Cutaneous mucinosis/ lichen myxedematosus?

Zizzle · Post by **Zizzle** » Wed Sep 28, 2011 9:38 am

Some of you may recall I had an autoimmune flare after the birth of my son 7 years ago. It started with a rash on my hips (triggered by metal grommets on tight jeans - nickel allergy), and soon covered my hips, buttocks and back. It lasted for almost 12 months, and only went away after I weaned. It was mildly itchy (but very uncomfortable), looked like goosebumps on sunburn, and eventually developed purple centers (vasculitis). I also had severe sun sensitivity and a malar rash on my face. I went to an incompetent dermatologist who wasted my time with creams, but eventually did a biopsy which "didn't point to anything specific, just excess mucin deposition under the skin."

I later learned this is the definition of cutaneous mucinosis (a.k.a papular lichen myxedematosus)!!! "A skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides characterized by the occurrence of waxy, flesh-colored papules"

She finally also tested my ANA level, which got the ball rolling into rheumatology...and fewer answered questions there. Just predictions of lupus, RA or hepatitis down the line. I had IBS symtoms then. The MC started 2 years ago.

Anyway, I'm noticing I have these flat, permament "googebumps" on several areas of my upper arms and upper legs. They have the familiar feeling of the old rash, without the waxiness and itchiness. I've had them in some form for a long time, but the areas seem to be growing. Does anyone here have this? Is this grounds for a doctor consultation? I see this is associated with lupus. One man with few other symptoms turned out to have lupus nephritis without abnormal kidney lab values. Yikes.

http://dermatology.cdlib.org/102/case_p ... s/hsu.html

Cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the dermis or within hair follicles. They can be divided into two groups: (1) those in which the mucin deposition is a distinctive histopathologic feature that is manifested as a clinically specific lesion; and (2) those associated with diseases with histopathologic mucin deposition as an additional finding. Papular and nodular mucinosis associated with either systemic or cutaneous lupus erythematosus (LE) is a rare condition with less than 40 cases documented to date. They have been reported either as part of the skin findings in SLE or as an isolated cutaneous form of LE. Red-purple to skin-colored papules and nodules, and in extreme cases plaques, are often present on the trunk, arm, head, and neck. Histopathologically, there are abundant mucin deposits in the dermis without the microscopic features of LE. Although mucin deposition is a common finding in LE, it is rarely present in sufficient quantity to produce clinically visible lesions.

The etiology and pathogenesis are still unknown. The documented cases have highlighted possible associations with the onset of PUVA photochemotherapy and immunosuppressive therapy with prednisone [1, 2]. One study has postulated a mechanism in which the production of glycosaminoglycans by dermal fibroblasts is increased and thought to be stimulated by a factor (or factors) in the patient's serum that are as yet unidentified [3]. Underlying vasculopathies or vascular changes are thought to be closely related to the mucin deposition [4, 5].

http://dermatology.cdlib.org/103/NYU/ca ... 103n6.html

Is there any relationship to MC, or am I in lupus territory alone? Incidentally, I was not diagnosed with lupus 7 years ago becuase I was missing a few of the key autoantibodies. Am I just being a hypochondriac?

Zizzle · Post by **Zizzle** » Wed Sep 28, 2011 9:59 am

Interestingly, this condition is also common in thyroiditis.

Post by **tex** » Wed Sep 28, 2011 12:16 pm

If it's associated with MC, it would almost surely have to be by way of mast cell involvement, IMO.

Do you have thyroid-related symptoms or antibodies?

Tex

Zizzle · Post by **Zizzle** » Wed Sep 28, 2011 10:49 pm

I tested negative for thyroid antibodies 7 years ago, and thyroid hormone levels have been normal ever since. My only associated symptoms are weight loss (5 lbs in the last 4 months without limiting fats or calories). I'm high school weight now, 118, and am having to buy a new winter wardrobe, since i've lost 10 lbs in the first year of GF/DF eating. I hate shopping. I'm also cold all the time, but I attribute that to my new lack of insulation. I guess those are opposing symptoms, so I figure they are not thyroid related. I haven't done much exercising this year, and i wonder if I'm losing muscle too. I plan to start weight bearing exercise ASAP.

sarkin · Post by **sarkin** » Thu Sep 29, 2011 7:05 am

Z,

You might increase your protein portions a little bit, too. Made a big difference to me - my weight went back up, and seems stable.

I don't think you have to overdo it, or do a lot of weighing or measuring. I just try to eat a meat/fish portion more like what my husband would go for, and less like what "former me" used to eat.

I hate shopping, too.

S

Post by **tex** » Thu Sep 29, 2011 8:17 am

Thyroid issues commonly show conflicting, contradictory symptoms. The weight loss could be due to MC.

Tex

Zizzle · Post by **Zizzle** » Mon Oct 24, 2011 11:51 am

So I did a little more research on my Lichen Myxedematosus/Papular Mucinosis skin condition, and I'm amazed to report THERE IS A GLUTEN CONNECTION!!

At first I freaked out to see it can be a precursor to multiple myeloma (an incurable blood cancer). But I dug deeper. Turns out, most patients with the skin disorder have the same defecttive paraprotein in their serum.

From Medscape:

Lichen myxedematosus is usually a chronic disease. Although most patients have a monoclonal paraproteinemia, they rarely have associated multiple myeloma.

So I was gearing up to ask to get some testing done for this monoclonal paraproteinemia (Monoclonal gammopathy of undetermined significance), when I came across this:

Celiac Disease and Paraproteinemia (Serum Monoclonal Proteins)

http://www.celiac.com/articles/21542/1/ ... Page1.html

A study looked at samples of serum from multiple Myeloma patients. In 35% of the samples the myeloma monoclonal proteins had antigliadin activity, and migrated just like celiac anti-gliadin antibodies when subjected to Electrophoresis. Monoclonal Gammopathy (MGUS) is a precursor stage to Multiple Myeloma, with the same or very similar sort of monoclonal proteins as in multiple myeloma, and converts to it at the rate of about 1.5% per year. Therefore if one lives for 20 years after diagnosis with MGUS, one has a 30% chance of ending up with deadly, so far incurable, multiple myeloma, which is a cancer of the bone marrow and blood. It was postulated by the researchers that multiple myeloma may actually be an end result of untreated celiac disease.

In another publication, a study that showed that when a patient with MGUS and Celiac Disease was put on a gluten-free diet the monoclonal proteins entirely disappeared by the end of 3 years!

The ramifications of this are that everyone with Celiac Disease really should undergo testing for MGUS/Myeloma which can be associated with various autoimmune diseases, increased rate of osteoporosis, and neuropathy, or no symptoms at all! Likewise all MGUS patients should be tested for celiac disease, which again can be associated with various autoimmune diseases, increased rate of osteoporosis, and neuropathy, or no symptoms at all! Do you see the similarities?

I suppose I'll discuss testing for MGUS when I see my new super-doc in November!

Then again,

Several other illnesses can present with a monoclonal gammopathy, and the monoclonal protein may be the first discovery before a formal diagnosis is made:

Multiple myeloma
AIDS
Chronic lymphocytic leukemia
Non-Hodgkin Lymphoma, particularly Splenic marginal zone lymphoma[3] and Lymphoplasmocytic lymphoma
Hepatitis C
Connective tissue disease such as lupus[4]
Immunosuppression following organ transplantation
Waldenström macroglobulinemia
Guillain-Barre syndrome[5]

So I'm stumped. But I intend to find out more!

Post by **tex** » Mon Oct 24, 2011 12:21 pm

Zizzle,

Remember that MC is a connective tissue disease, (at least the medical description of CC has always included that observation).

It was postulated by the researchers that multiple myeloma may actually be an end result of untreated celiac disease.

That kinda takes away some of the appeal of dragging one's feet about starting and maintaining a GF diet, when it's an indicated treatment.

Tex

garina · Post by **garina** » Mon Oct 24, 2011 12:39 pm

Hi Zizzle,

Interesting about MGUS. I had been diagnosed with MGUS back in 1999 and have been on a regular testing cycle twice a year to see if anything progressed. I did have an M-spike at the time, then with the Freelite test, which is very sensitive, it does show a slight Kappa IGA increase, with a slight increase in K/L ratio, but nothing to the extent that has to be treated, at least....yet. It has been my worse health fear for years. MC is horrible, but Myeloma is destructive.

"In another publication, a study that showed that when a patient with MGUS and Celiac Disease was put on a gluten-free diet the monoclonal proteins entirely disappeared by the end of 3 years!"

I do hope this publication will hold true and going gluten free for 3 years entirely eradicates the worry of Myeloma. It really is encouraging for us with MGUS.

Thanks.

garina