CDM2017

I have EB (acquisita) and didn't know there was an awareness week! It took 6 years to get a diagnosis and I thought I might lose my mind. I finally have it pretty well controlled, and have even had some healing, which I didn't think would ever happen.

Still, the treatment is a whole lot of meds with a whole lot of side effects, and the idea of losing insurance or ending up with a bad policy keeps me up some nights.

I'm curious about others in your life. Who knows you have it? How has it affected relationships?

My subtype is adult-onset, which is pretty rare even for EB, and while most get blisters across the trunk, hands, and feet, mine are mostly on my legs with some spread to the feet and arms. I saw countless dermatologists, because my skin was getting awful. They never helped. I was bandaging when I could, but wraps never stayed on because they couldn't be tight enough and it wasn't long before there was no clear skin to apply tape to.

I was straight up miserable, depressed, and in constant pain. A chance encounter with a plastic surgeon for a mole removal led to my current doctor, a rheumatologist. The surgeon said she had no idea what was up with my skin but she knew someone who could figure it out.

And he did. He started with high dose steroids and then tried a bunch of different things to help get it under control. I was on 8 different meds, including weekly injections of methotrexate, high levels of prednisone and dapsone, as well as several anti-inflammatories and antihistamines. It helped but I still had constant lesions.

Then, last year, he found some case studies using rituxan, a drug often used alongside chemo. There had been some actual clearing and healing. It's not great stuff for you, the possible side effects were scary to read and it leaves your immune system crushed for a bit after each infusion, but it's changed my life. I have to have 3 rounds per year, each round is 2 infusions 2 weeks apart, 5 hours each. There were some hoops to jump through for insurance because it's off label. But my doctor is awesome and stuck with it.

Now, my skin can often take a small scrape without ripping apart. I'm on 5 regular meds instead of 8 and I have days that are actually pain free (there's usually still some pain but less than ever before and some days I don't register pain at all). It's still fragile but so much better than it had been.

On the flip side, I had to push back treatment because of covid and I couldn't handle the pain when the lesions started coming back. It had originally come on pretty gradually and then I'd had it for years so it became my norm. Then it returned after months and it became almost impossible to do anything. I'm back on track now but it really showed me how essential the rituxan has become.

That's a pretty good idea. I do have some pictures from different time periods. It really is night and day. I'll have to get some video now, when it's doing better, to compare to if it gets bad again.

I'm hoping it'll be harder to deny it in the future just because my doctor has so much evidence that it works. But this is the USA so I know it's not that easy.

I did have biopsies, and the results of 1 of them led my current doctor to the diagnosis. I don't know what was wrong with the dermatologists, and I had a bit of anger I needed to work through once I knew what the condition was.

They told me the biopsies were inconclusive. They sent my records to places like NYU (who apparently first suggested EB, about a year into symptoms) and Johns Hopkins. I attended a meeting of the New England dermatology group, the exact name escapes me now. Dozens of doctors read my chart and came in to look at my skin and ask me questions. None of them had any suggestions. I don't know what happened, I followed up with the dermatologists dozens of times over about 3 years, before giving up.

Maybe they disregarded it because it's so rare and they just thought "nah, surely that's an error." I read later how the prognosis is much better if you start treatment within a year and frankly, even contacting them at that point would have just been bad for me.

So I can't explain why it took so long and my mental health is better served by looking forward from here.

My EB started as an adult, and I suddenly had a TON of cavities. I was ashamed of it, and am still pretty self conscious. It was years before I was diagnosed and even then I didn't hear about the effects on teeth right away.

So pre-EB, very few tooth problems, and post-onset, many tooth problems. It anecdotal but I feel better seeing others have the same issue now.