Early signs of skin involvement?

[Nw6London]

Hi all, recently diagnosed in April 2022 with diffuse systemic sclerosis. Raynauds since 2020, Telangiectasias and scl70 +ve. No organ involvement at baseline. I'm interested in if you do have skin involvement (of the hands ideally), how this presents itself in the very early stages. I do not currently have any visible skin involvement, but over the last 2 months, the skin on my right hand has become very tight. As in noticeably tighter from the wrist to roughly 4 inches down my arm; around to the base of my thumb and over to my little finger. There is pain but not acute. Pinch test is also normal.

Currently unmedicated and in the wait and see phase. (Although on glucosaminesulphate for osteoarthritis on my left hand). Wondering whether there is anything I can do to relieve the tightness and burning. Has anything worked for you and were there any signs of skin involvement before it appeared? Thanks so much in advance

[positivedaybyday]

Hi,Welcome to our sanctuary site! So sorry you've been diagnosed with Diffuse Systemic Sclerosis, especially at such a young age!

The issue for us all is that each case is individual as to its severity & no two cases are alike.

Watch out for subtle changes & make a note of strange things which may happen to your body however insignificant they may appear. Having this written diary of events is very powerful when you visit your Consultant. If you feel things are moving fast try to obtain an earlier appointment.

Mine started with burning hands which had a pins & needles sensation & a feeling of numbness. This was early 2017.

Then when trying to bite on an apple my mouth didn't seem to open as wide. This was followed by strange sensations in my ankles, legs & calves. Walking was becoming very difficult & I was very breathless.

After two weeks my hands returned to normal but my feet followed the same pattern.

By late July I was in a bad state. I saw my Consultant in late July, early August. She specialised in Diffuse Systemic Sclerosis which is so important! I was immediately taken in hospital for assessment & pain management & then began multiple stays in hospital.

As my skin tightened my legs were the worst. So itchy & red raw. Nothing seemed to take this madness away.

I tried multiple ointments & creams, a lot of which were very oily & heavy.

Finally Cetraban ointment in a tub helped relieve the itching.

By this stage I found it very difficult to grasp with my hands so the cream in a pump action was a non starter.

I had numerous visits to the Dermatology department to try & help the situation.

This involved various creams in luding a steroid cream & a lot of bandaging.

When you visit the Consultant they will probably conduct a Roden score test. They measure 17 areas on your body each with a top score of 3. A 3 score is the worst & an indication the skin tightening is progressing.

Mine progressed all over my body apart from my back & an area of 2/3 inches across my stomach.

I think on my first visit my score was low 20's. This increased rapidly to 48 by November which was critical as the top & worst score is 51.

I was placed on Microphenolate which is used to control the progression of the disease at the end of September. In my case it didn't work. I was similar to you with no organ involvement. Due to this & my extreme case I was given 2 options:

1. Continue on the Microphenolate & hope at some stage it would kick in

2. I could be put forward for Stem Cell Treatment & if I was a suitable candidate & passed the rigorous tests beforehand it could be a lifeline.

I took the 2nd option as I couldn't go on as I was & I was very lucky:

A) In being offered this option

B) Successfully passing the tests

C) Had the Stem Cell treatment May 2018.

Now, my case was extremely severe, hopefully your case will react favourable to medication & be less severe.

It is a lottery though remaining positive throughout your journey is exceptionally important.

Involve your family & friends at each step so they can give you support.

It's now 4 years on & my skin is back to normal.

I wish you a good outcome on the start of your journey & a BIG BIG hug! Xxc

[Barnclown in reply to positivedaybyday]

Thanks for every detail positivedaybyday (love your forum name)!

Hope Nw6london doesn’t mind me asking you 2 questions:

just checking: do you mean mycophenolate (instead of microphenolate?). If you do mean mycophenolate, what dose are you on?

And also: do you have Raynaud’s too? If you do, are you on prescription meds for this?

Wishing you every best wish 💞💞💞💞 Coco

[positivedaybyday in reply to Barnclown]

Hi,Silly me, yes it was Mycrophenolate but I wasn't on it long as it didn't help me. I was on 1000 mg twice daily & then taken off it prior to my Stem Cell treatment.

The secondary Raynaulds came on probably October 2017 with my fingers going irreversibly bent. I was on Nifdipine 30mg twice daily. This drug became difficult to source so I now take Sildenfil. I started on 3 tablets of 25mg three times per day but reduced it to 2 tablets in the summer as I had palpitations & quite nauseous.

I did originally try specially made hand restraints from the hospital to hopefully stop the bending but this didn't work. At least they haven't gone claw shaped!

I swim twice a week which I find dies help with hand movements.

Please come back to me if you have any further questions. Xxx

[Barnclown in reply to positivedaybyday]

thanks v much: your sharing here is helping so many! I’m very impressed & fascinated by how philosophically you’ve come through all this very sophisticated tertiary treatment. It’s really special!

Just to give a bit of background Am 69 & my autoimmune primary is infant onset lupus with several secondaries inc severe Raynaud’s , but also with features of scleroderma & I’m basically on the sort of meds many need for scleroderma: eg 1000mg myco + 10mg pred + of course hydroxy daily for the AIDs…& 150mg sildenafil + losartan 25 mg daily for the Raynaud’s. Also I’m seroneg for all my autoimmune conditions because I also have a childhood onset Primary Immunodeficiency Disease (panhypogammaglobulinaemia, aka Antibody Deficiency Disease) which means I simply can’t make antibodies to anything, including vaccines. So I reckon I’m on the right meds for my various scleroderma-like manifestations…until my consultants feel they need to take a closer look at that aspect of my case 🤷🏼‍♀️

[Nw6London in reply to positivedaybyday]

Thanks so much for your very detailed response. 😊 you're absolutely right about the disease presenting itself differently in individuals. I've maintained a log with pictures of symptoms since my first raynauds attack. It's hard to not be paranoid about any twing and twang in the body though. Lol Wishing you a continued sustained stable journey.