Highly accurate IIF serological assay for Laminin 332 Mucous Membrane Pemphigoidreduces the wait for confirmed diagnosis from months or years to 72 hours or less
On July 27, 2022 KSL Beutner Laboratories (Beutner), a global leader in immunologic testing for the diagnosis of bullous, vascular, connective tissue and inherited skin diseases, announced the launch of a first-to-market indirect immunofluorescence (IIF) serum blood testin the U.S. that positively identifies laminin 332, an antigen associated with the chronic, debilitating autoimmune disease mucous membrane pemphigoid (MMP).
Without a definitive interpretation, patients experience considerable pain and suffering due to misdiagnosis and treatment delays. Long sought after by oral pathologists, oral surgeons, periodontists and dentists, in addition to dermatologists, Beutner’s laminin 332-specific serological assay reduces the time to confirmed diagnosis from as much as two years to 72 hours or less.
“The development and launch of Beutner’s much-anticipated serological assay for laminin 332 MMP builds on the pioneering work of Dr. Ernst Beutner, the father of immunodermatology,” said Dr. Lakshmanan Suresh, Technical Director at Beutner and Chief Medical Officer of KSL Diagnostics, Inc. (www.ksldx.com), which counts Beutner among its clinical laboratories. “The innovative methods he established for diagnosing autoimmune blistering diseases are used worldwide, and we are proud to continue his legacy.”
MMP is an autoimmune blistering disease characterized by multisite lesions on mucous membranes. Anti-laminin 332 MMP lesions often scar and can lead to serious complications depending on the mucosal surfaces affected. Oral mucosa — gums, inner lining of the cheeks and lips, palate and tongue — are involved in 80-90% of cases. Scarring of ocular mucosa, present in half of patients, may lead to blindness. The disease can also impact mucous membranes in the nose, throat, genitals and anus, causing severe, irreversible damage. Another complication in 25-30% of patients is increased risk of cancer malignancies, primarily adenocarcinoma in the gastrointestinal and genital mucosa and lungs. The condition can be fatal if left untreated. Considered a rare disease of unknown cause, MMP occurs mainly in people between ages 60 and 80 and, infrequently, in children. Women are affected twice as often as men.
“The first assay of its kind available in the U.S., Beutner’s IIF serum blood test verifying the presence of specific autoantibodies for laminin 332 MMP now enables clinicians to accurately identify this hard-to-diagnose disease much faster,” said Dr. Raminder Grover, Laboratory Director at Beutner. “This in turn allows for available therapies to be started much earlier to alleviate patients’ significant ongoing discomfort and spare them the long-term medical complications of this devastating disease.”
The most common autoantigens associated with MMP are BP180 and laminin 332. About one-third of patients are afflicted with the laminin 332 variant. Since 2002, blood tests to detect BP180 have been widely available, but a laminin 332 serum test has not been approved in the U.S. until now. Anti-laminin 332 MMP cannot be differentiated from other forms of the disease based on clinical examination. It can only be distinguished by a serological test for IgG antibodies of the variant. Because it mimics other diseases in the mouth, patients may suffer increasing pain and decreased quality of life for six months to two years before obtaining conclusive test results. Reaching a positive diagnosis as soon as possible is critical so that physicians can begin treatment.
Confirming laminin 332 MMP relies on several laboratory tests offered by Beutner: first, a direct immunofluorescence (DIF) microscopy of a skin biopsy to detect tissue-bound immunoreactants. Although DIF is the gold standard for investigating all forms of MMP, it does not always differentiate between variants. Next, indirect immunofluorescence is applied to identify circulating antibodies targeting the autoantigens in the basement membrane zone (BMZ) of the skin. This type of analysis is done in two parts – an IIF test on salt-split skin and an IIF serum test on transfected cells for laminin 332 IgG/IgG4 antibodies using a EUROIMMUN assay. Beutner’s IIF serological assay has a sensitivity of 84% and a specificity of 99%.
Beutner recently received approval from the New York State Department of Health to perform the laminin 332 IIF blood test for U.S. customers at its Buffalo, NY lab.
Dr. Ernst H. Beutner, a co-founder of Beutner Laboratories, pioneered the DIF test 40 years ago after he and his associates at the University at Buffalo (UB) discovered the role of autoimmunity in pemphigus and pemphigoid. Development of defined, quantified immunofluorescent methods now used worldwide for the investigation of autoimmune skin diseases began with studies at UB led by senior researchers from Beutner, UB and Harvard University.
KSL Beutner Laboratories provides anti-laminin 332 IIF serological testing as a service, as well as several other assays for laminin 332 MMP and for the BP180 variant of MMP. Tests can be ordered at https://www.beutnerlabs.com/.
Akari Therapeutics recently announced that positive results from the Phase II study of investigational nomacopan in bullous pemphigoid (BP) were published online in the Journal of the American Medical Association (JAMA).
“These positive Phase II data advanced our understanding of the nomacopan safety profile and informed duration of treatment in the ARREST-BP Phase III clinical trial, which is open for enrollment now,” said Rachelle Jacques, President and CEO of Akari Therapeutics.
BP is the most common autoimmune blistering skin disease. It typically affects people over the age of 65. There are no approved therapies but superpotent topical steroids and high dose oral corticosteroids (OCS) are the current standard of care. The mortality rate in BP is ~three-fold higher than the general population due to the disease itself, and infections and cardiovascular conditions that are more common in older patients and are exacerbated by treatment with high dose OCS. There is significant unmet need for an effective steroid-sparing therapy.
April 28, 2021 (GLOBE NEWSWIRE) — Akari Therapeutics, Plc, a biopharmaceutical company focused on innovative therapeutics to treat orphan autoimmune and inflammatory diseases where the complement and leukotriene systems are implicated, today announced that the FDA has granted Fast Track designation to nomacopan for the treatment of patients with moderate and severe bullous pemphigoid (BP). Nomacopan has also been granted orphan drug designation for nomacopan for the treatment of BP by the FDA and the European Medicines Agency (EMA).
Fast track is a process designed to facilitate the development and expedite the review of drugs to treat serious conditions and fill an unmet medical need. The purpose is to get important new drugs to the patient earlier. A drug that receives Fast Track designation benefits from more frequent communications and meetings with the FDA to review the drug’s development plan including the design of the proposed clinical trials, use of biomarkers and the extent of data needed for approval.
Success in BP could potentially open up a range of other severe dermatological conditions for treatment with nomacopan where C5 and LTB4 are implicated, including hidradenitis suppurativa, epidermolysis bullosa acquisita and mucous membrane pemphigoid.
Our final story in our Patient Journey Series comes from Linda:
My journey began in August, 2017. I had a slight rash on my lower back that was extremely itchy. When I went to see my primary care doctor for my annual physical, I mentioned the rash, but he never looked at it. He just brushed it off.
One month later, I spent a week on Cape Cod with my husband, Gary, and family. We walked on the beach and enjoyed the sun, as it was unusually warm and sunny for so late in the season. By the time we got home from vacation, the tops of my arms had a slight rash and were extremely itchy. I didn’t have time to see a doctor because five days later I traveled to sunny Florida with friends, so I bought topical creams and enjoyed the sun and beach once again.
The Florida weather was perfect, and I spent several hours sitting at the edge of the water enjoying the sand and the cool water running over my legs. It was heaven . . . up until the evening when my legs broke out in a more severe rash that itched all night. I couldn’t handle how itchy my skin was, and the rash had spread. The topical cream wasn’t enough, so I bought Benadryl to help me sleep. I spent the rest of the vacation miserable with the rash and itchiness. I called my primary care doctor as soon as I got home to Connecticut.
The Advanced Practice Registered Nurse (APRN) was baffled when I went in for my appointment. I went through the routine questions about whether I had changed soap, detergent, diet, or medications. She brought another nurse in to consult, and I was asked if I traveled out of the country or whether it could be related to bedbugs. I was given prednisone and sent on my way. They told me if I wasn’t better after finishing the prescription they would refer me to a dermatologist.
While I was on 20mg of prednisone, the rash and itchiness did seem a little better. When I decreased the dosage, they got worse again. I asked for a referral to see a dermatologist and to increase my prednisone dosage back to 20mg; however, my dosage wasn’t increased and the rash and itchiness got worse once again. It was now the end of October and the dermatologist couldn’t see me for three weeks. I was getting worse every day. I couldn’t do anything because of how physically uncomfortable I was. I also felt mentally exhausted and confused about what was happening to me and why.
Finally, I saw the APRN at the dermatologist office. She thought it was something systemic and gave me cream and some antibiotics and told me to return after two weeks. In those two weeks my feet and hands were on fire—red, swollen, and severely itchy. When I returned, they changed my antibiotics, gave me clobetosol salve for my feet and hands, and said, “I think you’re allergic to black plastic.” What?!
I was tested for contact allergies, but all that showed up was a slight allergy to nickel. At this point, I had been taking antibiotics for over a month and everything seemed to be getting better. I stayed on them and I had no rash or itching through March of 2018. I was exercising, taking natural supplements, and following a diet of protein shakes for two meals and a healthy third meal. I had lost 30 pounds.
I stopped taking all medications at the beginning of March. A few weeks later, the itchiness came back, but I couldn’t get in to see the dermatologist right away. When I finally had my appointment, the APRN checked me out and said once again, “I swear you’re allergic to black plastic.” I got rid of my purse and black flip flops, and I was given another prescription for the clobetosol. They told me if I wasn’t better in five days I could come back for a steroid shot.
After the appointment I was on vacation when the palms of my hands and soles of my feet were hot and itchy. I spent the week with an ice bucket close by to put my feet in and a frozen water bottle to help my hands. My husband and I discussed how none of my doctors had ordered any blood tests. I found out there was a new APRN (an internist) at my primary care doctor’s office, so I made an appointment with her. I also decided at this point to change my diet again. I have suffered from eczema since I was a child, so I looked into the eczema skin diet. I started eliminating things like sugar, caffeine, gluten, histamines, MSG, and dairy.
I was hopeful at my next appointment, and my husband came with me to make sure that something was finally done to help with my symptoms. After one look at my hands, the internist said, “It all points to an autoimmune disease. Let’s run a complete work up and see what we can find. If nothing comes up, then we will do a skin biopsy.” Finally!
Bullous pemphigoid? What the heck is that?
The blood work results didn’t show anything definitive, but it did show the possibility of an autoimmune disease, so the internist suggested an immunofluorescence test. I asked about conducting a biopsy after it seemed like they weren’t going to follow up with one. The weekend before the biopsy was scheduled, I had strange blisters on my wrists, thighs, and stomach. Although it was sunny, humid, and in the 90s, I wore a long-sleeved jacket because I was afraid people would notice the blisters on my arms and think I was contagious or just freaky looking. My doctor reluctantly took a punch biopsy and told me it looked like possibly poison ivy or a bug bite. I was so frustrated! I asked him why I would have blisters all over from a bug bite? He told me that nine out of ten times nothing shows up on the biopsy results. I left his office super disgusted and on a mission to find a new primary care doctor with a little more care for their patients.
I was deteriorating—not just physically, but mentally. More blisters showed up. I was itchy and in pain. My skin was red like a sunburn, felt hot, and was slightly swollen. When I got the results from my biopsy, they pointed to bullous pemphigoid (BP). An immunofluorescence test was suggested, but wasn’t setup by my doctor. I had to wait a few days before seeing my doctor to discuss the results and treatment options.
Bullous pemphigoid? What the heck is that? I immediately started Googling it. The first site I saw was a medical site that explained it as a potentially fatal autoimmune disease, and usually older people get it. Are you kidding me? I searched again and found the IPPF’s website. I couldn’t believe that there was an organization for this strange disease! I found so much information. I was very excited and started crying. Finally, a place that knew all about this disease. I found information about the physician map, signed up for it, and was emailed the information a few days later. I found information about dermatologists that specialize in blistering diseases in Boston and Connecticut, including Dr. Mary Tomayko. She was located about an hour away from me and had a clinic. I felt like I had struck gold! Of course, I cried more.
When I saw my primary care doctor after my biopsy results, he prescribed 40mg of prednisone for a week, followed by tapering every five days by 10 mg. I told him I wanted to be referred to Dr. Tomayko, and he was less than pleasant about it. He basically dismissed me. He also deleted the suggestion of poison ivy and bug bites from my records before sending them off to Dr. Tomayko. I wasn’t able to get in to see Dr. Tomayko for a few months, but I was happy to have an appointment scheduled with someone that should know what to do for my treatment.
I had many setbacks over the two months leading to my appointment, but I also had support from the IPPF. Becky Strong, the IPPF Outreach Director, was amazing. She helped with my questions and sent me a patient guide to review before my appointment. I also had access to monthly webinars on different topics, and they were amazing. The first one I attended was on the side effects of prednisone. What great timing! After exploring the IPPF site more thoroughly, I saw information about the annual Patient Education Conference in October. My husband told me to sign us both up.
We needed to be more informed and meet other people who were dealing with similar issues. We became Healing Heroes (the IPPF’s monthly donation program) and received a discount on the Patient Education Conference registration. I followed my doctor’s advice about tapering down the prednisone dosage, but found that 20mg worked best for me and told him that’s what I wanted to do until my next appointment with Dr. Tomayko. Thankfully, he listened.
My husband suggested that I take pictures of my blisters in case they were cleared up before I saw Dr. Tomayko. Smart man! While I waited for my appointment, I got my list of questions ready, planned our trip to the conference, and felt somewhat relieved to know we would meet others with similar issues and have access to experts we could talk to.
When I finally met Dr. Tomayko, I loved her office staff and she was wonderful. I wasn’t able to receive a definitive BP diagnosis until further blood work was done. Since my doctor never did the immunofluorescence testing, she couldn’t be sure. She explained that they needed to look for markers in my blood, which I understood because that was one of the many things I read about on the IPPF website. She ordered additional blood work, a baseline bone scan, a tapering of the prednisone by 2.5mg every seven days, and continued use of the clobetosol as needed. I made another appointment for a month later.
The blood work came back showing BP. In the month prior to going back to see Dr. Tomayko, I had a slight flare up and developed a couple of new blisters. She increased my prednisone dosage again and told me we would talk about an additional drug at my next appointment. At that appointment she prescribed CellCept®. She told me how it works, about side effects, and gave more instructions on my care, including a schedule of how she wanted me to taper off the prednisone.
We told her that we were attending the IPPF Patient Education Conference, and she was very excited. She told us what a wonderful idea it was, that we were going to enjoy it, that we would have all our questions answered, and to say hello to her dear friend Dr. Donna Culton (the conference co-host). We left feeling even happier about our decision to attend.
A short time later, my husband and I were off to the conference! We were both excited to gather with other patients and experts in the field. For the first time in a long time, I felt comfortable and at ease. Not only would I have an opportunity to meet others that would completely understand what I was going through, but my husband would also have an opportunity to gather with other caregivers who were supporting each other in their journeys as well.
I may be the patient, but this journey includes everyone in my inner circle, and they need support just as much as I do. Our lives have been disrupted and changed forever. We as patients need to keep in mind that we are not the only ones who suffer—those around us have to watch, sometimes helplessly, as we go through this. They are unable to do anything but comfort us. And in many cases, they take on extra work that they haven’t previously done.
As a licensed massage therapist, my husband knows how muscles and tendons work and interact. He discovered that BP gets worse around your flexors. The palms of my hands were swollen, so the more I used my hands and flexed my fingers etc., the worse it got. He took over the cooking in our house after never cooking full meals before. But he conquered his fears in the kitchen and kept us going.
We arrived in Durham, NC, the day before the conference started due to a hurricane. There was an extra day planned for those of us who wanted to attend Dental Day at the University of North Carolina Dental School. Becky Strong started the day by sharing her story with pemphigus vulgaris (PV). It was hard to listen to because it brought up so many emotions for me. It was good for me to hear though, because listening to her in person made me truly feel that I was no longer alone. And she survived! She looked whole and happy. It was important for me to hear her story since I was still at the beginning stages of my disease. It was such a comforting and educational day. We met and spoke with so many other patients. In fact, one of the women I spoke to said they had just met another patient from Connecticut and planned to introduce us. We got the chance to speak with Dr. Culton as well, and I told her that my doctor was Dr. Tomayko. She was excited to know that I was seeing Dr. Tomayko. What a good feeling that was!
The next day was a full schedule. After welcome announcements, we listened to Kenny Metcalf (Elton John entertainer) tell about his journey with PV. It was an amazing story, which included pictures of him throughout his disease. After seeing the pictures and his condition, one would never think that he had survived. But he’s alive, whole, happy, and doing what he loves—entertaining people with his gift of music. The rest of the day was broken into different pemphigus and pemphigoid sessions geared toward our own journeys. Our diseases may be similar, but they are also different. It was great to know that the speakers would address our disease-specific issues. Additionally, those in each session shared similar issues. We were able to swap information and keep in contact after the conference ended.
In the evening, we attended the awards dinner. This was totally different than a typical awards dinner. We were all relaxed and got to know each other as we ate and listened to music. We didn’t walk into the room and wonder who we could sit with. This was now a large group of friends, and we all had a common thread that joined us together. We weren’t rare in this room full of people! The camaraderie between all of us, including the doctors, was truly wonderful. The most memorable of all was the entertainment by Kenny Metcalf. He started our day with an emotional story of his life with PV and ended the day with a great show. He was Kenny, playing the part of Elton John in the early years. He was amazing! He had everyone singing and dancing, and there was just pure joy throughout the room. I can tell you this, I will never hear the song “I’m Still Standing” without smiling—and maybe even shed a few tears—while thinking of Kenny. In my mind, that will forever be the IPPF theme song. We’re still standing!
The last day was a half day of workshops. There were so many good ones to choose from—women’s health issues, ocular issues, wound care, and an extremely important one that made a difference for us: caregiving. My husband joined the caregiver workshop led by Janet Segall, the IPPF’s founder. He felt so much relief after attending that session. He was able to discuss his concerns and hear what the other caregivers were going through. He said that Janet led the session with great empathy, concern, and experience.
When the conference was over, it seemed too soon. We covered a lot together in just a few days. Both Gary and I felt relieved and so much better. We felt like we were part of a much bigger family and had made new friends who we shared similar experiences with. We had names and numbers to reach out to when things seemed too crazy to deal with alone.
The IPPF conference was the light and guide that walked us through a very dark tunnel. A tunnel we would’ve walked through alone. But everyone involved turned up the light nice and bright, and we found we were two of many. We were not alone and we would never feel alone again! We cannot wait to attend the 2019 Patient Education Conference in Philadelphia this year. We cannot wait to have the opportunity to reconnect with those we met last year and to connect with new friends. We hope that we are able to answer questions or just be supportive to those who are new to the conference and/or these diseases, to find out what is new with research and medications, and to hear the voices of other experts in the field. If you’re able to join us, look for Gary and me. We would enjoy that. And if nothing else, you’ll see, I’m still standing!
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.
On April 23, 2019, Akari Therapeutics, Plc (Nasdaq: AKTX), a biopharmaceutical company focused on innovative therapeutics to treat orphan autoimmune and inflammatory diseases where the complement and/or leukotriene systems are implicated, announced positive initial Phase II clinical data from the first three of bullous pemphigoid (BP) patients in an ongoing clinical trial.
Bullous pemphigoid is a severe orphan inflammatory skin disease currently treated primarily with steroids and immunosuppressants which bring with them well known side effects. Treatment response and steroid potency varies significantly based on the severity of the disease, although flares and relapses frequently occur.
In patients with bullous pemphigoid there is evidence that both terminal complement activation (C5) and the lipid mediator leukotriene B4 (LTB4) have a central role in driving the disease. Ex vivo data, from a recent study at Lubeck University, in BP patients showed a pronounced accumulation of LTB4 and C5 and its activation products in the inflamed skin of bullous pemphigoid disease patients.
The Phase II trial for up to nine mild-to-moderate bullous pemphigoid patients is a six-week open-label single-arm study evaluating safety and with the main efficacy measure the Bullous Pemphigoid Disease Area Index (BPDAI) a frequently used evaluation of the extent and severity of the disease.
Initial results from the first three patients showed that Nomacopan (Coversin), dosed daily subcutaneously, was well tolerated in three elderly patients (>65 years), and that there were no drug-related adverse events.
Immune Pharmaceuticals, Inc., a biopharmaceutical company developing novel therapeutic agents for the treatment of immunologic and inflammatory diseases, announced today that the U.S. Food and Drug Administration (FDA) has granted Fast Track designation to bertilimumab for the treatment of bullous pemphigoid (BP).
“I am truly grateful that the FDA has granted Fast Track designation to bertilimumab for the treatment of bullous pemphigoid. This important achievement follows the recent granting of Orphan Drug Designation in both the United States and Europe, which together demonstrate the regulatory affairs momentum our team has achieved,” commented Immune’s Interim Chief Executive Officer, Tony Fiorino, MD, PhD. “There is no doubt that bertilimumab development will benefit from the opportunity to have more frequent contact with the FDA, particularly now, as we move forward with a new manufacturing process and plan for a phase 2/3 pivotal study in bullous pemphigoid.”
The FDA’s Fast Track program is designed to facilitate the development and expedite the review of drugs to treat serious conditions that are unmet medical needs. Fast Track designation enables more frequent interactions with the FDA in order to shorten the development and review process, and may include potential eligibility for Accelerated Approval, Priority Review and Rolling Review.
Immune Pharmaceuticals, Inc., a biopharmaceutical company developing novel therapeutic agents for the treatment of immunologic and inflammatory diseases, today announced that the U.S. Food and Drug Administration (FDA) has granted Orphan Drug Designation (ODD) to bertilimumab for the treatment of bullous pemphigoid (BP).
“We are incredibly gratified that bertilimumab has received Orphan Drug Designation for the treatment of bullous pemphigoid from the FDA and believe this designation, coupled with the recent positive opinion from the [European Medicines Agency] EMA’s Committee for Orphan Medicinal Products represent a significant regulatory milestone or bertilimumab,” commented Immune’s Chief Medical and Operating Officer, Tony Fiorino, MD, PhD. “We are focused on putting all of the manufacturing and regulatory pieces in place to launch a pivotal phase 2/3 study of bertilimumab in bullous pemphigoid next year.”
The FDA Orphan Drug Designation program provides a special status to drugs and biologics intended to treat, diagnose or prevent diseases and disorders that affect fewer than 200,000 people in the U.S. This designation provides for a seven-year marketing exclusivity period, as well as certain incentives, including federal grants, tax credits and a waiver of PDUFA filing fees.
Pemphigus and pemphigoid (P/P) patients may agree that it can be very difficult and painful in the early months of their condition. As a pemphigoid patient, I learned how to apply dressings (large and small), developed a schedule of self-care, and adopted a clothing style and activity level that fit my needs. I discussed my care plan with my internal medicine doctor, dermatologist, dentist, dental hygienist, and oral surgeon, who all approved. The following suggestions may be helpful, such as learning how to care for our skin by taking and applying topical medications, cleansers, moisturizers, dressings, and staying comfortable. Please review this article with your healthcare provider before implementing these ideas.
First, before purchasing any kind of care product, ask your dermatologist if they recommend using or avoiding anything specific. (Take into consideration any allergies, such as latex.) Check with your insurance company and see if they will help pay for any products. Many online stores may be cheaper and offer discounted prices. You may also work with your insurance company or Health Savings Account (HSA) when buying care products.
When applying or changing dressings, wash your hands with soap and water, and use gloves. In accordance with my dermatologist’s guidance, I kept my nails (hands and feet) short to avoid injuring my skin or spreading infection. (Please discuss applying any topical medications with your dermatologist.) For a small area, I applied topical steroid cream and Vaseline with a Q-tip. If it was a larger area (more than 4×4 inches) I squeezed a small film of both Vaseline and topical steroid cream onto my non-dominant gloved hand and applied the mixture onto the affected area with my dominant hand. My dermatologist had previously instructed me on the exact amount to use when applying topical steroid to avoid damaging the skin. Additionally, I made sure that the opening or mouth of the tube of medication didn’t touch my skin in order to avoid infection. Vaseline keeps damaged skin moist, and I still use Cetaphil cleanser and moisturizer when caring for my skin and hair (I find it very easy to use). I also use non-latex gloves when caring for my skin.
Both non-stick and regular pads come in various sizes and are easy on the skin. Gentle wrap bandages are also very easy to wear and they do not stick to skin or hair. Many product lines provide sensitive skin bandages and pads that are pain-free when removing. Gauze bandaging can help keep pads in place, and elastic stretch netting can help keep bandages and gauze in place. Lastly, gentle tape is a great product that adheres well to skin and removes easily. Make sure you review exactly how to use these and any products when caring for your skin–including how often to check and change your dressings–with your health care provider. I found it helpful to write down all instructions, medications, and medical products. I quickly learned which products worked well and whether to purchase them locally at a store, pharmacy, or online. I established a positive and team-focused relationship with three local pharmacies where I ordered my medications and care products.
I continue to work with my dentist, dental hygienist, and oral surgeon to manage my oral hygiene. I receive dental cleanings every four months instead of the typical six months from my dental hygienist per my oral surgeon’s guidance. I use soft children’s brushes to brush my teeth, soft dental floss, and a product called Biotene, which comes in various flavors. I gently brush my teeth after meals and floss once daily. I carry my dental products, medications, and any dressing products in a lunch bag; it’s very easy to carry, and it keeps all of my medical needs handy and discreet.
Like many P/P patients, I was misdiagnosed for months. As a result, a lot of my clothes were ruined. After I was diagnosed, the combination of added weight from various medications and dressings made wearing clothes hot and very uncomfortable. Wearing business clothes, slacks, skirts, sweaters, hose, blouses, and jeans made my skin hurt and itch even more! I found it helpful to wear lightweight, dark-colored athletic clothing with cooling wick technology. I wore dark colors because topical steroids stain and are very difficult to remove.
Over time my style changed to lightweight pullover athletic shorts, pullover short-sleeve shirts, large light long-sleeve shirts, athletic pants (no sweats or yoga pants), cotton underwear, and socks. I avoided button-down shirts, rings, watches, or jewelry. I made sure to wear hats and light coats and mittens outside. I learned that if my clothes let me breathe, felt cool and light–I had something to work with. I found wearing soft athletic sneakers and slippers worked best for me instead of shoes. We also kept our home cool, and I avoided exposure to heat or bright sunlight for extended periods of time.
My family and I developed a schedule after guidance from my medical team. We set up a cabinet in the bathroom that contained all of my medications, dressings, and medical supplies. My schedule included checking any areas of damaged skin (wound check), showering with lukewarm water and Cetaphil, changing dressings, and then changing into comfortable clothes. After this, I did another wound check on my mouth. I then gently washed my mouth out with cool water and brushed with Biotene using a soft children’s toothbrush. I found using a product called “magic mouth” helped greatly with my painful mouth sores. (This product can only be purchased with a prescription.)
I worked with a dietician to identify foods that both met my nutritional needs and kept me from gaining weight due to little activity and medication. My diet consisted of various soups, broths, frozen pops, and cooked soft foods. This helped a great deal, and I soon developed a menu. I took any medications by schedule and kept busy reading, watching TV and movies, doing word puzzles, art therapy, doing laundry, going on short walks, and following golf. Golf is a passion of mine, and it really helped divert my attention. Our church helped with visits, meals, and exploring my faith. I used meditation, stress relaxation, gentle yoga, calming music, and began seeing a licensed therapist. We also engaged with the International Pemphigus and Pemphigoid Foundation (IPPF), and they were the greatest help. I highly recommend that all new patients check out the IPPF’s Facebook page, Twitter account, website, and other amazing resources.
I tried to stay busy throughout the day while I was at home. Certain medications like steroids can play havoc on your sleep schedule. I worked with my doctor to identify medications that helped with itching, sleep, depression, anxiety, and mood swings. I found that taking my oral steroid medications early in the morning didn’t impact me as much at night during bedtime. Additionally, it also worked for me to take a low-dose oral antihistamine (Benadryl) twice a day.
I found that sleeping on a couch with light sheets and soft pillows helped me stay in a supported sleep position, which prevented me from rolling around all night and damaging my dressings. If I couldn’t sleep, I had a list of activities to do while not waking up my family. I also had medication if I couldn’t sleep or got too itchy. I recommend keeping your house (or at least a room where you spend your time in) cool, quiet, and filled with various activities like TV, movies, and books. This may be helpful during bedtime, and also relaxing on a bad day when a distraction is needed.
I used the Family Medical Leave Act (FMLA) to take time off from work. I stayed in constant contact with my employer, and as a result they were very supportive. I was able to return back to work several weeks later part-time, and then full-time. This was a decision that I made with my family and entire care team.
Over time, the long hours of wound care, special menus, dental care, sleepless nights, and meltdowns lessened since my diagnosis in February of 2016. Somewhere in the process, I learned what worked, developed new ways of coping and managing, and utilized resources. My family and I are very well-informed about bullous pemphigoid (BP), and we’re a part of the IPPF community. We’re not only living with BP–we’re thriving.
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.