Lipedema is a classically thought of as a congenital fatty enlargement of the legs almost exclusively seen in women by the third decade; two cases have been reported in men(1, 2). According to an epidemiologic study by Földi E and Földi M(3), lipedema affects 11% of the female population. Of women with lymphedema, estimates are that 15% (4) or 8% to 17% have lipedema as well(5).
Lipedema was initially described by Allen and Hines(1, 6) in 1940; its etiology remains unknown and it remains under-diagnosed. In 1951 Wold et al.(1)analyzed 119 cases and provided the diagnostic criteria for lipedema:
(1) Almost exclusive occurrence in women
(2) Bilateral and symmetrical manifestation with minimal involvement of the feet
(3) Minimal pitting edema; the Kaposi–Stemmer sign is negative
(4) Pain, tenderness on pressure
(5) Increased vascular fragility; easy bruising
(6) Persistent enlargement after elevation of the extremities or weight loss
And I have added:
(7) Arms are affected 30% of the time(7)
(8) Hypothermia of the skin.
(9) Swelling worsens with orthostasis in summer
(10)Unaffected by caloric restriction(8)
In my practice, I see many people with lipedema, familial multiple lipomatosis (FML), Madelung’s disease and Dercum’s disease (adiposis dolorosa). The description of Type III multiple symmetric lipomatosis (MSL; Madelung’s disease) and representative pictures of Type III MSL(9) are that of lipedema(10). Synonyms for lipedema also include adiposis dolorosa (Table 1). However, according to Cornely(11), the trunk, hands and feet are not involved in lipedema “Thus, lipedema differs clearly from Dercum’s disease”. Finally, many people with FML can go on to develop signs and symptoms of lipedema.
Table 1. Synonyms of Lipedema
Lipomatosis dolorosa of the legs
Painful column leg
Painful lipedema syndrome
I am not sure we can make such a distinction between fat disorders at this time. In fact, I think these data suggest the need for clinical clarity in differentiating these disorders and a need for a means of testing to distinguish them. I personally think that adiposis dolorosa Type I is the same thing as lipedema.
So what is going on in lipedema at the level of the tissue?
Since lymphedema is minimal in early lipedema (meaning the ankles and feet do not swell with fluid until later in the disease when the lymphatics begin to fail), the limb swelling is due, in part, to hypertrophy and hyperplasia of fat cells(12). The gross description of the fat in lipedema is similar to that of MSL with copious amounts of watery fluid upon incision of lesions and of ‘free fluid fat’ in biopsy specimens(13). The increased fluid suggests a basic abnormality in the interstitial transport and exchange of fluid, with resulting disruption of fat and accumulation of fluid due to increased hydrostatic pressure(14, 15). So this means that fluid is either entering fat at a faster rate or leaving fat at a slower rate. In fact, both are likely to be true.
I think lipedema has problems in both lymphatics and blood vessels:
First: Visualization of lymphatic vessels on a gross level by lymphoscintigraphy was normal in women with lipedema(12). Ok, so the big lymphatics seem to be functioning ok in early lipedema.In another report, they compared lymphatics in lymphedema to those in lipedema and found that the spread of dye in the microlymphatic network is enhanced in lymphedema but unremarkable in lipedema(16). Indeed, microlymphatic occlusions are absent in lipedema– Nothing is blocking the flow of lymph in the lymphatics – So early in lipedema, your lymphatics have normal flow; this is a problem because you have edema in your fat that you need to get rid of. In fact, Lipedema is always accompanied by a dynamic insufficiency of the lymphatic system(7).
Second: Here is the kicker, smaller lymphatic vessels in the subcutaneous fat were abnormal by lymphangiography and leaked(17). I am not clear that this problem is confined to fat.
Third: As lipedema progresses or is simply present for a longer period of time, microaneurysms appear in the lymphatics in the in the skin of lipedema patients by microlymphangiography(16) – this means that your lymphatics are stretched out and at risk for leaking
Fourth: But wait. Another report found increased lymphatic transport by functional scintigraphy in lipedema compared to controls and lymphedema(18). By the fifth decade (so after 20 years of having lipedema), values (of lymphatic flow) slightly decrease under the lower limit of normal. This situation may result in lipolymphedema(19-21) – The aneurysms in the lymphatics ultimately leak so now there is lipedema (fluid in fat from damaged blood vessels) and lymphedema (leakage from lymphatics) causing lipolymphedema. Also, when did the increased flow start
Fifth: In lipedema, on examination of tissue samples, they found dilation of subdermal blood capillaries, fibrosis of arterioles, fibrosis and dilation of venules, hypertrophy and hyperplasia of adipocytes(22, 23) – this means that the capillaries just under the surface of your skin are wide and can be seen as little red marks on your skin if you look closely enough, that the walls of small vessels have increased connective tissue (fibrosis).According to German, Italian and Swedish literature, lipedema starts as a microangiopathy. This means that there is damage to blood vessels and they leak their contents into fat. This is why you bruise easily – your blood vessels, at least in your fat and skin are not normal. Something is wrong we just don’t know what!
It could just be that increased fat in lipedema recruits macrophages that inflame blood vessels leading to leakage, edema and taxation of the lymphatic system. We and others have found increased macrophages in lipedema that surround blood vessels and form oil cysts(24). So in this case, we need to find out what makes the fat grow to such an extent.
Leaky lymphatics are seen in the homeobox gene Prox1 heterozygote mice(25).
Lymph stimulates adipogenesis in culture.
Abnormally clumped elastic fibers also have been seen in lipedematous tissue(26).
Free fatty acids may be altered in both blood and the lipedema tissue(13).
My Standard of Care for Lipedema
1) Complete Decongestive therapy – This includes decongestive massage, wrapping and garments. A trained therapist in your area can be found here: http://www.vodderschool.com/
and here: http://www.lymphnet.org/resourceGuide/manualDrainage.htm#CA
2) One of the first things I like to suggest for lipedema is to help the immune system function properly. It is well known that lipo-lymphedema or lymphedema reduces local immune function, increasing the risk of cellulitis. The immune system is also overwhelmed by the amount of inflammation in lipo-lymphedema, therefore, I usually start cimetidine as an immune modulator. The dose is 400mg twice daily. This is not a user friendly drug because it affects liver metabolic enzymes and therefore levels of other medications. I would recommend you discuss taking this drug with your primary doctor. An alternative is beta glucan 1,3/1,6 from the yeast cell wall (yeast Beta 1,3-D glucan) to boost your immune system. There are thousands of papers in the literature (pub med) demonstrating effects of Beta 1,3-D glucan on the immune system including increasing IgA in saliva (Lehne et al., 2006) and reduced post-operative infections (Dellinger, 1998); Beta 1,3-D glucan may enhance hematopoiesis, amplify killing of opsonized tumor cells and increase neutrophil chemotaxis and adhesion (Weitberg et al., 2008). The dose is 500 mg per day or more (or 2-6 mg/kg). The capsules are expensive so you need to shop around. You can find some information here: http://www.greenpathsupplements.com/beta-glucan
The problem with beta glucan is its absorption. It tends to clump when added to water, which prevents a bulk of it from being absorbed. You may try Now's version for $10. Open up and dissolve a few capsules into a small amount of warm/hot water. Use a spoon to de-clump. Drink with a few vitamin C capsules. This site may help you pick a brand:http://alternativecancer.us/betaglucan.htm. Epicor is a fermented yeast product that has beta-glucan as a main ingredient and which also has data supporting it’s prevention or improvement of colds/flu(27).
3) Selenium: Selenium has been shown to decrease edema in tissue. Selenium decreased edema in two placebo controlled trials for post-mastectomy and head and neck radiation(28, 29), it increases the efficacy of physical therapy for lymphedema, it reduces the incidence of erysipelas infections in patients with chronic lymphedema at various sites(28) (and others); it lowers oxygen radical production in part by increasing glutathione peroxidase and thioredoxin reductase; it dcreases glycoprotein adhesion molecules (eg, P-selectin, ICAM-1, VCAM-1, ELAM-1) in a dose-dependent manner(30) which may unclog lymphatic capillaries; it significantly enhanced cellular immunologic reactions improving efficacy of cytotoxic T-lymphocytes and stimulating macrophages degradation of excess tissue proteins(31). The US National Research Council has defined the individual maximum safe dietary intake for selenium as 600 μg daily and the no adverse effect level as 800 μg daily (100 μg selenium in 2 mL of isotonic solution (Biosyn Corporation, Carlsbad, California) = $1600/5 weeks. Anticipate a possible increase in pain or decreased well-being as the toxins come out of the tissue. However, if you don’t feel good taking the selenium, then stop it. You can get sodium selenite solution from Life Extension at 50 mcg per drop (you take 5-10 drops). Many other internet sites also sell the liquid. You can buy 250 mcg tablets from twin labs (try iHerb).
4) An older effective treatment for lymphedema, 4 benzo-pyrone, coumarin(32), is no longer available because of liver toxicity in some people; other 4-benzo-pyrones such as Daflon may or may not be effective(33). Daflon is as flavonoid, that has been used to treat lymphedema(34-36); it may be expensive and unlikely available by prescription. I treat lipedema with large doses of bioflavonoids. These can be purchased in powder bulk and mixed into smoothies, soup, spaghetti sauce, etc. These include horse chestnut seed extract (a very well-studied nutraceutical for varicose veins), citrus bioflavonoids, quercetin and pycnogenol as well as others. You can buy in bulk here: http://www.viable-herbal.com/singles/Herbs/s506.htm or http://eye-spy-cam.com/bunch_of_herbs.htmbut there are many other sites from which to choose. Quercetin also is known to inhibit fat growth and may help with allergies making this bioflavonoid a good choice. Divide the bioflavonoid powders so you take up to 3 grams a day. Mix up the bioflavonoids so you are not taking the same ones every day. Take smaller doses of pine bark extract (pycnogenol) such as 200 mg daily. You can buy 1000mg tablets of quercetin and 1000mg citrus bioflavonoids (but be careful – this one might be very tough on your gut so best to work up to anything that contains 1000mg of bioflavonoids). Then you can take 300mg of horse chestnut seed extract or grape seed extract three times a day to equal 3 grams a day.
5) If you have irritable bowel, make sure you wait to buy any of the citrus bioflavonoids until you are sure you do not react to citrus by forming immunoglobin G (IgG) or IgE. A blood test you can do through US Biotek can tell you what kind of foods that your immune system reacts to at a high level. It may not tell us all foods your gut is sensitive to causing your irritable bowel.
6) I like to combine the bioflavonoids with a potent antioxidant such as N-acetyl cysteine (NAC). The fluid in your tissue tends to be oxidized causing further tissue damage. The NAC helps keep the oxidation to a minimum. One to two 500mg capsules a day.
7) Enzymes such as serrapeptase and wobenzyme have also been shown to decrease lymphedema (www.lipomadoc.org). Take 20,000 international units of serrapeptase with meals or 100, 000 international units at bedtime. If you have leaky lymphatics, which you can assess by lymphoscintigraphy, taking large doses of enzymes may not be the best choice as the enzymes can leak out of the lymphatics into the tissue causing damage.
8) Liposuction - The Cure for Lipedema Fat: Liposuction at this time is the only method that we know of to remove the lipedema fat. Diet and exercise can reduce "normal" fat but the lipedema fat remains even after bariatric surgery. Surgical treatment of lipedema is available in Germany, the UK and elsewhere, usually using tumescent, or water jet assisted liposuction (WAL) lymph sparing liposuction to remove lipedema fat, under local anesthesia (not general). Research shows lymph sparing liposuction yields good long term results in reduction of lipedema pain and in stopping the progression of lipedema. (Cornely et al., 2006; Schmeller et al., 2006; Warren et al., 2007; Rapprich et al., 2011, 2012). The need for conservative therapies such as MLD and compression is greatly reduced in almost all patients, and in some cases, conservative therapies can be eliminated, after lymph sparing liposuction.
9) Networking – This is so important for you to find out what has worked for others. There is a very good chat site on Yahoo dedicated to lipedema and lymphedema that you should check out. There are also many sites on Facebook including Lipedema Sisters USA, Lipedema Unite,
My favorite lipedema sites (please let me know if I missed yours!):
1) Establishment of a grant program in New York for the study of lipedema and lymphedema:
2) A good article on lipedema: http://www.hanse-klinik.com/englisch/Lipoedema.pdf
8) Lipoedema Ladies: http://www.lipoedemaladies.com/
9) www.fatdisorders.org This website is an excellent resource for lipedema. You can also contribute to research in the area of lipedema here as well.
10) Lipoedema UK: www.lipoedema.co.uk
11) Talk Lipoedema: http://www.talklipoedema.org/
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