Has anyone had any tests which found a connection between our scls disease and scleroderma (scleromyxedma)? Both appear to be immune system related and IVIG is apparently becoming the treatment of choice for both. Ironically, the symptoms also seem to be similar except for the leaking in SCLS. MGUS is also prevelent in both. I am going to follow up on this topic at NIH this month. Posted August 16,2010

I just checked and in neither of the latest survey articles on SCLS — the ones that are listed first and second in the Disorder Resources section of this website, and which I will be glad to send to anyone upon request to aporzeca@american.edu — do they make mention of any connection to scleroderma (scleromyxedema), though you're right that both illnesses share the MGUS similarity, so it's an intriguing coincidence. However, the survey authors hardly mention IVIG as a good treatment option for SCLS, so feel free to bring up the matter to Dr. Druey when you meet him next week at NIH!

This subject is really odd, since i just did some research on the same topic. LIke a few days ago. Since my second episode last Feb. my skin has changed overall. I also am 30 pounds heavier. I have always been small, managing to stay around 120-122 lbs. But, this weight gain has been slow and steady. i still seem to be gaining! When we first noticed it( my skin), my husband would describe it as thickening. I also have complained to you about how i swell very easily in the abdominal area. i eat prunes, figs and other dried fruit to keep my gi tract active.(another symptom with scleraderma) I met a friend a few months ago who had been diagnosed with the milder version. I really didn't give it much thought until the other day. Something just told me to check into it. That's why i find this odd, for it to come up in discussion forum right now. i have had everything tested, at my insistence. My lungs, a ct of my brain, sonogram of my ab area, hormones, liver profile, bone density, even my thyroid, and nothing shows up. The only thing that is slightly different is my hormones which seem to be showing i am now entering peri-menopauseal. I have read up on that. i do i have hot flashes if i do not use a progesterone cream on my fore arms at night I am presently on 3 anti depressants and also high bp medicine. i now battle with anxiety. I don't know if i am battling a hefty dose of messed up hormones,or scleraderma, or both? Any further info you find, please share it with us. i know i would greatly appreciate it if there is a connection. sincerely, marilyn

Aug. 18, 2010 I will follow up with Dr. Druey next week and see what he says. If you are looking for more information on scleraderma, you might try Wikipedia; it had several pages of data including a good list of symptoms and their effect on heart, lung and other organs. Do you also have the MGUS blood "marker"?

Yes, i do. i actually was diagnosed with this a couple of years prior to my first scls episode.. something showed up in my regular bloodwork, so my doctor did the electrophoresis test to further investigate the abnormal result.And obtained a positive result.

All This is a confusing subject because the terms scleroderma, scleredema, and scleromyxedema are being used interchangably, when in fact scleroderma is a separate disease often called a collagen vascular disease and a subset of autoimmune diseases. Scleroderma is not associated with a monoclonal gammopathy. On the other hand, scleredema or scleromyxedema are the same condition with different names. Only the latter - (scleredema/sceromyxedema) are associated with a monoclonal protein and then it is almost universally a specific type (IgG lambda). Neither is associated with SCLS, but the skin can become edematous in SCLS and I believe that is where the confusion sets in. Of course, Arturo is correct when he points out that s/s is associated with a monoclonal gammopathy. The response to IVIG in yet another autoimmune disease is of great interest. We desperately need more published data on IVIG in SCLS. I hope that clarifies things a bit. Phil Greipp

You're right, this can be confusing. When you said edematous (edema?), you mean swelling of tissue because of extra fluid? My question now is, are my capillaries always leaky? Do i stay in a state of extra fluid in the tissue, but when an episode occurs it gets out of hand and i need the dopamine and etc.? Still trying to figure out this whole constant weight gain?

I'm sorry Marilyn but we cannot expect healthcare professionals to make diagnoses or recommendations through an Internet community such as this one. I suggest you make an appointment to see Dr. Greipp if you want him and his colleagues to help you with your specific weight gain issue, or else you can contact him individually to have him suggest someone to see closer to your home state. As you know, in the typical case of SCLS, the weight gain is strictly temporary and is related to the fluid administered by doctors in the ER or ICU. The weight gain is reversed once the kidneys eliminate that extra fluid.

I apologize for the confusion; I was refering to the scleromyxedema disease and its associated symptoms. I, in fact have the lgG Lambda MGUS and was led down this path by the IVIG association which I also found on Rare Share under the scleromyxedema community.

Hi I have just had just had blood tests done that showed positives for autoimmune response - the tests were ANA (DNA) I am now having further tests in a few weeks as I have just had a flare up. Both my hands have become very sore in the last few months with cracked and thickend skin. It seems there may be some systemic inflamatory thing happening that could be attacking my skin/muscles/connective tissue and arteries. These flares get much much worse if I eat dairy product on a regular basis As far as the swelling/fluid build up is concerned I am a woman approaching 50 and my menstrual cycle is mesisng around but I have noticed over years that my flare ups do relate somewhat to my hormone cycle too. As I approach menopause I am finding it nearly impossible to get rid of all the fluid after and attack - I understand that one aspect of the menapause is capillary dilation - Is it possible that the menapause could contribute to this condition?