Tag Archives: pemphigus

Todd Kuh and Marc Yale

On September 26th Todd Kuh and Mariah Lowinske, two patient advocates with Pemphigus Vulgaris, and I attended the American Academy of Dermatology Legislative Conference in Washington DC. As a member of the Coalition of Skin Diseases, the IPPF sends advocates to this conference annually so we can learn about the latest initiatives prior to lobbying Congress for support for treatment and research.

In a previous Coaches Corner I have given prednisone tips. It is a good time now for an update as there are new patients who are diagnosed and who are leery of the side effects that can come from taking a steroid treatment.

No one chooses to take prednisone as a medication.  However, it is used frequently for a variety of medical conditions, like pemphigus and pemphigoid (P/P). Prednisone is often used as the first line of defense against P/P. It works rather quickly and is effective in diminishing disease activity.

To learn more about prednisone, how it is used, what precautions to take before taking it, dietary suggestions, and side effects please go to this link from the U.S. Library of Medicine: http://www.nlm.nih.gov/medlineplus/druginfo/meds/a601102.html

A few of the side effects from taking prednisone can be:

Headache, dizziness, difficulty, falling asleep or staying asleep, extreme changes in mood, changes in the way fat is spread around the body, extreme tiredness, weak muscles, and more.

Some side effects can be serious. If you experience any of the following symptoms, call your doctor immediately:

vision problems, eye pain, redness, or tearing, sore throat, fever, chills, cough, or other signs of infection, depression, upset stomach, lightheadedness, shortness of breath(especially during the night), swelling of the eyes, face, lips, tongue, throat, arms, hands, feet, ankles, or lower legs, difficulty breathing or swallowing.

Please note that not everyone experiences every side effect and that even taking treatments like aspirin can come with side effects.  Everyone has his or her own unique physiological makeup. Therefore, while experiences with prednisone may be similar, they are not exactly alike.

Remember, when you need us we are in your corner!

After months of uncertainty waiting for a diagnosis and finally finding a physician who can treat your rare skin disease, you return from the mailbox with a denial letter from your insurance company. After the shock and frustration has subsided you are now faced with the decision of whether it is worth appealing this insurance determination or should you start back at “square one”.

Chances are that the insurance company is counting on you not appealing but according to the U.S. Government Accountability Office, one report revealed that in the four states that tracked such data, 39 to 59 percent of private health insurance appeals resulted in reversal. Those are pretty good odds!

Here are some tips on how to appeal:

1.      Read the denial letter carefully. Make sure that you learn about your insurer’s appeals process. In the coverage documents and summary of benefits, insurance companies are required to give all the tools needed to properly make an appeal. There are often deadlines to meet, so act quickly and send it to them as soon as possible.

2.      Ask for help from your doctor. Check the medical policy and ask your doctor to review it to prepare something called a letter of medical necessity to support your case. If your doctor is not able to help, be prepared to handle it yourself. You are your best advocate!

3.      Contact the IPPF. The foundation can help provide you with resources about the disease and publications citing the use of treatments used for your condition. These documents can help support your case to the insurance company.

4.      Write a testimonial letter. You have a rare disease and it is likely that the person making the decision knows nothing about your disease. A letter with pictures detailing your story and exactly what happened will make it personal. Tell them that you are seeking approval for treatment, note any supporting science, clinical evidence, expected benefits, etc. Be clear, firm and concise. Make it clear that you plan to pursue the appeal until it is resolved and is approved.

5.      Follow up. Many appeals take weeks, even months, so call often to check the status and take notes of each call. When you speak to the insurance company, write down the time and date, length of the call, the name and title of the person you speak with and all the details of the conversation. Make note of any follow-up activities and next steps to be taken.

Remember, many insurance companies have a tiered appeals process. The first level is processed by the company’s appeals staff or medical director responsible for the denial. Second-level appeals are reviewed by a medical director not involved in the original claim. The third level involves an independent, third-party reviewer. If your insurance company continues to deny the claim; you can then take the appeal to your state’s insurance department, state insurance commissioner or even your local legislators who have staff to assist you.

This process may seem overwhelming but it is worth it. Your health and the health of other pemphigus and pemphigoid patients may be impacted by the awareness you create with the insurance claim.

If you need assistance, just “Ask a Coach!” Remember, when you need us, we are in your corner!

Dear pemphigus & pemphigoid Community,

As you know, the IPPF and the rare disease community have been working with Congress to advance the 21st Century Cures Act (HR 6). This legislation contains billions in new funding for research along with critical incentives to develop treatments. In July, the 21st Century Cures Act passed the House with wide bipartisan support and it is now up to the Senate to pass their own version of the legislation, called “Innovation for Healthier Americans“. Unfortunately, the Senate initiative has stalled and we are concerned that additional delays could derail the entire bill.

The IPPF is partnering with patient advocacy groups across the country to hold a day of action to call the Senate and urge them to prioritize Innovation for Healthier Americans – a delay in legislation is a delay in treatment for patients.

We are asking you to call you Senators today to convey this message and to let them know that patients can’t wait! Thank you for your all of your support and for taking action. The entire pemphigus & pemphigoid is counting on you!

Please use this sample script to help you with your call and the attached list to contact your Senators.

Sample Script:

My name is XXXX from [your city/state] and I represent the [IPPF and/or pemphigus or pemphigoid].

[Briefly explain the IPPF and/or your disease].

I’m calling today to urge Senator XXXX to prioritize Innovation for Healthier Americans. Its companion legislation in the House includes vital funding for the NIH & the FDA and incentives that will dramatically boost the number of treatments for rare disease patients.
Please support the passage of the Innovation for Healthier Americans legislation this year. A delay in legislation is a delay in desperately-needed treatments. Patients simply cannot wait. [Provide your contact information if you wish for a response from your Senator].
Thank you so much for your consideration and your leadership.

*Senate phone numbers are listed below:

Alabama                SHELBY, Richard @SenShelby (202)224-5744                     SESSIONS, Jeff @SenatorSessions (202)224-4124

Alaska                     SULLIVAN, Dan @sendansullivan (202)224-3004                *MURKOWSKI, Lisa @lisamurkowski (202)224-6665

Arizona                  FLAKE, Jeff @JeffFlake (202)224-4521                                  McCAIN, John @SenJohnMcCain (202)224-2235

Arkansas                BOOZMAN, John @JohnBoozman (202)224-4843              COTTON, Tom @SenTomCotton (202)224-2353

California               BOXER, Barbara @SenatorBoxer (202)224-3553                FEINSTEIN, Dianne @SenFeinstein (202)224-3841

Colorado                *BENNET, Michael @SenBennetCO (202)224-5852           GARDNER, Cory @SenCoryGardner (202)224-5941

Connecticut          BLUMENTHAL, Richard @SenBlumenthal (202)224-2823 *MURPHY, Chris @SenMurphyOffice (202)224-4041

Delaware               CARPER, Thomas @SenatorCarper (202)224-2441             COONS, Christopher @SenCoonsOffice (202)224-5042

Florida                    NELSON, Bill @SenBillNelson (202)224-5274                       RUBIO, Marco @SenRubioPress (202)224-3041

Georgia                  *ISAKSON, Johnny @SenatorIsakson (202)224-3643        PERDUE, David @sendavidperdue (202)224-3521

Hawaii                    HIRONO, Mazie @maziehirono (202)224-6361                   SCHATZ, Brian @SenBrianSchatz (202)224-3934

Idaho                      CRAPO, Mike @MikeCrapo (202)224-6142                         RISCH, James @SenatorRisch (202)224-2752

Illinois                    *DURBIN, Richard @SenatorDurbin (202)224-2152          *KIRK, Mark @SenatorKirk (202)224-2854

Indiana                   COATS, Daniel @SenDanCoats (202)224-5623                    DONNELLY, Joe @SenDonnelly (202)224-4814

Iowa                       ERNST, Joni @SenJoniErnst (202)224-3254                          GRASSLEY, Chuck @ChuckGrassley (202)224-3744

Kansas                    MORAN, Jerry @JerryMoran (202)224-6521                      *ROBERTS, Pat @SenPatRoberts (202)224-4774

Kentucky               *McCONNELL, Mitch @SenateMajLdr (202)224-2541      *PAUL, Rand @RandPaul (202)224-4343

Louisiana               *CASSIDY, Bill @BillCassidy (202)224-5824                         VITTER, David @DavidVitter (202)224-4623

Maine                     COLLINS, Susan @SenatorCollins (202)224-2523                KING, Jr., Angus @SenAngusKing (202)224-5344

Maryland               CARDIN, Benjamin @SenatorCardin (202)224-4524           *MIKULSKI, Barbara @SenatorBarb (202)224-4654

Massachusetts      MARKEY, Edward @SenMarkey (202)224-2742                  *WARREN, Elizabeth @SenWarren (202)224-4543

Michigan                PETERS, Gary @SenGaryPeters (202)224-6221                   STABENOW, Debbie @SenStabenow (202)224-4822

Minnesota             *FRANKEN, Al @alfranken (202)224-5641                          KLOBUCHAR, Amy @amyklobuchar (202)224-3244

Mississippi             COCHRAN, Thad @SenThadCochran (202)224-5054          WICKER, Roger @SenatorWicker (202)224-6253

Missouri                 *BLUNT, Roy @RoyBlunt (202)224-5721                             McCASKILL, Claire @McCaskillOffice (202)224-6154

Montana                DAINES, Steve @SteveDaines (202)224-2651                      TESTER, Jon @SenatorTester (202)224-2644

Nebraska               FISCHER, Deb @SenatorFischer (202)224-6551                  SASSE, Ben @SenSasse (202)224-4224

Nevada                  HELLER, Dean @SenDeanHeller (202)224-6244                 *REID, Harry @SenatorReid (202)224-3542

New Hampshire   AYOTTE, Kelly @KellyAyotte (202)224-3324                       SHAHEEN, Jeanne @SenatorShaheen (202)224-2841

New Jersey           BOOKER, Cory @CoryBooker (202)224-3224                      MENENDEZ, Robert @SenatorMenendez (202)224-4744

New Mexico         HEINRICH, Martin @MartinHeinrich (202)224-5521           UDALL, Tom @SenatorTomUdall (202)224-6621

New York              GILLIBRAND, Kirsten @SenGillibrand (202)224-4451         *SCHUMER, Charles @SenSchumer (202)224-6542

North Carolina     *BURR, Richard @SenatorBurr (202)224-3154                   TILLIS, Thom @SenThomTillis (202)224-6342

North Dakota       HEITKAMP, Heidi @SenatorHeitkamp (202)224-2043        HOEVEN, John @SenJohnHoeven (202)224-2551

Ohio                       BROWN, Sherrod @SenSherrodBrown (202)224-2315     PORTMAN, Rob @PortmanPress (202)224-3353

Oklahoma              INHOFE, James @jiminhofe (202)224-4721                          LANKFORD, James @SenatorLankford (202)224-5754

Oregon                  MERKLEY, Jeff @SenJeffMerkley (202)224-3753                WYDEN, Ron @RonWyden (202)224-5244

Pennsylvania         *CASEY, Jr., Robert @SenBobCasey (202)224-6324          TOOMEY, Patrick @SenToomey (202)224-4254

Rhode Island         REED, Jack @SenJackReed (202)224-4642                     *WHITEHOUSE, Sheldon @SenWhitehouse (202)224-2921

South Carolina      GRAHAM, Lindsey @GrahamBlog (202)224-5972               *SCOTT, Tim @SenatorTimScott (202)224-6121

South Dakota        ROUNDS, Mike @SenatorRounds (202)224-5842               *THUNE, John @SenJohnThune (202)224-2321

Tennessee             *ALEXANDER, Lamar @SenAlexander (202)224-4944      CORKER, Bob @SenBobCorker (202)224-3344

Texas                      *CORNYN, John @JohnCornyn (202)224-2934                   CRUZ, Ted @SenTedCruz (202)224-5922

Utah                      *HATCH, Orrin @SenOrrinHatch (202)224-5251                LEE, Mike @SenMikeLee (202)224-5444

Vermont               *SANDERS, Bernard @SenSanders (202)224-5141            LEAHY, Patrick @SenatorLeahy (202) 224-4242

Virginia                  KAINE, Tim @SenKaineOffice (202)224-4024                      WARNER, Mark @MarkWarner (202)224-2023

Washington          CANTWELL, Maria @SenatorCantwell (202)224-3441       *MURRAY, Patty @PattyMurray (202)224-2621

West Virginia        CAPITO, Shelley Moore @SenCapito (202)224-6472         MANCHIN III, Joe @Sen_JoeManchin (202)224-3954

Wisconsin              *BALDWIN, Tammy @SenatorBaldwin (202)224-5653    JOHNSON, Ron @SenRonJohnson (202)224-5323

Wyoming               *BARRASSO, John @SenJohnBarrasso (202)224-6441      *ENZI, Michael @SenatorEnzi (202)224-3424
*indicates Senator is a Member of the Senate Health, Education, Labor, & Pensions (HELP) Committee or holds a leadership position


Pemphigus and pemphigoid (P/P) can take a long time to improve or reach remission. The process can take months or even years. Although it may seem that this disease appeared overnight, in fact, it has taken a long time to present itself, and it will most likely take just as long to resolve. So the question often arises, “How do I know if I am improving?” Improvement can be measured in a couple ways, so here are some tips to measure whether your disease is improving:

1.      Keep a log of disease activity – It’s easier to measure if you can see it on paper.

2.      Amount – Count the number of blisters, their location and write them in your log. If you have less blisters than before then you are improving.

3.      Frequency – Take note of the blisters and how long they take to resolve. If they are clearing up quicker than before then it is showing improvement.

4.      Fatigue – Pemphigus and Pemphigoid cause fatigue. A clinical sign of improvement should be less overall fatigue.

5.      Talk to you Doctor – Ask your doctor what signs they look for that indicate improvement.  Working together with your physician to reach remission should be your biggest priority.

Improvement is a step-by step process and can be slow. If you follow these tips you may just find that you are improving, even if it’s just a little at a time. Knowing that you are improving will help you realize that you are in control and on the road to remission.

Have other questions about measuring improvement? Just “Ask a Coach! Remember, when you need us, we are in your corner!

The main physical manisfestation of the P/P diseases is the presence of blisters on the skin and mucous membranes. Underlying those blisters are numerous molecular processes including recognition of keratinocyte cells of the skin and cell death. But how these blisters actually form, that is, what is the order of events leading up to their formation, has not been clear. A recent study by scientists Parviz Deyhimi and Payam Tavakoli suggests that in pemphigus vulgaris (PV), cell death comes first, then the formation of blisters (Journal of Oral Pathology and Medicine, doi: 10.1111/jop.12022).

The blisters that form in PV are referred to as lesions, or suprabasal vesicles, because of where they are found within the layers of the epidermis (supra meaning above, so above the basal layer, see Figure 1a). Because they are found so deep within the tissue, the blisters formed and PV disease itself is considered more severe than pemphigus foliaceus, where the blisters appear within the granular layer. The lesions formed during PV and in other mucocutaneous autoimmune blistering diseases are formed when the rogue antibodies formed during disease recognize proteins found at junctions formed by keratinocyte cells interacting with one another. The loss of these junctions that generates the tear in the skin is called acantholysis. Acantholysis is more than a tearing of the skin.

There is also cell death (also called apoptosis) within the lesions. But it has been unclear when and where apoptosis occurs with relation to acantholysis and to recognition of the junctions by antibodies generated by the immune system of the patient. Besides the ordering of events, it has been unclear which of the various types of apoptosis are at play. In the intrinsic pathway of apoptosis, a cell essentially commits suicide because of an internal trigger, perhaps as part of a genetic program as occurs during cell or tissue development. In the extrinsic pathway, the trigger to commit suicide is external. Perhaps this is where the antibodies of PV patients play a role, then? At least two models, both with excellent experimental support, exist for the ordering of events.

The first suggests that apoptosis is a late event in pemphigus and that it is not required for acantholysis and blister formation, while the second suggests that apoptosis occurs early, before significant acantholysis. A related viewpoint to the second is that the two occur simultaneously, though independently, though evidence exists for apoptosis actually causing acantholysis. For instance, chemical inhibitors of apoptosis have been shown to prevent lesion formation and a time-course study has shown that apoptotic cells were present before blisters in pemphigus foliaceus. The current authors looked at tissue samples from 25 patients with oral lesions due to PV. They used immunohistochemistry, the same technique that is used to diagnose PV.

Looking closely for regions where normal lesion-free tissue was adjacent to lesions, so-called peri-lesional regions, they found that 100% of the cells within lesions had fragmented DNA, the hallmark of apoptosis. In the adjacent normal tissue (in the parabasal region) of most of the samples, 75% of the cells had the marker of apoptosis. Looking at the acantholytic cells within the lesion, the result was strikingly close to 75%, at 76% and at the roof of the vesicle, it was even higher, at 80%. Given the presence of apoptotic cells in the lesion-free patient tissue, the authors concluded that apoptosis is not a late event, but an early one that may cause acantholysis. Recognizing that the structural damage (acantholysis) and death (apoptosis) of keratinocytes are mediated by the same molecular players – the caspase enzymes.

Research led by Sergei Grando has proposed a novel theory of“apoptolysis”, combining the two terms. The work of Deyhimi and Tavakoli supports this model and suggests that once a threshold level of apoptotic cells exist in the basal cell layer, somewhere north of 80%, then a lesion will form. According to the authors, conventional therapy of PV consisting of high-dose corticosteroids is based on the hypothesis that acantholysis leads to apoptosis, so it will be critical to unravel the current results and to determine if treatments might be tailored differently in the future. How apoptosis leads to formation of blisters and how antibodies to desmogleins may promote apoptosis is still under investigation, but one additional piece of information from the current work is that based on the absence of another cell death marker, Bax, the authors suspect the extrinsic cell death pathway.

The pieces to the pemphigus puzzle are beginning to be unraveled. Driven by the fact that the more we learn about the molecular events leading up to blisters, the more chances there will be to intervene before debilitating blisters can occur.

This article is meant for those of you who have recently or about to move to a new area. After living in the greater Seattle area for 58 years, I moved to the San Francisco Bay area in August ‘14.

I encountered more challenges that I had expected. I had to find new medical insurance, since the company that I was with in WA isn’t licensed to cover in California. I also had to find a new dermatologist and general practitioner.
First, I had to decide on what kind of insurance to get. In WA I was with a company for around four years. They were much like Kaiser in the west. You can only go to Kaiser providers, labs and prescription services. I was used to this system, however it felt restrictive. The other option was to pick an insurance company that was a PPO.

Finding a GP was probably the hardest, in the sense that I felt a bit like I was shooting in the dark. My insurance agent unofficially suggested I look for a doctor within the John Muir system. They are close, and generally good. It was much easier to find a dermatologist, because I already knew who I wanted. When moving though you should always contact the IPPF and have them re-send you the physician’s referral list. This way you can see the doctors in your new area.

I was familiar with Dr. Peter Marinkovich at Stanford Medical Center. He is on the medical advisory with the IPPF and I have heard him speak before. I felt great about working with him, and his staff. It’s over an hour drive away from me, but worth it.
I have a few suggestions to keep in mind. If you are moving due to a job change, or your spouse’s, then I believe you are more restricted if you don’t want to self-pay. Read up on what kind of choices you have for a dermatologist in your area. Talk to your insurance company’s customer service if you have any questions regarding coverages. Find out how much experience the dermatologist has treating pemphigus/pemphigoid. In addition to that, it’s important to be able to feel connected, and your doctor understands you.
If you need help finding a dermatologist, feel free to contact Noelle Madsen at the IPPF office. She can make some recommendations, as well as send you a list we have. She can be reached by phone at 916-922-1288 x105 or by email at noelle@pemphigus.org.
Remember, when you need us we are in your corner!

Having a rare disease like pemphigus or pemphigoid can make you feel like you have lost control of your life. After being diagnosed you may experience fear, confusion, and you may feel unclear about your future. You may even feel helpless, vulnerable, and at the mercy of others. Your self- esteem may be compromised along with your immune system. The anxiety or stress that can bring may be overwhelming. The good news is that these feelings are normal and more importantly that you truly do have the power to stay in control!

Here are a few tips on how to keep calm and stay in control:

1.      Offer help to others – this will help you keep your disease in perspective

2.      Find the “silver-lining,” in every situation – there is always something positive to gain

3.      Respect and accept that you can’t control everything – the sooner you realize this, the better!

4.      Don’t be a victim, be a contender!

5.      Be proud of yourself – every day is a step in the right direction

6.      Learn about your disease – knowledge is power

7.      Be self-determined – you are the master of your fate

8.      Speak up for yourself – being heard builds affirmation

9.      Consider how your disease will help you grow as an individual

10.  Don’t be afraid to ask for help and support!

You are not in this alone and there are many patients that are experiencing the struggles that you are going through. If you reach out to others through the IPPF you will find that you really are in control and together with other patients like you we have the power to overcome this disease.

Not sure how to connect with others?  Just “Ask a Coach!” Remember, when you need us, we are in your corner!

Living with a bullous skin disease is a challenge in more ways than one.  In addition to taking medication we also need to be taking supplements, avoid certain foods and spices, take caution in how we move and bathe, and relax to reduce stress.

We also need to take extra precautions against the sun’s harmful rays…more so than the average person who does not have pemphigus or pemphigoid.

It isn’t always a sunny day that can bring the harmful rays.  Cloudy days can be deceiving – you can get your worst sunburns through the clouds.  Reflections from the water in swimming pools, lakes, oceans, etc. increase the harmful effects of the sun’s rays as well as the reflections from the snow when skiing.

Women also need to make sure that their foundation has an SPF ingredient – this was told to me by the national makeup advisor for Dior.  I never knew this!  But it does help…even if we don’t actively stay out in the sun and are just running around doing errands.

According to the American Academy of Dermatology:

“Dermatologists recommend using a sunscreen with an SPF block of at least 30, which blocks 97% of the sun’s rays.  Just make sure it offers a broad-spectrum (UVA and UVB) protection, an SPF of 30 or greater, and is water resistant.”

Before trying to figure out which brand is the best to buy, discuss with your dermatologist. He/she can make suggestions for you based on your skin’s level of activity.

Don’t forget your ears, too!  Ear lobes are very sensitive and need protection. For those of you with scalp involvement, it is best to consult with your dermatologist who will recommend what sunscreen products are best for the scalp.  Hats are advisable when venturing outside.  Solid hats…not straw as the sun’s rays will stream through the weaves and cause damage!  Take extra care with the “driver’s arm” — you know, your arm that is exposed to the sun when you’re driving?  The sun’s rays are intensified through the glass windows. Best to be sure you are either wearing long sleeves or extra sunscreen.  If you are going in the water, sunscreen needs to be reapplied often.

UV radiation also impairs the skin’s immune system in alarming ways. Sun exposure reduces the number of watchdog cells that help recognize and respond to antigens, and alters their function so they are as effective as dozing prison guards. “This effect on immune suppression can set in even before a sunburn,” Dr. Baron said. Reference: http://www.nytimes.com/2009/05/14/fashion/14SKIN.html?pagewanted=all&_r=0

Remember, when you need us we are always in your corner!

Studies have shown that there is a mind-body connection.  It is known that stress can cause headaches, muscle aches, tummy aches, and blisters!   For those affected by pemphigus and pemphigoid (P/P), when your stress levels are high, the antibodies know when it is time to come out and play.  Horrors!  It is easier said than done to remain calm and stress-free.

Have you experienced when you have a headache and you worry more about something and the headache gets worse?  That is an example of the mind-body connection.

The brain signals the immune system, and we never know until after it happens.  In order to ward off these signals, it is important for patients to remain even-minded.  HA!  You say!  You’re right!  However, there are ways for us to get better at this.  It just takes practice.

Meditation can be very helpful.  Even if only for 5 minutes at a time.  Never feel that you must sit in a lotus position and keep stiff for an hour!  That is not what meditation is about!

There are a lot of meditation videos on YouTube that you could take a look at.  Just type in the word “Meditation” and a lot of suggestions come up!

Breathing exercises are a great way to help keep your blood pressure down. They help to bring a sense of calmness to envelope you.  Essentially, you are inhaling slowly to a count of 10 or 20 and holding the breath for 10 or 20 and then exhaling slowly for 10 or 20.  When you exhale, think of a happy word (like an affirmation) ie: joy or peace.