Category Archives: Issue 54 – Fall 2008

It’s the dog days of summer here in Sacramento, the two weeks in August where our temperatures rise above 100 day after day and the tomatoes drown the garden. By the time you get this we will have a break in the weather; then the long low lines of sunshine signaling Fall, will touch us all.

This is the last issue of the IPPF Newsletter before the November election. Please register to vote. I know I talk about this often, but I cannot emphasize enough how important it is to vote and hold our elected officials and ourselves accountable. This election will decide the turn which the country will take for the next four years. Since our healthcare system is overburdened and costs have skyrocketed, one of the big issues being discussed is how best to fix our broken healthcare system.

Vote! Personally, this issue is high on my agenda in deciding which person I will vote for. I also encourage you to check out the health plans of the two candidates. They are very different. You can find their plans on their websites. For John McCain’s health program visit; for Barack Obama’s plan visit But just don’t believe what they say, read descending opinions on both sides.

My level of concern is about making sure that everyone who has pemphigus or pemphigoid gets immediate coverage whether they have health insurance or not. I want the costs to be reasonable and accessible.

Although many of us have had insurance when we were first diagnosed, there are numbers of patients who do not. There are also numbers of patients who have lost their insurance because they lost their jobs for one reason or another. COBRA will cover people for a while, but the premiums are often exorbitant. If someone loses their job, what opportunities are there for a person living with a chronic illness to be eligible for employment especially if they have active disease and are over 40 Of course disability is an option, but you have to make sure that you fight like hell for it because it just isn’t that easy to get especially if you get rejected the first time. Sometimes it can take years.

For those who are doing well with insurance plans, I want you to consider the issues that all of us face with our health care needs when you vote. I have voted in every Presidential election since I was able to vote. I consider it a privilege to be in a country that allows us to pick the people we think would make our best leaders especially when, still, in this 21st Century, there are men and especially women unable to make these kinds of choices. Don’t take your vote for granted. Your vote does count! So, please, if you are not yet registered, Register! And, don’t forget to vote in November.


Recent articles in the medical literature will be of interest to our readers. A Letter to the Editor in the April 2008 issue of the Journal of Ophthalmology discussed a preliminary study from Italy of intravenous immunoglobulins (IVIg) for the treatment of Mucous Membrane Pemphigoid (MMP). In it, 6 patients with severe MMP were treated with IVIg for 8 to 20 months.

Ed Tenner MD All responded to this treatment with healing of mouth lesions and stoppage of eye inflammation and scarring. As the treatments went on and symptoms decreased, steroids and immunosuppressants were slowly reduced and the time between IVIg cycles was lengthened. There were side effects but all were mild. They will continue to follow these patients as IVIg is discontinued. The authors also called for a larger study to confirm their findings. This study seems to indicate IVIg works in MMP as it has been found to work in pemphigus patients.

The journey for patients and caregivers trying to get diagnosed, educated and treated for pemphigus or pemphigoid is difficult. The June 14, 2008 issue of The Lancet has articles, and an editorial on rare diseases and orphan drugs. One article indicates, though not by name, that pemphigus and pemphigoid are probably best defined as ultra-rare. Titles in the issue included:

  • Making rare diseases a public-health and research priority;
  • Why rare diseases are an important medical and social issue;
  • Empowerment of patients: lessons from the rare diseases community; and
  • Does orphan drug legislation really answer the needs of patients.

As you can see these are all topics that involve the pemphigus and pemphigoid community. The fact that a major medical journal is committing so much space to these problems is good news. However, progress is slow and costly.

The National Organization of Rare Disorders (NORD) is discussed. The IPPF is a member of this group. Eurordis is a similar organization for Europeans with rare diseases and Orphanet is a resource for rare disease web sites. The articles show that patients and their organizations like the IPPF have moved the general medical community to some action. They have also moved legislative and pharmaceutical companies to pursue orphan drug legislation. Because the accumulative number of people with rare disease is substantial, it is pointed out that treating and helping these people benefits the whole community. However much remains to be done.

In conclusion, I would like to quote from the lead editorial:

There are encouraging signs that rare diseases are being included in public-health plans, and that more research is being directed in finding the causes of and treatments for at least some of these conditions. Patients with rare diseases have the right to first class medical knowledge, rapid diagnosis, research, and treatment [emphasis added]. In reality, though, their lives are often blighted by medical ignorance, lack of professional advice, delayed diagnosis, and no treatment options at all. Patients should not have to depend on their families to find potential treatments for rare diseases.

First I want to apologize for not having time to answer all the questions at the Dallas Conference in April. I answered additional questions which were emailed to me in the aftermath. If you still have one, please let me know.

One particularly intriguing comment after my presentation was: It is what it is. Deal with it. Move on. We all need to find ways to deal with it and move on. Making adjustments and learning more positive coping strategies are a large part of where the mental health piece (of the puzzle) fits.

{quotes}When diagnosed with a serious and life threatening physical illness, no one expects patients to physically heal themselves, accept it, and just move on.{/quotes} Why the double standard with emotional and psychological difficulties which accompany these types of illnesses The challenges of dealing with the illness and necessary treatments are not existential ones for the patient or caregivers/family.

Instead of talkin about problems, I will devote the rest of this column to walking the walk. Everyone will recognize themselves in some of the following common cognitive distortions as per Dr’s Albert Ellis and David Burns:

  1. All or none thinking: Seeing things in extremes with no shades of grey.
  2. Overgeneralization: Interpreting a single negative event as endless defeat.
  3. Mental Filter: Picking out negative details and focusing on them to the exclusion of other aspects of the situation.
  4. Disqualifying the Positive: Positive or successful experiences are rejected or belittled, which maintains a negative view of the self.
  5. Mind Reading: Jumping to a negative conclusion rapidly without bothering to check out the facts. Assuming negative things apply to you when they don’t (Personalization).
  6. Magnification (Catastrophizing and Minimization): Magnifying things that will make you feel worse (e.g., your faults or physical symptoms) and minimizing things that will make you feel better. This is also known as the binocular trick.
  7. Emotional Reasoning: Assuming that negative feelings reflect facts about the way things actually are.
  8. Should Statements: Using excessive, harsh and unreasonable commands and self-punishing thoughts.
  9. Labeling and Mislabeling: Instead of simply describing a situation or behavior, you attach a negative label to yourself. Mislabeling involves using an incorrect label that incorrectly attaches greater danger to the situation.
  10. Magical Thinking: Perceiving cause and effect relationships when they don’t exist or wishing for things rather than working for them.

It is helpful to read through these cognitive distortions several times to make sure you understand them and the subtle differences between them. You may want to circle a few that you believe you do in excess. Once you have done this, you will find it helpful to write down examples of how you may use these distortions. Identifying the distortions properly, and understanding how you may use them, will give you insight into your thinking and behaviors. We all use some of these at times, so try to focus on the ones which clearly stand out for you. This may help you manage your thoughts when they are difficult.

For more suggestions and exercises, please visit Try the exercises there over time, to evaluate how you tend to think about emotionally charged and/or unpleasant situations, events or interactions. You will learn more about your particular style and cognitive/emotional process. In turn, this knowledge will allow you to evaluate yourself and others more objectively. The goal is to learn to respond more rationally versus reacting emotionally. As always, your comments, questions, and topics for future columns are welcome.

It is what it is...

One of the newest treatments for pemphigus is intravenous immunoglobulin (IVIG). Immunoglobulins are one of the major classes of proteins in blood. IVIG is collected from normal blood donors, pooled, highly purified, and treated to destroy viruses and bacteria. IVIG is now used to treat a number of autoimmune diseases including pemphigus.

There are two particularly attractive features of IVIG as a treatment for pemphigus:

  1. It can rapidly control active pemphigus without resorting to increasing steroid doses, in many but not all patients.
  2. It is unique in being able to selectively decrease blood levels of pemphigus antibodies (the antibodies that cause the disease) without lowering the level of normal antibodies. This feature of IVIG is very desirable, and unique, as all other treatments for pemphigus interfere with the production of all antibodies — the good along with the bad — resulting in unwanted side effects.

IVIG appears to work by speeding the degradation or inactivation of all antibodies in blood  the good with the bad. Then, the normal antibodies are replaced by those present in the IVIG that is administered, whereas abnormal antibodies are absent in IVIG and so they are not replaced — only they remain reduced following IVIG.

Complicating this story is that a regulatory mechanism in the body maintains constant levels of each individual antibody in the blood. Decrease in blood level of any antibody (including the pemphigus ones) stimulates new production of that antibody, and a rebound in their levels in the blood. Thus, even though IVIG can decrease serum level of pemphigus antibodies, these will go right back up shortly after the procedure.

This rebound can be minimized, in animals, by administering a cytotoxic drug that blocks the cells that make new antibodies. This approach has been used in humans to improve the effectiveness of plasmapheresis, another procedure that lowers serum level of antibodies. Similarly, this approach should also improve the effectiveness of IVIG.
[box type=”orange”]Antibody: A protein produced by a B cell in response to a specific foreign substance. Antibodies are the ?soldiers? which protect us against bacteria and viruses and infections.

Cytotoxic drugs: Affect the growth and action of some cells that cause the joint pain, swelling, warmth, and damage of arthritis. Cytotoxic drugs work over a long period of time, however, patients may not notice much effect for the first several weeks or months of treatment.

Immunoglobulins: See Antibody

Plasmapheresis: Removal, treatment, and return of blood plasma from blood circulation.

* more definitions available here:[/box]

Editors Note: This is an excerpt from a letter we received from the daughter of a patient expressing her gratitiude for the Foundation and its efforts. We welcome letters like this – and letters for any reason – by postal mail or email and will gladly reprint them with your permission.

Enclosed is my donation in memory of my dear mother Phyllis Sigman who passed away 2002 from all the therapies and drugs as well of lack of knowledge of Bullous Pemphigoid. These brutal years of coming out of the shock and awe campaign that I witnessed from her disease have truly been the most challenging of my life.

{quotes}I don’t know why there is not a spokesperson in every single town in this country (to) educate the public about these horrifying and debilitating Pemphigoid diseases.{/quotes} It is an atrocity on the behalf of the medical community that this is not more mainstream. My mother suffered 6 different misdiagnoses from 6 different dermatologists in Arizona. I have shared this with people and told them to find out where they can go (to learn) about Pemphigus, including your Foundation.

It is a matter of life and death in some cases, as I found out the hard way. I appreciate what the Foundation is working to do and I also think that all dermatologists should not only be well educated in this disease but that the medical community should take this more seriously in every small town in this country.

I just want the Foundation to know that those of us who have lost our loved ones to the ravages of these diseases and the lack of interest or funding are never forgotten. Whenever I have a couple of extra dollars I will always support the Foundation. God Bless you all for what you are doing and I pray that no other families have to suffer the frustrations and desperation that my family had to endure. My mother was an extraordinary trooper through her 3 month battle, and perhaps if the big wheels who make the rules funded and took this more seriously she might be here today. God only knows.

Very Best Regards,

Diane Slocum

[box type=”orange”]

Dear IPPF,

Thanks for all you are doing for this condition. I am a remission patient. If it had not been for the Dermatology Department at the University of Michigan I would be DEAD!! Before them, I spent 15 days in a hospital where no one ever heard of PEMPHIGUS.

I arrived at the UofM I was met by 4 Doctors & 3 nurses. They knew where they were going and what I needed. But, they gave me little hope because early intervention is the answer. I spent nearly 2 months there.

Today I am scarred and bruised but I AM ALIVE! If anyone is having a hard time finding treatment-please tell them my story of hope, persistence, remission! Unless you have been there you cannot imagine that hopelessness!! Bless you & all who are suffering.

Wanda Duncan



When I was first diagnosed with PV, I was devastated as most people are. It had come on very fast, three weeks from first symptom, and very viciously. I could not eat any solids, I could not stand — due to huge blisters right across both insteps — and I could not get comfortable in bed due to blisters covering 70% of my body.

The IPPF website redesign continues to bring in compliments and comments from around the world. The IPPF Community site is where people interact with other patients and caregivers on all issues related to pemphigus and pemphigoid. You can register for FREE at

There have been two key challenges to raising awareness and funds about the devastating effects of pemphigus/pemphigoid — to front-line medical professionals, donors, and corporate sponsors. In the early stages people tell me they feel ashamed, embarrassed, and frustrated when they explain this disease to others. A very courageous and accomplished woman said to me recently, No one here who knows me knows that I have this disease.

The other challenge is that once people have achieved a level of maintenance, or even remission, they no longer want to reflect on some of the darkest and most terrifying days of their life.

And yet, telling these stories is probably the most powerful way to move people to action and inspire people to support the development of resources, information, and perhaps even cures. Recently, people are speaking up about these trying times, and reaching out to educate others or to extend a helping hand to those just beginning this horrific journey.

If you haven’t yet, please take the time to go to our web site at and listen to Rebecca’s interview with Richard Cohen on WABC.

Opportunities like this national radio broadcast, stories in local newspapers, and presentations to relevant groups such as dermatologists and dentists help us spread awareness, and make an early and effective diagnosis of P/P possible. {quotes align=right}Personal stories also raise awareness of philanthropists and good social citizens who want to make a difference for people who are suffering.{/quotes}

Since I have been a part of the IPPF, many people have shared with me their stories of tragedies and triumph. These stories provide the incentive and the inspiration for donors, corporate citizens, and medical professionals to work to make a difference.

We thank all of the courageous people, such as Rebecca, who share their stories in order to help others, and honor those who suffer with P/P alone, or in silence.