I have a rare autoimmune condition called Pemphigus Foliaceus (PF). And I really DO mean rare. My dermatology specialist Dr H has told me it’s so rare, that she’s never actually seen a case of it in 20 years of dermatology until mine. She wasn’t quite sure what it was to begin with, but I think she suspected she’d hit the “dermatology jackpot” with me. My words not hers – she’s a total professional and lovely with it, and I like her and trust her. It takes a biopsy to confirm Pemphigus, and yep, that’s what I’ve got.
So I’m “special.” Lucky old me. I always suspected I was and now I’ve had it confirmed by medical science.
There are two types of Pemphigus, mine which is the rarer Foliaceus version (PF), and the more common and nastier Vulgaris version (PV) – still rare, but not quite as so. The difference between the two is that the PV version also affects the eyes and mouth, and if you’re reading this and are at the stage before reaching treatment where you’ve been affected in those areas, my heart goes out to you. Hopefully you’ll be on medication (steroids) to be relieving and stabilising the condition, as am I.
There are also 6 subsets of Pemphigus and it may take a while (and I think some trial and error and head scratching) before discovering which one or combination is affecting your body. At any rate, the initial treatment is the same. Immunosuppression and inflammation reduction by the use of steroids and then possibly onto immunosuppressant drugs such as MMF to relieve the use of steroids if you’re lucky. If you’re luckier, then you’ll get moved onto Doxycycline (antibiotic) and Vitamin B3. Don’t hold your breath as to those working, but they might, and it’s a much better option than long term steroids if it works out for you.
What does having one of the two main Pemphigus family mean?
It means the autoimmune system attacks the desmosome “glue” that holds together the outer layer of skin cells by attaching antibodies to the protein (glue) which causes them to lose adhesion to the cells around them. The space is then filled by microscopic quantities of fluid and because it’s the outer layer of skin, a slight blister, redness and flaking happens, which is what I’ve been seeing. It gets worse and more widespread over time. If this itchy, embarrassing (I’ve seen the looks already as it’s now affected my face) and uncomfortable condition isn’t brought under control and the skin returned to as close to normal as possible, then the danger is infection at one of the open blister sites which could then spread and cause major complications and possibly death.
So it’s pretty serious. Don’t let anyone under sell it to you. It will get worse and kill you if left untreated.
A desmosome by the way is just a sciency term for any kind of biochemical structure that holds two adjacent cells together. Glue. It’s formed from protein plaques linked by filaments. Glue. Isn’t the human body wonderful? Now you can go around telling people it’s all held together by glue. They’ll look at you sceptically, but it’s true. Glue.
So it’s not good when the glue that’s responsible for the skin that holds all your bits inside is made unsticky by your own body is it?
Without medical science to help me, my guess is I’d be horribly dead in a year given how fast it spread once it got moving.
It was very slow to begin with, just tiny little itchy mark on my left shoulder at the end of November / beginning of December 2016 was when I first became aware of it. By the begining of January 2017 it was a little bit more noticeable, but not really any bigger. I had it looked at and first thoughts by me and my GP was a bacterial infection. Two courses of antibiotics and it had spread slightly. Over the course of the next few weeks I tried various creams, antifungals, you name it, and all this time it was spreading and getting faster doing so, moving across my shoulders and down onto my chest.
And then onto my face, which was the worst feeling.
I was finally referred by one of the GPs at my local surgery who was a dermatology specialist himself before becoming a GP. He wanted me to be checked out by the experts and have a biopsy at the local hospital.
When I first saw him, I asked what could cause something like this, and looking back thinking about it, maybe he suspected it was Pemphigus at the time because it doesn’t look like any of the more common skin conditions. He’s a smart guy and likeable. He told me something like a virus could trigger it, which is exactly how it’s believed Pemphigus starts up.
I explained in that first sitting with him that about the same time as the mark appeared on my shoulder, I had a little sharp piece of hard skin appear between by left thumb and forefinger, which I’d been told was a wart by the first GP I saw, and that I could either freeze it off myself or get one of the surgery nurses to do it. Until then I’d thought it was a piece of sand or glass finally worked its way to the surface from a childhood injury as it was sharp. But no, it turns out it was a wart, which is a hard skin structure that the skin throws around a viral infection to protect your body. Veruccas are the same thing but on your feet.
After seeing the first GP, I bought a freeze kit from the local pharmacy and 3 times tried to freeze it off and failed as it reappeared each time.
The dermatology GP who referred me that day offered to freeze it off for me there and then, which I accepted. He attacked it with much more confidence than I had, and it HURT, but I just gritted my teeth and squinted my eyes and let it go. Big brave Frank. It took another session a couple of weeks later where he shaved it with a scalpel and then re-froze the remainder off, and that’s it, all gone, not a mark there to see now. To my surprise it didn’t hurt so much the second time even though it was still a little tender from the first session.
I’m now convinced that it was the wart that started up the Pemphigus. How would you ever know what it was though? I’d never even heard of Pemphigus until recently.
The biopsy took place in June and I was told it takes 3-4 weeks for the lab results to be confirmed. Sure enough I got a phone call from the hospital saying could I come in today at 4.00pm? That’s not good is it? If it’s good news, you get a letter for an appointment, if it’s bad you get a phone call saying come in NOW.
I went in to see Dr H and she gave me the exciting news. She told me it’s so rare it’s difficult to find anything online about it, which I now see as good news because you’re only going to find genuinely knowledgable information about it, most of it cold medical science.
Until this site where I’m trying to add some humanity and easier to understand knowledge to a scary condition.
She told me about the steroids and the MMF and I think somewhere in there she told me about being on steroids for possibly years which I knew was very bad, and I think I rejected that from my mind as something I didn’t want to hear. I’ve sort of come to terms with it now and have a plan not to go down that long term route, but we’ll have to see how that goes.
I’ll detail that plan here on the blog. By the time you read this I will have probably written it all out, but not yet.