When I was initially diagnosed with pemphigus vulgaris in 2011, there was very little information online. With the exception of the International Pemphigus and Pemphigoid Foundation, most of the listings pointed to scientific periodicals, and offered little in the way of personal stories. I wanted to know what it was like to live with pemphigus vulgaris? What could I expect? If the online pictures were any indication, I had a painful journey ahead. Did everyone have the same outcome with this rare disease?

Disclaimer:  I’m not a doctor or nurse and the information below should not be used as a substitute for medical advice or treatment. I can’t offer any guarantees that what has worked for me will work for you. Always consult with your own doctor before reducing or discontinuing your medication.

Pemphigus vulgaris is a rare autoimmune disease that causes blisters and erosions of the skin, scalp and mucous membranes. The disease often starts out as thin, clear blisters usually in the mouth. As the disease progresses skin on other parts of the body may also be affected. Blisters rupture easily leaving painful erosions.

Although these are the classic symptoms you typically read about online, I wasn’t aware of any blistering when I first began experiencing symptoms. Instead I had an assortment of seemingly unrelated issues. I remember feeling a stinging sensation in my throat which I attributed to excessive coughing from a cold. There was the soreness on the sides of my tongue , that made talking difficult, and inflamed gums that bled when brushed and appeared to sag in some areas. I also noticed what felt like two small scabs in my scalp.

A year or so after these initial symptoms I noticed a blood-filled blister near one of my molars while eating. I mistook it for an abrasion caused from biting down into my cheek. But as it grew in size I became more and more alarmed. I mention this because not everyone notices the classic signs at the initial onset of there disease. Even after being diagnosed I remained in denial because my symptoms didn’t fit what I considered to be the classic appearance.

There is no known cause of pemphigus vulgaris, though stress is a known trigger for pemphigus vulgaris flare-ups. In my case, I was under an unbelievable amount of stress shortly before the initial onset of my disease. 

Stress and other environmental factors are known to trigger the onset of autoimmune disease in susceptible individuals. While genetic factors  can put you at higher risk for developing an autoimmune disease, environmental factors play an important part in whether or not you actually develop an autoimmune disease.

In rare instances the onset of pemphigus vulgaris has been linked with certain drugs such as penicillamine and ACE inhibitors.

Autoimmune diseases, in this case pemphigus vulgaris, occur when an overactive immune system attacks the body it was meant to protect. With pemphigus vulgaris the immune system attacks proteins that bind skin cells together.

As with all autoimmune diseases, pemphigus vulgaris isn’t contagious. You can’t catch pemphigus vulgaris by being near someone who has the disease, or from drinking from the same glass.

There is no known cure for pemphigus vulgaris. However many patients have achieved remission with the help of certain medications.

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Immunosuppressants as the name implies are drugs that suppress the immune system. These medications have proven to be effective in keeping pemphigus vulgaris flare-ups under control. Some common medications for the treatment of pemphigus include Prednisone (a steroid), Mycophenolate (also known as Cellcept) Azathioprine, Methotrexate and Rituximab. 

Back in the early days of my disease when I had more flare-ups, my doctor prescribed Mycophenolate to take along with my Prednisone, with the hopes of one day taking me off of the Prednisone. After a year or so I had to discontinue using Mycophenolate because of all the weird side effects it gave me. Thankfully around this time I had begun to notice that there was a direct correlation between certain foods that I ate and my flare-ups. When I avoided those foods I had less flare-ups.  

Currently I take a very low dose of Prednisone each day (2 mg). I also “swish and spit” with Dexamethasone, a liquid solution, as in recent years, my flare-ups have been confined to my mouth. To help prevent against flare-ups in the back of my throat, I also gargle with the Dexamethasone. While I “swish and spit” twice daily, I only gargle once each day. I found that gargling twice daily caused the back of my throat to feel rough. Also I developed thrush when I gargled twice daily.

In any event I find the Dexamethasone to be a perfect alternative for me rather than experimenting with other stronger medications, particularly because my disease is mild. Why kill an ant with a hammer when a tissue will do just fine?

I find treatment to be trial and error, as there is no one-size-fits-all approach. What may work great for one person may be totally ineffective for another.

I’ve lived with pemphigus vulgaris for over a decade now, and somehow, I’ve managed to avoid severe flare-ups. In fact, while the disease initially impacted my scalp at the onset, flare-ups have been confined to my mouth, and they have been extremely mild. I attribute my success in part due to the lifestyle changes I summarized in Autoimmune Arsenal. 

I’ve also come to the realization that my body is very sensitive to foods, hair care and skin care products. Keeping my skin happy has become a subtle obsession. I say “subtle” because I’ve been doing the suggestions in the list below for so long, it has become second nature to me. Hopefully one or more of these suggestions will resonate with you. 

1. Avoid harsh chemicals in skin care and hair care products like parabens, SLS (sodium lauryl sulfate), and formaldehyde. 
2. Use a gentle soap.
3. Use gentle, moisturizing skin care products with as few ingredients as possible.
4. Use a mild toothpaste. Toothpaste with a strong, minty flavor can leave your mouth feeling raw. I had to give up my favorite toothpaste that I had been using since I was a kid because it caused my gums to sting and sag.
5. The same applies to mouthwash. 
6. Use a soft toothbrush with gentle strokes. 
7. Try visiting the dentist every 3 months instead of every 6 months. I find that going more often prevents excessive build-up of plaque. Excessive plaque build-up means the dental hygienist will have to work that much harder to remove it. Even if you don’t have active flare-ups, the digging and scraping can leave sensitive gums in a painful, bloody state.
8. Monitor your symptoms when changing hair care and skin care products. If you begin to flare up, discontinue the product immediately.
9. Avoid foods and topical products you may either be allergic or have a sensitivity to. Years ago I had flare-ups in my scalp that seemed to worsen as time went on. I didn’t realize at the time that I had a severe allergy to shea butter, which seems to be in most shampoos and conditioners these days. Since discontinuing those products, my scalp has been fine. Every so often other products have caused mild symptoms which disappeared when I discontinued use.
10. Avoid consuming foods that contain gluten and dairy. Gluten and dairy can be inflammatory.
11. Avoid spicy foods, hard foods, acidic foods and foods with sharp edges like potato chips and bacon while having active oral flare-ups, or if your mouth just feels sensitive. One workaround I found for eating bacon: breaking it up in pieces and adding it to eggs just before scrambling. This tends to soften the bacon. Check out Best Nutrient Rich Soft Foods for Managing Painful Mouth Sores for suggestions.
12. Be careful with seasonings and seasoning blends. I personally react to foods that contain too many seasonings. Also seasonings like black pepper, chili powder and curry can cause me to flare, especially if my mouth is already sensitive. Some seasoning blends contain gluten, so I avoid blends for this reason. If the seasoning blend sounds tasty, I’ll read the label to see what ingredients it contains. Then I’ll buy the ingredients individually if I know I can tolerate them. 
13. Pay close attention to how you feel while eating, particularly if you tend to get oral flare-ups. If a particular food triggers a flare-up, you may want to stop eating that food. For me, I noticed the longer the exposure to the offending food, the greater the flare-up. The reverse has also proven true for me. 
14. Certain foods can be common triggers for people with pemphigus vulgaris. This includes onions, garlic, and foods high in tannins like berries. However, foods that are problematic for some might be harmless for others. For example, I react to foods that are high in FODMAPS. This includes wheat and other grains (which happen to contain gluten), onions, and some fruit. Ultimately you will have to use trial and error to determine what you’re reacting to.  

My oral flare-ups are always the result of something I’ve eaten. If I immediately stop eating the offending food at the first sign of oral discomfort, I can minimize the flare-up. Discomfort may feel like a pinching or a stinging sensation or what feels like a raised area where a blister is starting to form.  Rinsing any remnants of the food out of my mouth or drinking a glass of water if the discomfort is in my throat helps to minimize the effect.  This only works for me if I stop eating the offending food at the first sign of discomfort. For the next few days I eat a bland diet and I gargle and rinse a little longer with the Dexamethasone. While I can’t guarantee this will work for everyone, this has been pretty effective for me. On rare occasions I may need to increase my Prednisone for a few days, then taper back to my normal dose. 

Ultimately, when I began paying closer attention to my body, responding to the subtle and not-so-subtle indications of an impending flare-up, I got better control over my flare-ups. Also by following the steps in the Autoimmune Arsenal I greatly reduced the amount of flare-ups. Now I barely get flare-ups at all. I’m wishing the same for you. 

To learn more about pemphigus vulgaris, visit the International Pemphigus & Pemphigoid Foundation.