. My previous chronic condition, along with other acute health issues at various times, probably gave me an advantage regarding resilience. I went through the same basic stages as anyone else, but also felt very strongly almost from day one that it was all for a reason – for a purpose. I soon learned that the psychological and emotional components of these orphan diseases were not being addressed. It is, of course, always critical to get the disease itself under control, but I would argue that being as mentally healthy as possible and with a strong support group is nearly equally important. I have not been among the fortunate who have been in remission for any considerable length of time. My one partial remission included symptomatology that was atypical. That diagnosis was made and addressed; monitoring myself daily is routine. After a particularly nasty and stubborn flare this year, I felt the need to make some serious new life decisions. I did use the Cognitive model of decision making, examining all the facts and then carefully weighing options, while taking a few steps back for perspective. I have made some decisive strategic moves, including stepping down from the IPPF Board of Directors. However, I will always remain very involved in the IPPF, especially where patient support is involved. his will allow me to have more time to make necessary decisions regarding my own health, volunteer and professional work and family. It is just one more life transition along the road. The coming years will bring new and different challenges and changes, but these transitions are necessary and very positive. Our IPPF Board of Directors has several new and very capable and passionate people who will continue to lead this Foundation forward in new and important ways. It is a very exciting time for the IPPF and the welfare of all patients, families and friends. My participation with the IPPF remains an integral part of my life, but it is a time to re-prioritize, take the time to smell the roses in the moment, enjoy the beauty of the fall/autumn leaves and then make snowmen or just watch the winter wonderland, before spring and the start of the next season. I look forward to this transition and to the new and interesting places and opportunities which will be part of the next phase of my own personal journey. Not only do I not fear transitions, but I look forward to them and what they will add to my personal human experience. Also, your Annual Meeting Committee is working hard to make the 17th Annual Patient Conference in Chicago, April 25-27, 2014, a fulfilling experience for all who attend. We are looking at trying more changes, based in large part on feedback from patients and their families and friends who’ve attended the conferences. Yes, we are listening and making necessary changes. Please consider what special talents or interests you may have or have access to as we ramp up our volunteer system and add ad hoc members to committees. Those experiences will not only add to your personal journeys, but also the future of the IPPF. And who knows? Maybe it will be a special and transitional time for you!
Summer is drawing to an end, kids are back in school, and the IPPF is preparing for our end-of-year activities. I want to thank everyone help us carry our mission to the four corners of the globe. Volunteers are becoming more and more important in our daily operations, and every bit counts.

Helping us in the office will be not one, but two Cristo Rey High School student interns. For the fourth year in a row, we welcome back Kevin Cruz. Kevin has spent the past few years entering data into our patient database, uploading content to the website and Facebook page (who can forget his Chuck Norris quotes?), and other IT functions. This year he is working on patient and caregiver guides to provide essential information and tools.

And we welcome Freshman Isaac Silva who will help on the administrative side as he learns about our mission, programs, and services. Isaac will also help with the patient and caregiver guides to help him learn more about our community and how the diseases affect patients, caregivers, and families.

Cristo Rey Work Study allows low-income students to gain real-world experience in professional settings while receiving a private education. For more information on Cristo Rey High School Sacramento visit

And while I’m on the subject of helping move the IPPF forward, let’s keep talk volunteering. The IPPF needs people to help us keep overhead low and mission success high. For example, during 2012 there were 64.5 million volunteers in the United States. The United States Red Cross’ workforce is 94% volunteers ( The Pacific Crest Trail Association ( has over 1,600 volunteers along the West Coast. Please consider volunteering with the IPPF (related stories p. 6 and 11).

Pemphigus and pemphigoid awareness has always been an issue, but that is changing. In 2012 we helped introduce a resolution in New Jersey. The Canadian Prime Minister of Finance announced he had pemphigus vulgaris. Marc Yale visited Washington D.C. and spoke with members of the House and Senate. And our Awareness Campaign began to develop.

To help the IPPF with the Awareness Campaign, please join me in welcoming our new Awareness Program Manager, Kathryn Fraser, to our family. Kathryn is working with the Awareness Campaign Committee to develop next steps and prepare for a Best Practices Consensus Conference in Spring 2014. Along the way, Kathryn will provide updates right here in the Quarterly and online.

As our pemphamily grows, we remain close to one another online and in person. Family. That’s what we are.

What is cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. It is also called benign mucous membrane pemphigoid or oral pemphigoid. Areas commonly involved are the oral mucosa (lining of the mouth) and conjunctiva (mucous membrane that coats the inner surface of the eyelids and the outer surface of the eye). Other areas that may be affected include the nostrils, oesophagus, trachea and genitals. Sometimes the skin may also be involved where blistering lesions can be found on the face, neck and scalp.

Brunsting Perry cicatricial pemphigoid is a rare variant in which localised crops of recurrent blisters arise within urticarial plaques, usually on the head and neck. The blisters may burst resulting in blood-crusted plaques and scars.

Who gets cicatricial pemphigoid?

Cicatricial pemphigoid is predominantly a disease of the elderly with a peak incidence at around 70 years. However, childhood cases have been reported. It appears to be twice as common in women than men.

What are the signs and symptoms of cicatricial pemphigoid?

Site Features
  • Sensation of grittiness or pain
  • Conjunctivitis
  • Lesions form, erode and heal to leave scar tissue
  • May lead to impaired vision or blindness
  • Blisters form first on the gums near the teeth
  • Palate, tongue, lips, buccal mucosa, floor of the mouth and throat may be affected
  • Painful and make it difficult to eat
  • Lesions occurring in the throat (oesophagus, trachea and larynx) can become life-threatening
  • Blisters on the skin develop in 25-30% of patients
  • May be itchy
  • Bleeding may occur if traumatised
  • Nose bleeds after blowing the nose
  • Crusting causing discomfort
  • Painful blisters and erosions on the clitoris, labia, shaft of the penis, perianal area

What causes cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune blistering disease, which basically means that an individual’s immune systems starts reacting against his or her own tissue. In this particular instance autoantibodies react with proteins found in mucous membranes and skin tissue resulting in blistering lesions. The binding site appears to be within the anchoring filaments that help the epidermis (outside layer of skin) stick to the dermis (inner layer of skin).

Full article from DermNet NZ


The relationship between bullous pemphigoid (BP) and neurological disease has been the subject of numerous recent studies and BP antigens and their isoforms have been identified in the central nervous system (CNS). Whilst epidemiological data support this association, little is known about the pathomechanism behind this link and the immunological characteristics of patients with BP and neurological disease, other than multiple sclerosis (MS), has not been studied. We aimed to compare the cutaneous immune response in BP patients with and without neurological disease, to investigate whether or not there is a distinctive immunopathological profile in patients with concomitant BP and neurological disease. Seventy-two patients with BP were included and divided into two groups; those with neurological disease (BP+N, n = 43) and those without (BP−N, n = 29).

Patients in BP+N group had a confirmed neurological disease by a hospital physician, neurologist or psychiatrist with positive neurological imaging where appropriate, or a Karnofsky score of 50 or less due to mental impairment. All sera were analysed with indirect immunofluorescence (IIF) using serial dilutions up to 1:120000, immunoblotting (IB) and Enzyme-linked immunosorbent assay (ELISA) for BP180 and BP230. Median antibody titres by IIF were 1:1600 vs. 1:800 for BP−N and BP+N, respectively, although the difference did not reach statistic significance (P = 0.93, Mann–Whitney U-test).

ELISA values for both BP180 and BP230 did not differ significantly between the two groups. Similarly, autoantibodies to specific antigens as identified by ELISA and IB were not related to the presence of neurological disease. The results of this study indicate that patients with BP and neurological disease exhibit an immune response to both BP180 and BP230, thus the link between the CNS and the skin is not dependent on a specific antigen, but possibly both antigens or their isoforms may be exposed following a neurological insult, and play a role in generation of an immune response. 

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