May 2020, post correctional procedure.

Erdheim-Chester Disease – Typical presentation

I thought that a good place to start might be to discuss the typical presentation of ECD, because that is how most people who suffer from it might see the disease as it affects them. In my case, the presentation is not typical, and I am basically asymptomatic.

And that is quite typical of me: I am uniquely unique, if I may put it that way.

In many ways it presents as if it’s a cancer, but ECD is not a cancer. It’s a cancer imitator. It manifests itself with the over-production of cells, which is what a cancer does, but I am told that it does not spread, which is what a cancer will do.

Curiously, I also suffer from Psoriasis which also manifests in the overproduction of cells, and specifically for Psoriasis, skin cells. But I am assured that despite what may seem like some sort of a similarity, no such similarity exists, and they are very different ailments.

The typical presentation for ECD is in our long bones. The shins, for instance. It will be seen in the shins, and it will cause a patient pain in the legs. This can be seen when one undergoes a bone scan. I do not appear to suffer from this, and my bone scans for this were negative. My full body scan was also negative in this regard.

Another typical, but less common presentation, is in the orbit. That’s around the eyes, and that’s where I have this. It’s not too painful, but it’s also not good, because it’s having an adverse affect on me, my vision, my ability to work effectively, and my lifestyle and my quality of life.

The diagnostic processes, as you shall see, have been very frustrating for me. It’s been a long process to get to where I currently am, and as I write this, I am still not 100% convinced that this is what I have.

But it’s where I am, and there are some factors that absolutely point to me having ECD, even though I am basically asymptomatic.

This photo shows the point where the problems started to manifest themselves, in May 2020, and thus it might be described as where official diagnosis really started, but the problems started much earlier, in September 2019.

This photo is absolutely not a typical presentation.