Elastosis Perfa…what?

Elastosis Perforans Serpiginosa.

The three words that a dermatologist used to describe the ring-worm-looking thing on my arm that I had struggled with for a over a year. And the first three words I was given that gave an on-paper clue to my Vascular Ehlers Danlos Syndrome (vEDS).

This had shown up on my arm one day in 2012, and was soon in not one but two spots on my arm. Ringworm. Gross.

First I tried all of the over-the-counter ringworm medications I could, with no success. After a while of trying these I went to my doctor. Two different prescription strength ringworm medications didn’t work either.

By this time, several months had gone by, and I was disgusted with myself. My girlfriend didn’t want to touch my arm. I would wear bandaids to work to try to cover it, but after seeing me with bandaids for so long it was hard to hide that something was going on.

I tried scraping and cutting it off, but it would just come back.

I tried wart remover acid several times. Again, it would just come back. So finally I saw a dermatologist.

This dermatologist made me so self-conscious. It took less than a minute for him to look at me and determine I needed to see a rheumatologist.

“What is going on with your hands? Have you been tested for scleroderma?”

“Your hair is really thinning on your scalp, you need to go get some Rogaine for that.”

“I don’t want to do anything until you see a rheumatologist.”

I tried explaining that I had already seen a rheumatologist, and all my tests came back clear years ago.

It was a no-go. Things change after several years, he told me.

So off to a rheumatologist I went. Again.

And again, all the tests came back clear. No scleroderma. No lupus. Nothing.

A few days later I was scrolling through pictures on my old computer and came across a picture of me petting an armadillo.

GASP!

That was several years prior. Maybe I had leprosy!

I call the rheumatologist. He agreed it could be a possibility, since leprosy does have a very long incubation period (2-10 years).

But nope, the test came back negative. No leprosy. DAMNIT.

I did NOT want to go back to this dermatologist. I felt so gross at this point. Scabby thing on my arm…bony, wrinkly, old-looking hands…balding apparently. But with a clean bill of health from the rheumatologist, back I went.

The first biopsy he took came back as inconclusive, so I had to come back for a second one. That was the one that did it. Perforating dermatosis, with something called Elastosis Perforans Serpiginosa (EPS) in the differential. He had it circled on the paper, and thought it fit best out of the three options.

EPS is a skin disorder where abnormal elastic tissue is basically pushed up to the top of the skin, causing reddish bumps in a linear, circular, or serpiginous (snake-like) pattern. This website has some good pictures of it. I’ve searched everywhere for a picture of mine, but it seems I avoided any pictures of my arm for the time that I had it!

So after a year and a half of trying everything to get this thing off my arm, he just froze it off with nitrogen. So simple. He warned it would scar, but I didn’t care. My body is already covered in scars anyway, no biggy! In fact, somewhere around this time my belly button ring had ripped out too. I was no stranger to scars.

He explained that EPS was pretty rare, and was sometimes seen in people with Down Syndrome or Ehlers Danlos Syndrome. He told me I should see a geneticist to rule that stuff out, and I thanked him and went on my way. A few months later I was in a geneticist office worried I had the vascular type of Ehlers Danlos Syndrome, and the rest is history. It wasn’t until several years later that I finally got the genetic test and the diagnosis that confirmed the vEDS.

Ultimately the first on-paper clue was provided to me by a dermatologist. Someone who recognized that I had a bigger issue going on, when no other doctor had taken that step back to look at me as a whole.

At the time it made me feel pretty disgusting, but now I am grateful for that dermatologist. And luckily my EPS never came back after he froze it off. Such a simple solution for something I tried to cut off or burn off myself multiple times between 2012 and 2013.

I’ve been told now it probably wasn’t actually EPS, but I’m okay with not knowing for sure what it was. It never came back, and if a misdiagnosis of EPS is what got me to vEDS several years later then I consider the diagnosis an overall success.

A real win. 😉

Update: I looked hard and found only two pictures of the EPS on my arm. It was truly difficult, as I seemed to have avoided taking pictures of it. Here is one:

Check out my other once-a-month podcast, Staying Connected, where I talk to other people with vEDS! https://translucentone.blog/category/staying-connected/

4 thoughts on “Elastosis Perfa…what?

  1. Found my sons (nephew) by same website after multiple wrong biopsy results. And yes, EPS can go away for years and come back again. I noticed something through the years in regards to my sis and her son, they only came up it seemed months or years after contrast was used to scan for health problems. Never failed! I think some vEDS patients skin reacts to it. If it wasn’t for my taking the time to search for 5 months going through pictures, we’d never know what killed my sister in childbirth and/or properly diagnosing our son.

    1. Translucent One May 21, 2018 — 1:39 am

      Wow! I’m so glad you got the proper diagnosis for your son, and I’m saddened to hear about your sister. That’s so interesting that EPS showed up after contrast for them. I’m not sure what triggered mine, but I have luckily been EPS free for several years now. Crazy that something so small provided such a clue!

  2. That is really interesting Katie. I had a similar rash two years ago which got me referred to a rheumatologist to check for Lupus after antifungals and antibiotics did not work. Wasn’t Lupus, they had no clue where that rash came from, but that’s when they noticed I was hypermobile and gave me a hEDS diagnosis. Until someone realised there are too many bleeding problems and ruptures in my family for hEDS and I got genetic testing (which has still not come back after 6 months 🙁 ). Luckily my rash eventually disappeared on its own, but I wonder if it could have been EPS.

    1. Translucent One May 28, 2018 — 2:29 pm

      I am shocked you still don’t have a result after 6 months! That is so interesting about the rash too. I was told it could go away on its own, but since it had stuck around for over a year I was really ready for him to freeze it off.

      I hope you get your answer soon!

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