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Our sixth story in the Patient Journey Series comes from Toby:

When I was diagnosed with pemphigus vulgaris (PV) in late 2010, like most patients I’d never heard of the disease. Also, like most, I’d endured about six months of worsening symptoms before finding a doctor who was able to help me. After starting me on medication, the next thing my dermatologist did was to tell me about the IPPF. He encouraged me to reach out to them if I needed support, but I shelved the idea for a long time because I was sure I could manage by myself. I had the mistaken idea that if I followed my doctor’s instructions, I would “get better,”and soon.

Having PV was a wake-up call for me, however, as the disease proved to be tenacious, unpredictable, and unmanageable in my case on prednisone and CellCept alone. I embarked on a series of rituximab infusions ⎯ four over the past eight years ⎯ which have helped to control my symptoms. But the most important part of that wake-up call was the realization that I couldn’t go it alone. I called the IPPF nearly a year after diagnosis and talked to a Peer Health Coach, and the cloud lifted right away. For a while I attended support group meetings in the New York area. It was good to meet others who were facing the same challenges I was.

When I moved to New Hampshire three years later, I researched dermatologists to find one who was familiar with PV. Lo and behold, one turned up at UVM Medical Center in Burlington, VT, who had worked with IPPF Medical Advisory Board Member Dr. Victoria Werth at the University of Pennsylvania and co-authored an article on blistering diseases. I emailed Dr. Werth and asked her if she recommended this doctor, and she sent a thoughtful, encouraging response. I remained under the care of that doctor for five years until this year, when I moved to North Carolina.

This was a lucky move in many ways. Last October I attended the IPPF annual conference in Raleigh, NC, co-hosted by Dr. Donna Culton at UNC’s School of Medicine, and she was incredibly knowledgeable and inspiring. Because the IPPF set up the conference to make it easy for patients, staff, and physicians to mix, I was able to have one-on-one conversations with anyone I liked. There were cocktail hours, meals, and time between presentations, and everyone was approachable. I sought out Dr. Culton to say hello and chat with her. Afterwards, I told my daughter Kate, who lives in the area and had attended the conference with me, that I could do worse than moving down to NC and having Dr. Culton as my dermatologist. Then I made it happen. Two weeks before I packed up my car and drove the 800 miles with my cat to my new home, I had an appointment with Dr. Culton on the calendar!

Having excellent care and support (and even an infusion center) right here in my neighborhood gives me a sense of security and well-being, and I can trace that support directly back to the IPPF, who helped me find the way. Thank you, IPPF!

Give your share to show you care. Support patients like Toby,

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Every day, our patient services team hears stories from our community about what it’s like to live with pemphigus and pemphigoid. From getting diagnosed to finding the right doctor to thriving post-treatment, many patients express similar frustrations. And yet, there’s a common hope that runs through many of the stories we hear at the IPPF.

Each week through August and September, we’re featuring a story that highlights a specific part of the patient journey. OUR HOPE is that by sharing stories from our community, more patients and caregivers will realize they are not alone.

Check out the rest of the Patient Journey Series:

Our fifth story in the Patient Journey Series comes from Fred Wish:

It was January 2007, and I was sitting across the desk from the second dermatologist I had seen in five months. For the second time, I was told that my condition was probably “seborrheic something-or-other” and was prescribed an alcohol-based topical steroid that I knew would be painful to apply. I had endured three months of increasingly widespread lesions on my scalp and back, but hadn’t associated those with the bad mouth sores and sloughing of my gums that started the previous summer.

The following week, I was scheduled for a tooth cleaning that I’d put off because I was afraid it would hurt. The poor dental hygienist probably never had performed such a bloody cleaning. When the dentist inspected my mouth, he immediately scheduled me for a biopsy. The result came back positive for pemphigus vulgaris (PV). Lucky me.

Actually, I was lucky—I lived within a short train ride of Manhattan and Langone Health at New York University (NYU), which meant I had access to medical professionals with extensive experience with PV. Even luckier, in early March of 2007 I came under the care of Dr. Jean-Claude Bystryn, one of the world’s premier pemphigus experts. Dr. Bystryn treated me until shortly before his death in 2010.

I was diagnosed with PV, was the patient of a top medical professional, had begun to manage my disease with corticosteroids, and had an extraordinarily supportive caregiver in my wife. I wasn’t even aware at the time that I was missing a crucial part of what I needed in my battle with my disease: I didn’t have the slightest clue about how to live with PV day-to-day.

Help patients like Fred connect with the resources they need to live—and thrive—with pemphigus and pemphigoid.

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That’s when the IPPF came into the picture. Much of my career was spent associated in one way or another with the medical profession, which has perhaps made locating and comprehending detailed information about causes, treatments, side effects, insurance issues, etc., a bit easier for me than for some patients. My internet searches had turned up a ton of technical information and research papers about autoimmune blistering diseases, but not much about what it’s like to work, play, travel, or simply get out to a decent restaurant while dealing with stares and questions (and some well-intended but really bad advice) from friends and strangers. I felt alone. I knew there must be others out there who were struggling like I was, but until I found the IPPF website, I didn’t know how to connect with them. From my first tentative emails with Mei Ling Moore and Marc Yale, I knew: there were people who understood.

Of course, the IPPF website itself contains much information about the various forms of pemphigus and pemphigoid (P/P) as well as the services the IPPF provides. However, it has been the interaction with fellow patients through the peer health coaches, support groups, and the annual Patient Education Conferences, that have proven the most valuable to me. My work schedule kept me from attending a Patient Education Conference until 2014, when I traveled to Chicago and was able to meet so many people I had already encountered either through the IPPF website or various social media outlets. It was in Chicago that I learned of the potential of rituximab as a treatment option and attended meetings on topics such as relaxation techniques, dietary considerations, and the Awareness Ambassador Program (the outreach effort the IPPF put together to reduce the time gap between first symptoms and diagnosis). Just spending time with people who knew exactly what I was going through, who knew the instantaneous fear and rage that goes along with each new lesion, who were discovering how to find the shady spots at high noon, was an amazing comfort.

In Chicago, I not only signed on to help with the Awareness Program, but I also joined the Patient Registry, which has now been succeeded by the Natural History Study. Since then, I’ve attended Patient Education Conferences in New York (including a very chilly Mets-Yankees game!), Newport Beach, and Durham (where I re-established old friendships and met newer members of the P/P community). I’ve also met with members of my local support group; volunteered to help staff the IPPF booth at the Greater New York Dental Meeting in 2017 and 2018; provided P/P information to dozens of local dentists; and most recently advocated for the IPPF and supported legislation as part of Rare Disease Week on Capitol Hill.

I’m not writing all this to tell you what a good guy and solid citizen I am. I’m not a “joiner” by nature, and it surprises me that I’ve become involved as much as I have. The IPPF has provided such a solid foundation of support, both practical and intangible, to me over the years that I truly feel a part of the family. That’s what has instilled in me the impulse to roll up my sleeves and help out.

My PV has been in remission (no flares and no medication) for more than three years, but the IPPF continues to be an important part of my life. The Patient Education Series webinars help keep me up-to-date, and I look forward to each new issue of the Quarterly. I have already set aside time for the Patient Education Conference in Philadelphia this October.

Dealing with this ugly group of diseases is nothing any of us would have chosen. Make no mistake: I wish there wasn’t a need for this organization. I wish it had never been necessary to meet the patients, caregivers, and staff I’ve come to love and admire and whose friendship and commitment mean so much to me. But I am grateful every single day for having the opportunity to share this journey with them.

Give your share to show you care. Help patients like Fred

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Every day, our patient services team hears stories from our community about what it’s like to live with pemphigus and pemphigoid. From getting diagnosed to finding the right doctor to thriving post-treatment, many patients express similar frustrations. And yet, there’s a common hope that runs through many of the stories we hear at the IPPF.

Each week through August and September, we’re featuring a story that highlights a specific part of the patient journey. OUR HOPE is that by sharing stories from our community, more patients and caregivers will realize they are not alone.

Check out the rest of the Patient Journey Series:

Ellen Levine, IPPF Patient Educator, and Debra Teperman, IPPF Board of Directors. (Photo by Jeff Thiebauth Photography)

The IPPF is excited to announce that Ellen Levine, IPPF Patient Educator and pemphigus vulgaris (PV) patient, received the Harvard Medical School Dean’s Community Service Award for her work with the IPPF. The award identifies individuals whose dedication and commitment to community service have made an outstanding positive impact on the local and/or global community. The award includes a $1,000 donation to the IPPF.

Ellen lives in Boston and works for the Armenise-Harvard Foundation, a foundation at Harvard Medical School (HMS) that supports leading scientists at HMS and at institutions in Italy in the pursuit of scientific discovery in the field of medicine. As an IPPF Patient Educator with the Awareness Program, Ellen has traveled to various universities and professional exhibits to share her diagnosis story with dental students and professionals in order to accelerate pemphigus and pemphigoid (P/P) diagnosis times.

Upon receiving her award, Ellen acknowledged, “It wasn’t long ago that I wouldn’t disclose the nature of my disease with people who I didn’t know. I didn’t feel comfortable sharing the fact that I had a chronic illness, let alone such a rare one, and one with such a strange-sounding name. Now I realize how important it is to spread awareness and to do my part to make an impact. With this award, I hope to raise even more awareness of P/P among those in the HMS community and beyond.”

Read more about the award ceremony here.

Genentech announced this week that positive top line results from the Roche-sponsored Phase III PEMPHIX study evaluating the efficacy and safety of Rituxan® (rituximab) compared to mycophenolate mofetil (MMF) in adults with moderate to severe pemphigus vulgaris (PV). The study met the primary endpoint, and demonstrated that Rituxan is superior to MMF in achieving sustained complete remission.

“The PEMPHIX study provides additional clinical evidence for the use of Rituxan for the treatment of pemphigus vulgaris,” said Sandra Horning, M.D., chief medical officer and head of Global Product Development. “These data also demonstrated that Rituxan may provide complete remission rates and successful tapering of corticosteroid therapy that is superior to MMF in adults with pemphigus vulgaris.”

Read the full press release.

On March 15, 2019,  Roche announced that the European Commission has approved MabThera® (rituximab) for the treatment of adults with moderate to severe pemphigus vulgaris (PV), a rare condition characterised by progressive painful blistering of the skin and/or mucous membranes. Extensive blistering can lead to serious, life-threatening fluid loss, infection and/or death.

MabThera is the first biologic therapy approved by the European Commission for PV and the first major advancement in the treatment of the disease in more than 60 years. Following approval by the US Food and Drug Administration (FDA) in June 2018 and today’s decision, MabThera is now approved to treat four autoimmune diseases in the US and Europe.

“We’re pleased to bring the first biologic medicine to the more than 50,000 people in Europe suffering from pemphigus vulgaris,” said Sandra Horning, MD, Roche’s Chief Medical Officer and Head of Global Product Development. “This MabThera approval provides a much needed new treatment that has been shown to provide higher remission rates than corticosteroids alone, which can cause debilitating side effects.”

The European approval is based on data from the phase III Ritux 3 trial, a Roche-supported randomised controlled study, conducted in France, which evaluated MabThera plus a tapering regimen of oral corticosteroids (CS) compared to a standard dose of CS alone, as a first-line treatment in patients with newly diagnosed moderate to severe pemphigus. The primary endpoint of the study was complete remission at month 24 without the use of CS for two or more months. The study demonstrated that 89.5% of people with PV treated with MabThera, in combination with short-term oral CS treatment, achieved complete remission without the use of CS for two or more months, compared to 27.8% of people with PV receiving CS alone, the current standard of care. The results of the Ritux 3 trial were published in The Lancet in March 2017.

Read the full press release here.

On March 2, 2019, Principia Biopharma Inc., a late-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology, today announced Phase 2 clinical data from the Believe-PV study for PRN1008 as part of the Late-breaking Research: Clinical Trials program at the American Academy of Dermatology (AAD) annual meeting in Washington D.C. PRN1008 is being developed for the potential treatment of pemphigus, including pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Confirming interim clinical results previously reported, the Phase 2 study reached the primary efficacy measurement of control of disease activity (CDA) on low dose corticosteroids.

“The primary goal of treating patients with pemphigus is to control the disease and heal the skin, however a significant challenge is to avoid adverse events associated with the prolonged use of corticosteroids that are typically required to achieve clinical improvement,” stated Dr. Dedee Murrell, Professor and Head of the Department of Dermatology at The St. George Hospital Clinical School, University of New South Wales in Sydney, Australia and the lead principal investigator. “PRN1008 has the potential to rapidly and effectively treat patients’ disease, while significantly reducing the exposure to moderate to high corticosteroid doses.”

The open-label Phase 2 study enrolled 27 patients with newly diagnosed and relapsed, mild to severe pemphigus (including both PV and PF). Twenty-four patients were treated with oral PRN1008 and low dose corticosteroids (LDCS; <0.5mg/kg/day) for twelve weeks, with twelve weeks of follow-up. The primary endpoint was CDA (where new lesions cease to form, and existing lesions begin to heal) at Week 4. Other secondary endpoints included complete remission rates and reduction in anti-desmoglein autoantibody levels.

Read the full press release here. 

 

On February 22, 2019,  Principia Biopharma Inc., a late-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology, announced that an abstract has been accepted for oral presentation at the Late-Breaking Research during the 2019 American Academy of Dermatology annual meeting in Washington, D.C.

The abstract, “Final results of the Believe-PV proof of concept study of PRN1008 in pemphigus,” will be presented as part of the Late-Breaking Research: Clinical Trials on Saturday, March 2, 2019 at 2:10pm ET in Ballroom A of the Walter E. Washington Convention Center.

About Principia Biopharma
Principia is a late-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology. Principia’s proprietary Tailored Covalency® platform enables Principia to design and develop reversible and irreversible covalent, small molecule inhibitors with potencies and selectivities that have the potential to rival those of injectable biologics yet maintain the convenience of a pill. PRN1008, a reversible covalent BTK inhibitor, is being evaluated in a Phase 3 clinical trial in patients with pemphigus, an orphan autoimmune disease, and in a Phase 2 clinical trial in patients with immune thrombocytopenic purpura, a rare hematological disease. PRN2246, a covalent BTK inhibitor which crosses the blood-brain barrier, has completed a Phase 1 clinical trial in healthy volunteers, and has been partnered with Sanofi for development in multiple sclerosis and, potentially, for other diseases of the central nervous system. PRN1371, a covalent inhibitor of Fibroblast Growth Factor Receptor (FGFR) is being evaluated in a Phase 1 trial in patients with bladder cancer. For more information, please visit www.principiabio.com.

Read the full press release here.

The following is an excerpt from the article, “Breaking Bad: IgG4 in Autoimmunity” from Science Trends.

IgG4: friend or foe?

by ERIC M. MUKHERJEE & AIMEE S. PAYNE

Our laboratory researches a rare and debilitating autoantibody-mediated disease called pemphigus vulgaris, in which the immune system generates antibodies against desmogleins, which are proteins responsible for holding skin cells together. The result is painful, sometimes deadly, blistering of the skin and mucous membranes, requiring powerful immune suppressants such as steroids, mycophenolate, and the anti-B cell agent rituximab for disease control. Because pemphigus vulgaris has a well-characterized target, we study it as a model autoimmune disease to better understand why and how the immune system makes mistakes, so that we can develop better ways of reversing these mistakes and hence, better treat disease.

Patients with pemphigus vulgaris are known to have autoantibodies of the IgA1, IgA2, IgG1, and IgG4 subclasses, with IgG4, in particular, being a marker of active disease.  As mentioned above, IgG4 is typically considered an anti-inflammatory antibody, induced during states of chronic immune stimulation to dampen disease.

Beekeepers and individuals undergoing allergic desensitization therapy develop IgG4 antibody responses that are thought to block symptoms caused by IgG1 or IgE reacting to the same stimuli, one of the reasons why people can outgrow an allergy from childhood. However, in pemphigus vulgaris, the antibody variable region is sufficient to disrupt skin cell adhesion and cause blisters, so trying to suppress these unruly variable regions with a law-abiding constant region can’t override this pathologic effect. Thus, although the immune system is trying all its usual tricks to stop the disease, the switch of B cells to the IgG4 subclass does not allow pemphigus vulgaris patients to outgrow or remit disease but instead becomes the defining immunologic feature of their disease.

Continue reading the full article on Science Trends.

This article was originally published in Science Trends on October 19, 2018.

 

Principia Biopharma Inc, a clinical-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology, today announced positive top-line data from the completed open-label Phase 2 trial of PRN1008 in patients with pemphigus (including both pemphigus vulgaris (PV) and pemphigus foliaceus (PF)) and the initiation of a Phase 3 trial of PRN1008 in pemphigus. The primary efficacy endpoint of the Phase 2 trial – Control of Disease Activity (CDA) within four weeks – was achieved by more than 50% of patients and PRN1008 was generally well tolerated. Based on the results of the Phase 2 trial, Principia has initiated the PEGASUS study, a global, randomized, double-blind, placebo-controlled, pivotal, Phase 3 clinical trial of PRN1008 in patients with moderate to severe pemphigus.

“Pemphigus is a debilitating disease with high unmet need. We are encouraged by the efficacy and safety results from the Phase 2 trial and are initiating the Phase 3 trial based on these results. This is a key milestone for Principia, and the next step toward bringing this novel oral therapy to patients in need,” said Martin Babler, Chief Executive Officer of Principia.

“PRN1008 has the potential to effectively treat patients’ disease and significantly reduce dependence on harmful corticosteroid (CS) doses, which have been the mainstay of treatment in this disease, and may usher in a new era of steroid-sparing, oral therapy for patients suffering from the disease,” stated Dr. Dedee Murrell, Professor and Head of the Department of Dermatology at The St. George Hospital Clinical School, University of New South Wales in Sydney, Australia and the lead principal investigator.

The full data from the Phase 2 trial will be submitted for presentation at a future scientific conference.

Read the full press release here. 

On Thursday, June 7th, the FDA approved Rituxan for the treatment of adults with moderate to severe pemphigus vulgaris (PV).

Rituxan is the first biologic therapy approved by the FDA for PV and the first major advancement in the treatment of PV in more than 60 years. The FDA previously granted Priority Review, Breakthrough Therapy Designation and Orphan Drug Designation to Rituxan for the treatment of PV. With this decision, Rituxan is now approved to treat four autoimmune diseases.

“It is our hope that this announcement will open the door to approval for other indications in our diseases and usher in a renewed focus on available treatments,” said Marc Yale, Executive Director of the International Pemphigus & Pemphigoid Foundation.

The FDA approval is based on data from the Ritux 3 trial, a Roche-supported, randomized, controlled trial conducted in France that used Roche-manufactured, European Union (EU)-approved rituximab product as the clinical trial material. The study compared the Ritux 3 regimen (EU-approved rituximab product plus short-term corticosteroids [CS]) to CS alone as a first-line treatment in patients with newly diagnosed, moderate to severe pemphigus. The primary endpoint of the study was complete remission at month 24 without the use of steroids for two or more months. (Complete remission defined as complete epithelialization and absence of new and/or established lesions.)

Results of the study showed that 90 percent of PV patients treated with the Ritux 3 regimen met the endpoint, compared to 28 percent of PV patients treated with CS alone. These results supported the efficacy of Rituxan in treating patients with moderate to severe PV, while tapering off of CS therapy. These results were published in The Lancet in March 2017.

An international panel of experts called the International Bullous Disease Consensus Group recently provided new recommendations on the diagnosis and management of pemphigus in the Journal of the American Academy of Dermatology. Based on existing European treatment guidelines, a Delphi survey process was used to help achieve international expert consensus. The consensus includes the recommendation to use an anti-CD20 monoclonal antibody (Rituxan) and corticosteroids as first line therapy options for moderate to severe pemphigus.

The Role of the IPPF

The IPPF aims to serve as a primary source of information for you regarding this approved treatment and is available to help answer your questions in the upcoming months. If you are considering Rituxan as a potential therapy, please consult your healthcare provider. Inform them of your medical history, and ask about the potential side effects.

The IPPF’s Peer Health Coaches (PHC) are pemphigus and pemphigoid patients who help more than 1,200 patients and caregivers each year. These specially trained PHCs reduce patient anxiety and uncertainty while providing unbiased disease and treatment knowledge. You can find our PHCs engaging the community through social media, emails, phone calls, and in-person support. The goal of our PHC program is to ensure we help every person who needs assistance in the shortest amount of time possible.

Genentech Access Solutions

Genentech is the drug company that produces Rituxan (rituximab). Genentech Access Solutions is a resource for people considering Rituxan as a treatment option. It may be worth contacting Access Solutions directly regardless of whether or not you have health insurance.

Access Solutions may be able to help by:

  • Checking your insurance coverage and costs
  • Helping you find ways to pay for your medicine
  • Working to get your medicine to you

Visit Access Solutions to learn more.

Syntimmune recently announced positive preliminary results from its phase 1b proof-of-concept trial of SYNT001 in pemphigus vulgaris and foliaceus patients. It’s exciting for the IPPF to share good news related to research and treatments. The full press release from Syntimmune can be found here. The following is an excerpt:

Syntimmune, Inc., a clinical-stage biotechnology company developing antibody therapeutics targeting FcRn, today announced positive preliminary results from its Phase 1b proof-of-concept trial of SYNT001 in patients with pemphigus vulgaris and pemphigus foliaceus. The data showed clinically meaningful benefit of SYNT001, with a favorable safety and tolerability profile similar to that observed in the Phase 1a study.

“There remains a clear unmet need for a safe and fast-acting treatment for patients with pemphigus, who face serious symptoms and complications associated with their disease,” said Donna Culton, M.D., Ph.D., an assistant professor at the University of North Carolina School of Medicine. Culton presented preliminary results of the Phase 1b study at the International Investigative Dermatology conference being held on May 16-19, 2018 in Orlando, FL. “These preliminary data demonstrate safety as well as a rapid reduction in PDAI scores and lowering of IgG levels with treatment of SYNT001, which support further studies of this drug as a potential new therapeutic option,” Culton said.

Read Syntimmune’s press release, including additional information, here. 

Genentech recently announced an important FDA decision that could potentially impact future treatment options for pemphigus. Here at the IPPF, it’s especially exciting when we get to share good news related to research and treatments. The full press release from Genentech can be found here. The following is an excerpt:

The U.S. Food and Drug Administration (FDA) has accepted Genentech’s Supplemental Biologics License Application (sBLA) and granted Priority Review for the use of Rituxan® (rituximab) for the treatment of pemphigus vulgaris (PV). Last year, the FDA granted Breakthrough Therapy Designation and Orphan Drug Designation to Rituxan for the treatment of PV.

“We are committed to developing medicines for rare diseases with limited treatment options, such as pemphigus vulgaris,” said Sandra Horning, M.D., chief medical officer and head of Global Product Development. “We look forward to continued work with the FDA to hopefully provide patients with a new treatment for this serious and potentially life-threatening disease.”

The sBLA submission is based on data from a Roche-supported randomized trial conducted in France which evaluated Rituxan plus a tapering regimen of low dose oral corticosteroid (CS) treatment compared to a standard dose of CS alone as a first-line treatment in patients with newly diagnosed moderate to severe pemphigus. Results of the study show that Rituxan provides substantial improvement in pemphigus vulgaris remission rates and successful tapering and/or cessation of CS therapy. These results were published in The Lancet in March 2017. Genentech is currently conducting another Phase III study in PV which is evaluating Rituxan plus a tapering regimen of CS compared to Cellcept (PEMPHIX, NCT02383589).

Read Genentech’s press release, including additional information and references, here. 

In January 2013 my husband was diagnosed with prostate cancer. Thankfully, after surgery he was cancer free.

But in May 2015 our whole world seemed to turn upside down when he came home with a very unusual sore in his mouth. Fearing the cancer had returned, Tony went to our family doctor and was immediately sent to an oral surgeon for a biopsy. He had the results within days, but the outbreak spread like wildfire. By the following week, it had reached his entire mouth, sinus cavities, and throat. He was in severe pain, he couldn’t eat or drink and could barely speak.

The drive home that day was silent, both of us trying to stay calm and remember what the doctor had said. When we arrived home, we both went directly to the computer to research PV — which was the wrong thing to do.

While sitting in the oral surgeon’s office for what seemed like an eternity waiting for the results, the doctor assured us it wasn’t cancer, but instead told us Tony had pemphigus vulgaris. It was something we had never heard of and the doctor wasn’t that familiar with it either. He quickly explained to us what he knew about the disease and referred Tony to a dermatologist.

The drive home that day was silent, both of us trying to stay calm and remember what the doctor had said. When we arrived home, we both went directly to the computer to research PV — which was the wrong thing to do. Thankfully, the dermatologist, who was familiar with PV, was able to see him quickly.

Needing to understand this disease, I searched the internet for a support group and stumbled upon a video of Becky Strong. After a bad experience with someone claiming to be a PV support group, I researched more about the IPPF and finally reached out to Becky. We met for coffee and it was our first connection with a patient – someone who understood – someone who had been through the same pain and treatment – finally, someone who connected with Tony. That talk with Becky gave Tony the confidence to contact peer health coach Jack Sherman, who gave him the support he needed when he needed it the most. We can’t thank Jack enough. Our Austin, TX visit for the IPPF Patient Conference was priceless. How can you put a price on finding someone with the same rare disease, let alone an entire room of patients willing to share their stories and assure you there is hope?

Unfortunately after 21 months of a combination of prednisone and Cellcept, the treatment wasn’t enough to control Tony’s disease. He is now seeing a specialist at University of Illinois at Chicago and has been recommended for IVIg and Rituxan treatment. He has completed all of his pre-testing and is awaiting insurance clearance and a start date. These treatments are very expensive so the family is hosting a benefit to help cover some of the medical costs. IPPF has been generous in supplying us with materials to help educate everyone and we hope to spread awareness of this crazy, confusing disease.

Last year was shaping up to be a very important year. I had just gotten an associate degree in journalism and was all set to transfer to a four-year university in the fall to pursue my B.A. On top of that, I turned 21 in February and was looking forward to savoring all the exciting things young adulthood had to offer. It was during this very same month that everything changed.

I noticed a few innocent-looking blisters on my nose that were fragile to the touch. Although this was something I had never experienced before, I thought nothing of it and waited for the broken skin to heal on its own. It never did. The following weeks showed a worsening of my then-unknown condition: the taste of blood and large blotches of red in the sink were always present after brushing my teeth, the open sores on my face had multiplied to startling numbers, and the skin on the rest of my body threatened to tear away with any slight use of force or friction.

By the time May came around, I was living a horror film. Mornings began with my skin peeling away at the slightest touch and my clothes sticking to the popped blisters that covered my body.

By this point, I had seen my dentist and three different dermatologists, none of whom could provide me with a direct answer as to what exactly was wrong with me. The dentist blamed my symptoms on neglectful hygienic practices, and the others deemed it as an aggressive form of acne before prescribing me various ointments and creams to slather on my face. Weeks of waiting to see improvement turned into months, and the sores that started on my face were also manifesting on my chest and back. My appointments with the dermatologist were beginning to feel like an episode of The Twilight Zone–while exposing me to different kinds of light therapy and promising no results, no one was able to fully respond to my questions. But they were always willing to prescribe more pharmaceuticals.

By the time May came around, I was living a horror film. Mornings began with my skin peeling away at the slightest touch and my clothes sticking to the popped blisters that covered my body. My days were plagued by the excruciating pain of exposed flesh, my wavering faith for recovery, and the growing fear of the unknown. When it was time to sleep, I dreaded waking up to see what new bodily wound awaited me in the morning. I began to think, “Will I ever be the same again?”

I had decided that instead of waiting around for empty promises to be fulfilled, I needed results. Taking matters into our own hands, my mother and I researched my symptoms and visited a contagious disease specialist. Still clueless as to what ailment was literally tearing me apart all those months, I had come to believe it was something I caught from an outside source.

When the specialist analyzed my skin biopsy, he told me what I had was not an infection but an autoimmune disorder called pemphigus vulgaris.

. . . you know your body better than anyone else.

Only producing 1-10 new cases per 1 million people worldwide each year, pemphigus vulgaris is a very rare autoimmune disorder that causes the body to mistakenly attack itself, forming painful blistering lesions on the skin and inside the mouth. While there is currently no cure for this condition, there have been many cases of remission brought on by the use of immunosuppressive drugs and IV therapy.

Initially, I was shocked when I found out it wasn’t a foreign bacteria or severe allergic reaction that was ravaging my skin–it was my own body turning against me. This surprising diagnosis has taught me to never take my health for granted, and even though I have improved significantly since that fateful day in May, I am still on the mend and learning to cope with the healing process of my condition.

Through this long journey, I have learned valuable lessons regarding myself and what truly matters in life. Whether you have a blistering autoimmune disorder or any other visibly noticeable condition, it does not matter what other people think of your appearance. They will never know the challenges you’ve faced and the battles you’ve won. The best part is knowing that you have survived through it all and become stronger because of all those times of hardship.

Without a doubt, some days will be better than others, but when you feel you are being overpowered by those negative emotions, remember to surround yourself with love. I am immensely grateful for the support and care my loved ones have shown me, especially my parents, who have been by my side at every doctor’s appointment from here in the Coachella Valley all the way to San Bernardino. They are the light that still shines on my darkest moments. Even if it’s a hobby or a friend to talk to, find what gives you joy and hold onto that feeling. Happiness is the strongest medicine, and you deserve to feel your best even when your situation may not be.

Finally, the most important lesson that I’ve learned is that you know your body better than anyone else. If you should encounter a physician that does not satisfy your concerns or answer important questions you have, do not settle; seek the help you need until you find a doctor you feel confident will listen to you. I spent months misdiagnosed and being treated as though my observations and concerns weren’t relevant. When you know something is deeply wrong, you should always have the full support of a physician who has your best interests in mind.

Sometimes life can throw the most unexpected challenges at us, but we must carry on with the comfort that there will be better days ahead. Despite being confined to my home a majority of the year, I am currently attending college at the Cal State San Bernardino campus in Palm Desert with no intention of letting my condition get the best of me. My body may be at war with itself, but I know I’ll come out victorious in the end.

Hello all! Working for the IPPF has been something I have been interested in doing for about five years, since my pemphigus vulgaris finally got under control. I knew from my first contact with the Foundation that this is an amazing group of people. I am proud of the way our community pulls together and rallies for each other; it’s amazing how we sincerely care about one another.

The position of Outreach Manager is new to me, as well as to the IPPF. This position was created to be a link between Patient Support and the Awareness Campaign. While these programs have different focuses, they are united by the common goals of education, early diagnosis, and promoting the best treatments for P/P patients.

As a result, half of my time will be spent supporting patients. If anybody touched by our diseases has a question, comment, or concern, I am their first point of contact. It is my responsibility to provide all who contact me with clear, precise information and resources. I will also be creating and revising material for print and online, as well as helping to facilitate local support groups, meetings, and patient education calls.

The other half of my job will be spent raising awareness of P/P in the medical community with the goal of reducing patient suffering and diagnostic delays. Currently, the Awareness Campaign focuses on dental outreach. I will be coordinating patient presentations at universities and looking to recruit, train, and supervise other patient speakers and volunteers. I will also take over coordinating exhibit booths at conferences for dentists and medical professionals. It is my goal to pass along what I’ve learned and use my knowledge to make a difference in the lives of P/P patients. And the IPPF has given me this wonderful opportunity to do so.” It sounds like a lot of work — and it is — but I am up for the challenge! Because of my status as a PV patient and my past work in the healthcare field, I feel that I am qualified to work with both communities simultaneously. I promise to work tirelessly to promote quality of life for the members of our community, as well as in the dental, medical, and nursing communities.

I know I would not be where I am today if it weren’t for the IPPF community. So many of you provided me with support, compassion, and encouragement during one of the lowest periods of my life. I was young when I was diagnosed. I was scared and felt isolated. But my IPPF friends changed all of that. I want to do that for others. It is my goal to pass along what I’ve learned and use my knowledge to make a difference in the lives of P/P patients. And the IPPF has given me this wonderful opportunity to do so.

Please know you can contact me by email at becky@pemphigus.org or by phone at  (916) 992-1298 x105. I will work to get you the answers you need. I am here for you.

As a Patient Educator for the IPPF, I have the fantastic opportunity to travel around the country to different dental schools and give lectures on my journey with pemphigus vulgaris (PV). It’s an empowering experience to have a hundred people listen to my story at once. But it’s also important that the audience relates to me. I’m a person, not just a patient. I have to be open and honest about my journey. The audience must relate to the pain, suffering, sense of humor, and all the ups and downs of having PV in order to form a powerful memory. But how does this happen? How do I find unique ways to raise awareness not only in the classroom, but also on the trip to and from the university?

  1. Appearance matters – We’ve all heard our mothers tell us to put on clean underwear before leaving the house, right? This is no different. Looking tidy helps provide credibility. It also helps you to look engaging. Plus, the more you enjoy life, the more others will want to be around you.
  2. Don’t forget the Orange Sunglasses – The orange #healourskin sunglasses are a huge attention grabber. I wear mine EVERYWHERE! I keep them in the car, hang them from my collar, and wear them on top of my head. I wear them on vacation and around town. Many people have stopped me and asked, “What’s with the orange glasses?” It’s important to have a brief, but open answer to this question. Mine is, “You know the pink ribbons for breast cancer? Well, I have a rare autoimmune disease, and these are part of the campaign to raise awareness.” People usually follow up with, “What’s it called?” The conversation takes off from there. I also travel with a couple of extra pairs to give to people I meet along the way, and this leads to others inquiring about the glasses and PV.
  3. HI! – It’s the single, most important thing an advocate can say. It’s the door that opens communication with another person, group, or audience. It’s not intimidating for the listener, and most everybody smiles when they say it. Don’t forget to make eye contact, too.
  4. Be engaging – It’s important to not only talk, but also to listen. I don’t usually start off by talking about PV. Rather, I talk about the long line, the beautiful weather, or even a great piece of jewelry that somebody is wearing. Be aware of how people respond to you. While you may want to tell your life story, it can be overwhelming for some. Listen to their questions, and answer them honestly without over-sharing.
  5. Be Brave – This tactic works the same in the grocery store or at the park as it does on an airplane or at a university. Tell somebody about the glasses and ask if you can take their picture to use as part of the Awareness Campaign. When I fly, I usually ask the flight attendants to take a picture with the glasses. I explain that I’m traveling to lecture at a university and the glasses are part of the Awareness Campaign. What’s the worst that will happen? They’ll say no. In that case, I’ll smile, thank them kindly, and take my seat. I’ll probably never see them again anyway. But if they say yes, it opens a whole dialog. Sometimes, they even ask the pilots if I can take their picture once we’ve landed. I ask the same thing of the professors who invite me into their class. The Profs know I’m part of the Awareness Campaign and are usually happy to oblige. Once one person does it, others inquire about what’s going on. Each inquiry is an opportunity to spread awareness.
  6. Selfie-sticks – Ok, so maybe approaching others isn’t your thing. But I bet you’ve taken a selfie. Take your picture wearing the orange sunglasses while reading The Quarterly, petting the dog, holding a new baby, or with your best friend. Just don’t forget to post the pic with #healourskin!

There are opportunities to spread awareness everywhere. It just takes a moment to recognize and act on them.

There are also hundreds of other ways to be an advocate that I haven’t even thought of yet. That’s where you come in. Take my suggestions and run. I hope I’ve given you ideas of what you can do to help the IPPF spread the word. Put your unique spin on the Awareness Campaign. Please don’t forget to share your pictures – tag the IPPF and use the hash tag #healourskin. I can’t wait to see what you come up with!

While you are seeing a qualified dermatologist who is treating you for your Pemphigus Vulgaris, Bullous Pemphigoid, Pemphigus Foliaceus, Mucous Membrane Pemphigoid, etc. you might also be seeing your own dentist, OB/GYN, internist, ophthalmologist or ear/nose/throat specialist.

Please be sure that all of your doctors are aware of your condition and that they have access to your dermatologist.  It is important that they know the medications and dosage that you are taking for each medication.

All of your doctors need to be able to communicate with one another if necessary.  Being left in the dark will leave you at a disadvantage.  Also, if you are going to be scheduled for any major dental work, advise your dermatologist.  Depending on the procedure, your medications may be adjusted for a few days prior and a few days following to prevent any flare-ups.

Remember when you need us we are in your corner!

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