Wednesday, July 1, 2015

Lipedema, Part 4: Possible Causes and Associated Conditions

You can read the story of this mother
and daughter with lipedema here.
We have been talking about Lipedema (also spelled Lipoedema, as to the left), sometimes known as "painful fat syndrome," as part of Lipedema Awareness Month.

In this condition, an abnormal overgrowth of fat cells occurs in the legs and lower trunk, sometimes also including the arms or upper body as well.

Lipedema is rarely recognized by doctors. Often it is thought to be simple obesity, or it is confused with "lymphedema," the accumulation of lymph fluid in the interstitial areas.

In Part One of this series, we discussed the typical features of lipedema and how differentiate between lipedema and lymphedema.

In Part Two of the series, we discussed how it progresses, the different Stages of progression, and why it's so important to be aware of lipedema

In Part Three of the series, we discussed the different Types of fat distribution patterns, looked at some pictures to illustrate Type and Stage of lipedema, and detailed how lipedema is diagnosed.

Today we discuss the various theories of what might cause lipedema, as well as conditions that are often associated with it.

In later installments, we will discuss treatment options, as well as practical suggestions on how to deal with lipedema.

What Causes Lipedema?

No one knows what causes lipedema, but there are several theories floating around. More than one of these theories is probably involved.

Hormonal

Since lipedema is diagnosed almost exclusively in women and usually gets worse starting around puberty, most researchers assume it is hormonal:
As lipedema appears to be expressed most commonly at puberty, it is reasonable to assume that hormonal influence underlies the marked female limitation shown in our studies to date
Since lipedema often gets worse during times of major hormonal shifts, like puberty, pregnancy, and perimenopause, many researchers suspect that estrogen somehow plays a role in its development.

The estrogen connection is logical because usually only women are affected. However, occasional case reports of males can be found in the medical literature, usually secondary to other causes like liver issues.

On the other hand, some people with lipedema believe that males are also affected by lipedema but its presentation in males simply goes under-recognized because it does not have the classic pear shape that women get. It might be that male sufferers instead have an increased tendency towards weight gain or lymphedema but avoid the pear shape or extreme leg size that women tend to get.

It is also conceivable that males might merely be carriers of the traits, even if they don't present with symptoms themselves. Either way, it would be revealing to look in more detail at the close male relatives of women with lipedema.

However, estrogen can't be the only answer to lipedema because all women go through puberty, most go through pregnancy and menopause, yet only some develop lipedema. And there are lots of women who develop pretty severe lipedema even when they are childless or have not been through menopause yet. There has to be more to the story.

Estrogen-related events may trigger a cascade of reaction that makes lipedema worse, but it seems obvious that there has to be some other underlying issue to be triggered.

Genetics

It seems clear there is a strong hereditary component to lipedema. Anecdotally, it often seems to be handed down from generation to generation. In the research, reports of family history of lipedema ranges from 16% to 45%.

The hereditary link is not 100%, of course. Few things are. Lipedema can occur without a family history of it. But just as a tendency towards certain heights tends to run strongly in families, so does lipedema.

The picture below shows a mother and a daughter with Type III (full leg) lipedema.

The mother looks to be in Stage 3, with orange-peel texture on her skin, large fat pads on the knees, and the classic bracelet of fat at the ankle.

The daughter looks like she is transitioning into Stage 2. Her skin is mostly still smooth but starting to change, she has overgrowth at the hips, and fat pads are just starting at her knees. The beginnings of a bump at the ankle can be seen if you look closely.

Attempts have been made to document the family trees of people affected by lipedema. Although not every woman in family histories is affected by lipedema, there often does seem to be a history of at least a few women with notably big legs, hips, or arms.

This led some researchers to propose that lipedema is "a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation."

However, this has not yet been conclusively proven. At this time, no specific genes responsible for developing lipedema have been identified.

Inflammation

Some researchers now believe that inflammation plays a key role in lipedema. This is the trendy theory on many lipedema websites now, with many women promoting "RAD" (Rare Adipose Disorder) anti-inflammatory diets in hopes of halting the progression of lipedema or lessening its symptoms. One lipedema resource states:
Lipedema is...an inflammatory reaction to our environment, processed foods, high carb diets, hormonal events such as pregnancy and menopause and chronic, everyday, or event related stress.
That seems an overstatement of the evidence to me; we do not really know yet if lipedema is caused by an inflammatory reaction. And lots of other people are also exposed to processed foods, a toxic environment, and extreme stress, yet never develop lipedema. So again, there has to be more to the picture.

However, that's not to say that an inflammatory reaction could not be part of the picture. But is it really the main source of lipedema-related issues?

Many women with lipedema report that at times they have experienced a flare, a sudden MAJOR worsening of their lipedema despite no changes in diet or exercise, sometimes also with lymphedema, infection, or a change in skin texture. Some have come to call this an "inflammatory cascade."

If the inflammation theory is true, then people with lipedema may be able to minimize their condition by avoiding foods that tend to be inflammatory (dairy, sugar, and gluten are the usual culprits named), avoiding stress, and avoiding possible environmental triggers like the chemicals in plastics.

On the other hand, the field of nutrition, weight management, and alternative medicine is filled with fad diets and nutritional trends. The anti-inflammatory diet is extremely trendy these days, so it's not surprising to see inflammation proposed as the cause du jour in various diseases. It's far from proven that inflammation is the main culprit behind lipedema flares, though it could be one piece of the puzzle.

Bottom line, we should not jump to too-broad conclusions about inflammation's role without good supporting evidence. Women with lipedema may have prematurely latched on to an anti-inflammatory diet as a "cure" because it gives them a sense of control and a magical totem against the possibility of developing severe disabling complications.

Lipedema experts these days recommend many different diets, not just anti-inflammatory ones. Take the anti-inflammatory buzz with a big grain of salt, but don't dismiss it either. An anti-inflammatory diet is mostly just common sense, and it's probably good for everyone to avoid plastics and highly processed food anyhow. It's unlikely to harm anyone if it is reasonable and moderate. Women with lipedema may want to try a RAD diet ─ not for weight loss, but to see if it helps their symptoms. But don't take it as a guarantee that it will help.

On the other hand, no one is obligated to follow an anti-inflammatory diet just because they have lipedema. Some lipedema groups lay a pretty heavy guilt trip on anyone not following an anti-inflammatory diet, despite the lack of proof of any benefit from it. Remember, at this point, its value is unproven. In the end it may be shown to have some effect, but at this point, it's all conjecture.

Inflammation is an intriguing theory and deserves to be investigated more thoroughly. However, inflammation is an unproven theory, and this must be kept in mind.

Auto-Immune System

Some people also speculate that lipedema may have an auto-immune component. This seems to tie in with the inflammation theory in that the inflammation may be an auto-immune response to insidious environmental cues around us, including food sensitivities, plastics, or estrogen-promoting foods and substances.

Although there is little proof of ANY of these theories at this time, an auto-immune connection is one theory I am inclined to take seriously, since anecdotally, many women with lipedema also report high rates of autoimmune diseases running in their families, such as thyroid issues or lupus.

Of course, the same argument made against the inflammation theory can be made here. Autoimmune is another trendy theory these days, so caution must be applied when considering it too.

Endocrine Issues

Lipedema often seems to be accompanied by endocrine issues, leading some to speculate that this may be the original trigger for the overgrowth of fat cells or inflammation cascades.

One lipedema article states, "90 per cent of...cases have accompanying diagnoses of hormonal disturbance (thyroid, pituitary, or ovarian)." And the new Dutch guidelines on lipedema encourage testing patients for various endocrine issues.

On the other hand, many lipedema resources do not pay much attention to endocrine problems as a possible cause. In my opinion, this is a vastly under-researched aspect of lipedema and I would like to see MUCH more research done on it in the future.

Honestly, lipedema reminds me a bit of acromegaly. It's not the same condition at all, but the process behind it might have some similarities. Acromegaly occurs when a benign pituitary tumor causes too much Growth Hormone to be produced, and as a result the body size increases immensely, though in different ways than lipedema (affecting bones and certain soft tissues rather than fat cells). People with acromegaly grow so much they can end up very tall, with large hands, feet, jaw, prominent forehead bulges, and enlarged organs.

What if there were something similar happening with lipedema? What if the pituitary overproduces (or underproduces!) an important hormone and this causes hypertrophy/hyperplasia of fat cells? Wouldn't that explain why some people have such overgrowth of fat, despite strict diet and exercise? And why so many people with lipedema seem to have other endocrine issues? And why people get lipedema in such differing severities?

It just seems to me that some sort of endocrine issue HAS to be involved somewhere in this condition, and pituitary seems logical since it is the "master gland" controlling the other glands.

However, as with the other theories, there is no proof of any connection between pituitary issues and lipedema, so this idea also has to be taken with a large grain of salt. 

Abnormalities of the Lymph and Vascular System

It's possible that lipedema may only develop in people with underlying abnormalities in the vascular or lymph system, as one resource notes:
Foeldi and Foeldi have proposed that microangiopathy in the area of the affected adipose tissue sets off the condition leading to increased permeability to proteins and increased capillary fragility. 
Dr. Karen Herbst, one of the most pre-eminent specialists in Rare Adipose Disorders, speculates that abnormalities in the way the body transports fluids is at the heart of the problem in lipedema. She notes:
...Fat in lipedema...[shows] copious amounts of watery fluid upon incision of lesions and of ‘free fluid fat’ in biopsy specimens. 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. 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.
In other words, the capillaries that supply fat cells in lipedema women are fragile and damage easily, resulting in frequent bruising. In addition, they may be more permeable and leak proteins into the spaces around fat cells. These proteins tend to attract lymph fluids and cause swelling. The lymph system itself may also be somewhat defective, and the two of these factors (vascular permeability and lymph fluid transport issues) can add together to make things worse.

Summary

The bottom line is that nobody really knows what causes lipedema, but it's probably a combination of several things. One recent hypothesis proposes:
Lipedema is an estrogen-regulated polygenetic disease, which manifests in parallel with feminine hormonal changes and leads to vasculo- and lymphangiopathy. Inflammation of the peripheral nerves and sympathetic innervation abnormalities of the subcutaneous adipose tissue also involving estrogen may be responsible for neuropathy. Adipocyte hyperproliferation is likely to be a secondary phenomenon maintaining a vicious cycle.
This is more or less saying "all of the above." In all likelihood there is a tendency to inherit lipedema, perhaps through an underlying problem with the lymph or vascular system. Or perhaps it's underlying endocrine or autoimmune issues, with a strong sensitivity to inflammation and estrogen exposure. Or perhaps it's all of these, or something else entirely.

Whatever the combination is, it tends to create overgrowth of fat cells in certain areas. It is not yet known whether this is due to hypertrophy (an increase in size of fat cells) or hyperplasia (an increase in number of fat cells) but it's probably both.

These fatty deposits have particularly fragile capillaries that tend to leak fluids and proteins. These stress the lymph system over time, leading to the secondary development of lymphedema in some cases.

One major question that lingers in my mind is why some women do not progress very severely in their lipedema, yet other women do. Some never go beyond Stage 1 or Stage 2, yet others rapidly develop Stage 4 lipo-lymphedema. Why do some experience such severe manifestations of this condition and others do not?

If we can figure this out, I believe we will find many of our answers about the base cause of lipedema and how to treat it.

Possible Associated Conditions

Many women with lipedema also have other conditions, commonly referred to as "co-morbidities." Here are a few that are commonly seen.

Hypothyroidism or Other Endocrine Issues

As noted above, there may be a connection between lipedema and endocrine issues.

Two of the leading experts in the field note that many women with lipedema have thyroid issues. If you think you have lipedema and have not been diagnosed with hypothyroidism, you might want to have your thyroid levels checked periodically.

Please note that it's important to read up on the controversies of thyroid testing before having these labs drawn. Have the Free T3 and Free T4 levels checked as well as the TSH levels.

Get your exact numbers, and know how your lab defines normal and abnormal results.  Compare your results to various cut-off recommendations; don't accept just "normal" or "borderline" from your care provider. Different doctors and labs use different cut-off recommendations; the same reading can get you an "abnormal" flag from one provider and a "normal" flag from another provider.

Although not all "borderline" numbers need treatment, many people with borderline thyroid numbers and symptoms find they benefit significantly from treatment. In my non-professional opinion, the presence of lipedema plus a TSH around 2.5 or more and symptoms of thyroid issues deserves consideration of a careful trial of meds, especially if there is a history of autoimmune issues in your family. You may need to seek out a naturopath, integrative medicine specialist, or other "alternative" practitioner in order to get this treatment, however, as many traditional doctors will not treat unless the TSH number is well above 5.5.

Some women with lipedema also find that they have adrenal issues, especially adrenal fatigue. This seems not uncommon in women with thyroid or other endocrine issues. Some lipedema doctors recommend getting adrenal testing to check adrenal functioning. However, whether adrenal fatigue is real is controversial in some circles.

Again, some speculate there is a connection between lipedema and pituitary issues. Since the pituitary is the "master gland" controlling all the other glands, perhaps that is the real source of the thyroid or adrenal issues, rather than something limited only to those specific glands. 

Polycystic Ovarian Syndrome (PCOS)

PCOS seems to be a common co-condition with lipedema, although many women with PCOS do not have lipedema. Since women with PCOS tend to have an excess of estrogen compared to progesterone, and since lipedema seems to be associated with estrogen-related life changes (puberty, pregnancy, perimenopause), it seems logical that there could be a crossover between PCOS and lipedema.

Typically, women with PCOS have "apple-shaped" bodies (lots of abdominal fat but average-sized legs and hips), which doesn't fit the lipedema body type at all. However, some women with PCOS have more of a pear-shaped body and might want to look at the symptoms of lipedema more closely.

Many women with lipedema have symptoms of PCOS and borderline thyroid levels. It's my own unscientific observation that these women often have fibromyalgia as well.

Not much research has been done on a possible connection between PCOS and lipedema, but this is an area ripe for exploration in the future. Best guess is that many women who have both PCOS and lipedema will benefit from treating their PCOS. It won't make the lipedema go away, but it might help lessen its impact and progression.

Insulin Resistance

Many women with lipedema also have strong insulin resistance, perhaps related to concurrent PCOS.

On the other hand, most doctors would blame the obesity common with lipedema as the reason for insulin resistance. But insulin resistance and obesity is a chicken-and-egg question ─ which causes which? Was there an underlying insulin resistance which predisposed the woman to gain a lot of weight and worsen the lipedema? Or did the person gain a lot of weight first and then develop insulin resistance and worsening lipedema?

Women's experiences suggest that the insulin resistance usually comes first and predisposes to everything else later, but it's hard to prove that. And of course, weight gain may make everything worse in a vicious circle.

Either way, if you have lipedema, you should check your insulin levels. If they are high, it would probably benefit you to try to normalize them. It might not affect the lipedema much, but it might help lessen further weight gain, and it probably would lessen the risk for diabetes, stroke, and heart disease later on.

Nutritional Deficiencies

Some resources note that many women with lipedema have very low levels of Vitamin D, iron, and Vitamin B12. No one is sure why this might be, but there are a couple of theories around.

Vitamin D is fat-soluble, so most doctors assume that extra fat sequesters Vitamin D in the body, making it less available in the blood. However, other doctors point out that there is actually no proof of this theory and it's often promoted as fact when it is really just a theory with no supporting evidence. Whatever the reason, many doctors now believe that high-BMI people need higher doses of Vitamin D supplementation than average-sized people.

Low iron levels (or low ferritin levels, which lead to iron-deficiency anemia) may be associated with hypothyroidism. This may particularly be a problem for women in perimenopause, when some experience very heavy periods before their bodies shut down menstrual cycles altogether.

People with hypothyroidism can tend to have lower stomach acid, which may be one reason why they don't absorb dietary iron or Vitamin B12 very well. Low stomach acid can also predispose to intestinal yeast overgrowth, which may also inhibit absorption of nutrients.

If women with lipedema have higher rates of hypothyroidism, and if hypothyroidism tends to lower stomach acid and make it harder to absorb certain nutrients, then it makes sense that women with lipedema might have more nutritional deficiencies. On top of that, years of restrictive dieting and bariatric surgeries like gastric bypass put some women even more at risk.

Some women with lipedema report improvement with an anti-inflammatory diet, as noted above. This diet often involves leaving out dairy and gluten, among other things. Interestingly enough, people with low stomach acid often report having trouble with dairy and gluten. So perhaps there is a connection.

None of these connections have been proven conclusively, but they certainly are interesting. Perhaps taking out dairy and gluten can help, or perhaps increasing stomach acid levels (via daily apple cider vinegar or Betaine consumption) makes it possible to process those foods more easily and improve levels of ferritin, Vitamin D, and Vitamin B12.

Either way, women with lipedema (especially those with hypothyroidism) should watch for nutritional deficiencies and treat if needed.

Eating Disorders

A number of lipedema resources note the possibility of eating-disorders. One clinic in Germany found that 74% of their patients with lipedema had eating-disordered behaviors.

This is probably an overestimate. Most women with lipedema do not have true eating disorders, but some have flirted with them. Others clearly do have eating disorders, and that must be addressed in order to improve health.

Eating disorders can occur with lipedema because people have spent years being shamed and lectured into trying to lose weight. Yet lipedemic fat is extremely resistant. Often their approaches get more and more extreme as each diet fails. Is it any wonder, then, that some develop eating-disordered behaviors?

Some do truly develop into the binge-eating, housebound stereotype that TV programs love to feature. Some overeat for emotional reasons. Some learn to binge and purge to try and control their weight. Others become fat anorexics, following rigid diet restrictions and extreme over-exercising in a futile effort to control their size. The study from Germany found that 16% of their lipedema patients were actually anorexic in their behaviors, more than were bulimic or binge-eaters.

Although most people with lipedema do not have eating disorders, it's important to recognize that some do, and that this is a recipe for serious difficulties. Care providers shouldn't automatically assume that a woman with lipedema has an eating disorder, but if one exists, it is vitally important that it get treated too.

Immune System

Some resources describe a generally depressed immune system in women with lipedema. This may make you prone to easily getting illnesses and having a hard time healing from them.

Whether this is a function of the lipedema itself or the possible accompanying issues (i.e. hypothyroidism, auto-immune issues, chronic nutritional deficiencies) is not clear, but it is probably a good idea to be very proactive about avoiding illness.

Fibromyalgia

Anecdotally, there seem to be a lot of women with lipedema who also experience fibromyalgia. Since some believe that fibromyalgia is an autoimmune issue, the possibility that lipedema also has autoimmune components might explain a connection.

Fibromyalgia is not the same as lipedema, of course, since fibromyalgia patients have tremendous sensitivity to touch/pressure all over their bodies, and pain is mostly limited to the legs in lipedema.

However, perhaps difficulty with efficient lymph drainage explains similar symptoms. One Manual Lymph Drainage (MLD) therapist notes that many women with fibromyalgia find some relief from their pain levels with MLD therapy. Research does seem to support that MLD can be an effective treatment both for lipedema and for fibromyalgia.

Varicose Veins

Many women with lipedema develop telangiectasias, or spider veins.

In spider veins, small red spidery capillaries are visible just beneath the surface of the skin, spreading out like a spider web. This is not dangerous, but some people are bothered by its looks.

If you look very closely you can see spider veins in the picture to the left. Look just above and to the inside of the knees at the faint red lines. (You can see other pictures of spider veins here.)

Varicose veins and Venous Insufficiency can also be a problem for some people with lipedema. Good blood flow can be impeded by the extra fat deposits and the fragility and permeability issues. This may lead to blood pooling in some areas instead of circulating efficiently.

Hypermobility

Some resources have noted that many women with lipedema tend to be hypermobile, that is, their ligaments tend to be looser than average and therefore the women more flexible, at least in childhood.

This seems to be borne out in anecdotal evidence, though the hypermobility can vary from mild to significant. This can then lead to joint issues later in life.

Fallen Arches

Some lipedema resources report that "flat feet" (a.k.a. fallen arches) is more common in women with lipedema.

Is this just a function of a typically higher weight, or is this related to the lax ligaments and hypermobility that some have noted with lipedema? No one is sure, but it does seem logical that if lipedema women have hypermobility and joint issues, they might also tend to have fallen arches.

Joint Issues

Knee osteoarthritis is another issue common to women with lipedema. One 2009 study found that more than half of their patients diagnosed with lipedema also experienced significant knee pain.

Knee osteoarthritis seems to be common as people with lipedema age, and at a fairly early onset compared with the general population. One resource notes "early onset of degenerative joint changes and arthritis" among the symptoms commonly associated with lipedema. 

Of course, some providers question whether knee arthritis is more common simply because women with lipedema tend to be heavier. However, the authors of the 2009 study noted (my emphasis):
Knee pain is a feature of lipedema, but can also be found in simple obesity. Nevertheless, it appears to be a phenotypic feature and is often a complaint in pure lipedema where the BMI is normal.
Doctors think that this increased rate of joint issues is probably because of three different factors. First, because the fat deposits tend to have pockets of fluid around them and because lymph flow is inhibited, this excess fluid will tend to flow to areas of least resistance ─ the joints. This can irritate the joints and cause pain.

Second, as lipedema progresses, fat pads around the knees build up and can alter the mechanics of the knees. The gait may be thrown off and stress the knees.

Third, hypermobility issues may exacerbate joint issues. As women with lipedema age, this hypermobility combines with gait changes and fluid irritation in the knee to result in significant knee pain and rapid development of arthritis, especially in the face of an injury or trauma to the area.

Muscle Weakness

In addition to joint issues, there may be an increased level of muscle weakness, as well as weak connective tissue and joint laxity in those with lipedema.

This is a significant finding because one way to treat knee issues is to strengthen the muscles around the knees. However, if these muscles weaken as lipedema worsens (and do not respond to physical therapy and strengthening exercises), that may be another reason why mobility can be so affected.

Mobility Issues

Between the joint issues, hypermobility, muscle weakness, pain, and lipo-lymphedema, mobility issues often become very significant in people with lipedema as they age. 

Unfortunately, many people with lipedema are denied joint replacement surgery because they have elevated BMIs and doctors refuse to operate on them until they lose weight. But significant weight loss to "acceptable" BMIs is nearly impossible for people with lipedema, so they are never able to get their knees or hips replaced, and their increasing pain levels continues to decrease their mobility. Some end up in wheelchairs or scooters, and this lack of mobility creates even more lymphedema and fat deposits in a vicious feedback loop.

That's why it's so important to stay as active as your circumstances permit. It slows the progression of lipedema and lymphedema, as well as being good for your heart and other systems.

Mobility issues is another reason why it's so important that lipedema becomes more recognized as a medical condition deserving treatment.

At this point, the medical stigma of obesity among many doctors means that this condition often goes undiagnosed, with unrealistic weight loss prescribed when symptoms arise. Sufferers are often denied access to the treatments that do work until they become progressively more disabled and immobile, and then are made fun of in society when they use mobility aids like scooters.

It's only by raising awareness of lipedema and its treatments that more women will get the treatments that will help most and the stigma surrounding this condition will diminish.

Skin Infections

Cellulitis infection with lipo-lymphedema;
notice the foot swelling from secondary lymphedema
Another common problem in the latter stages of lipedema is skin infections.

This is usually not a serious issue until Stage 3 lipedema, or if secondary lymphedema starts to develop. However, sometimes things like bug bites or a seemingly minor cuts or burn can result in serious swelling, redness, and infection, even in the earlier stages of lipedema.

This means that all people with lipedema need to be very vigilant in caring for their legs and watch for minor problems before they develop into major ones.

Erysipelas and cellulitis (bacterial infections of the skin and underlying tissues) are a problem in some people as lipedema becomes severe, and it can be hard to treat because decreased lymph flow inhibits healing.

Infections secondary to lipolymphedema are hard to heal. Many care providers prescribe inadequate dosages of antibiotics in heavier people with skin infections, not realizing that weight-based dosing is needed with some types of antibiotics, or unnecessarily worrying about overloading the person's kidney function.

If not stopped with antibiotics, a skin infection can spread to deeper layers and become necrotizing fasciitis. If it gets into the blood system, a person can get sepsis and even die. Therefore, preventing, watching for and early treatment of any skin infections is an important part of care in people with lipedema, especially in the later stages.


When high BMI people go to the E.R. with cellulitis or other skin infections, they are often under-dosed with antibiotics, even in hospitals that specialize in treating heavier people. 

This is a MAJOR problem. Research shows that high BMI people, especially people with a BMI over 50, are often given an antibiotic dose that does not reach full inhibitory response. This may result in an initial improvement of symptoms, but a drastic relapse later on as the bacteria mutate and adapt to the inadequate dosage of antibiotics.

If you have lipedema and develop a serious skin infection, you may need to be very proactive in advocating for yourself. Ask for a wound infection specialist, ask if the type of antibiotic they are prescribing should have weight-based dosing (some do and some don't).

If the wound does not respond well, ask about more proactive wound care, including IV antibiotics, more frequent dosing, and larger doses, as research shows that proactive care like this can improve outcomes better than traditional care.

Summary

As we have discussed in this series, lipedema is a condition that is often mistaken for simple obesity. Diets and weight loss do not substantially reduce its presence, and lipedema often gets worse as people age, sometimes leading to significant mobility issues and life-threatening infections.

Many fat people with lipedema go undiagnosed for years because physicians either have not been taught about this condition (despite it having been discovered in 1940), or have so much weight stigma that they do not recognize the condition as anything other than obesity. Some even deny it is a real clinical entity, despite the fact that is recognized as one by many groups, including the NHS in the U.K.

Fat disorders are only now beginning to get the research attention needed to expand our knowledge but recognition of this disorder still has a long way to go in many parts of the world. Thus many people with lipedema will likely continue to go undiagnosed for some time to come.

Many women discover their condition via massage therapists or physical therapists rather than doctors because doctors are so under-informed about lipedema. Even today, women are being told that there is "no such thing" as lipedema, that they just need to lose weight, to exercise more, or that they are just making excuses for being fat and lazy.

Others are accused of lying about their food intake or exercise habits. Friends, family, and acquaintances may harass them about their weight and think that "lipedema" is just making excuses for bad habits.

Some lipedema sufferers are diagnosed with lymphedema without any mention of lipedema. Some are only diagnosed with lipedema once significant lipo-lymphedema develops and their condition becomes very severe. The fact that many cases of lipedema are only diagnosed after severe lipo-lymphedema develops is a sad statement about the ignorance of lipedema among most doctors.

Even once a woman has a diagnosis of lipedema, care providers can be very apathetic about managing it, or overly pessimistic about its progression so they don't bother providing much care. Women with lipedema often have to fight for adequate care or insurance coverage of needed treatments.

No one really knows what causes lipedema but it likely is hereditary in many cases. It may involve some sort of endocrine, inflammation, and/or auto-immune disorder, and there may also be underlying abnormalities in the lymph or vascular system. As one resource puts it:
Often mistaken for obesity or unusual weight gain, lipedema is a fat-storage disease that almost exclusively affects women...This chronic disorder affects the legs and arms, causing an abnormal accumulation of fat cells that produce a noticeably disproportionate appearance. There's a genetic component in approximately half of all lipedema cases, and it's not just being overweight or out of shape -- it's truly a disproportion in the affected areas of the body. It's a condition that is not only an overgrowth of fat tissue, but it also has a vascular or lymphatic component with swelling.
In other words, it's really NOT about diet and exercise, but about something we don't completely understand yet. Hopefully, finding and treating the hormone or endocrine issues and/or reducing inflammation as much as possible will help. It likely won't make the lipedema go away, but it might help minimize it or slow the progression.

More care providers need to familiarize themselves with lipedema so women get diagnosed and treated earlier to keep the condition from progressing. Furthermore, if complications such as knee pain, cellulitis, or lymphedema occur, care providers need to know how to treat them appropriately, instead of just telling the patient to lose weight, as so often happens now.

As one research summary notes:
Lipedema is a genetically mediated disorder of fat deposition. It results in a characteristic pattern of lower-extremity enlargement that is resistant to diet and thus very demoralizing. It can eventually lead to lymphedema but should not be mistaken for lymphedema in its early stages...This frustrating genetic disorder of fatty deposition is not particularly rare, but is rarely diagnosed because clinicians fail to recognize it.
Hopefully, once more doctors get past their stigma and prejudices about obesity, more research will be done into how to treat (and hopefully prevent) lipedema.

*Next post: Treatment options



References and Resources

If you think you have lipedema and need help in getting it diagnosed, see this resource. Also print out some of the papers from the links below and take those to your appointment with you. Remind your doctor that this condition is now recognized by the National Health Service in the U.K. and that there is information online about how to diagnose and treat it.

Resources

*Trigger Warning: Many of these sites are not size-friendly. However, because they also contain valuable information about lipedema and its treatment, they are included here.
Blogs, Websites, and Facebook Pages from Those Dealing with Lipedema
Possible Causes of Lipedema

Child AH, Gordon KD, Sharpe P, Brice G, Ostergaard P, Jeffery S, Mortimer PS. 2010. Lipedema: An inherited condition. Am J Med Genet Part A 152A:970–976. PMID: 20358611 Free full text available here.
Lipedema is a condition characterized by swelling and enlargement of the lower limbs due to abnormal deposition of subcutaneous fat. Lipedema is an under-recognized condition, often misdiagnosed as lymphedema or dismissed as simple obesity. We present a series of pedigrees and propose that lipedema is a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation. Lipedema appears to be a condition almost exclusively affecting females, presumably estrogen-requiring as it usually manifests at puberty. Lipedema is an entity distinct from obesity, but may be wrongly diagnosed as primary obesity, due to clinical overlap. The phenotype suggests a condition distinct from obesity and associated with pain, tenderness, and easy bruising in affected areas.
Med Hypotheses. 2014 Aug 23. pii: S0306-9877(14)00295-3. doi: 10.1016/j.mehy.2014.08.011. [Epub ahead of print] Pathophysiological dilemmas of lipedema. Szél E1, Kemény L2, Groma G2, Szolnoky G2. PMID: 25200646
Lipedema is a common, but often underdiagnosed masquerading disease of obesity, which almost exclusively affects females. There are many debates regarding the diagnosis as well as the treatment strategies of the disease. The clinical diagnosis is relatively simple, however, knowledge regarding the pathomechanism is less than limited and curative therapy does not exist at all demanding an urgent need for extensive research. According to our hypothesis, lipedema is an estrogen-regulated polygenetic disease, which manifests in parallel with feminine hormonal changes and leads to vasculo- and lymphangiopathy. Inflammation of the peripheral nerves and sympathetic innervation abnormalities of the subcutaneous adipose tissue also involving estrogen may be responsible for neuropathy. Adipocyte hyperproliferation is likely to be a secondary phenomenon maintaining a vicious cycle. Herein, the relevant articles are reviewed from 1913 until now and discussed in context of the most likely mechanisms leading to the disease, which could serve as a starting point for further research.
Lipedema and Undertreatment of Skin Infections

Am J Emerg Med. 2012 Sep;30(7):1212-4. doi: 10.1016/j.ajem.2011.05.027. Epub 2011 Dec 12. Underdosing of common antibiotics for obese patients in the ED. Roe JL1, Fuentes JM, Mullins ME. PMID: 22169576
BACKGROUND: ...Obesity alters the pharmacokinetic profiles of various drugs. Although there are guidelines for dose adjustments for many of the antibiotics commonly used in the emergency department (ED), they are seldom used. METHODS: This is an institutional review board-approved retrospective study at an American Society of Metabolic and Bariatric Surgery Center of Excellence and a level I trauma center with annual ED volumes of more than 80,000 visits. Data were retrospectively collected from ED pharmacy records during a 3-month period in 2008. Any first dose of cefepime, cefazolin, or ciprofloxacin administered in our ED to a patient recorded as both more than 100 kg and with a body mass index greater than 40 kg/m(2) was compared with our hospital guidelines and found to either adhere or not adhere to those guidelines. RESULTS: ...Adherence rates for first dose of cefepime, cefazolin, and ciprofloxacin administered, respectively, were 8.0%, 3.0%, and 1.2%. CONCLUSION: Emergency physicians frequently underdose cefepime, cefazolin, and ciprofloxacin in obese patients. Underdosing antimicrobials presents risk of treatment failure and may promote antimicrobial resistance. Education is necessary to improve early antibiotic administration to obese patients.
J Prim Care Community Health. 2015 Apr 29. pii: 2150131915583659. [Epub ahead of print] Cellulitis in Obesity: Adverse Outcomes Affected by Increases in Body Mass Index. Theofiles M1, Maxson J2, Herges L2, Marcelin A3, Angstman KB2. PMID: 25925834
...This study was a retrospective chart review of 637 adult primary care patients designed to evaluate rates of treatment failure of outpatient cellulitis among patients of varying BMI. Treatment failure was defined as (a) hospital admission for intravenous antibiotics, (b) prolonged antibiotic course, or (c) requiring a different antibiotic after initial course. RESULTS: Adverse outcomes were not statistically significant between normal BMI and those with BMI ≥40 kg/m2. A subset of patients with a BMI ≥50 kg/m2 was noted to have approximately twice the rate of adverse outcomes as the normal BMI group...CONCLUSIONS: Patients with cellulitis weighing >120 kg or with a BMI ≥50 kg/m2 were at greatest risk for treatment failure in the outpatient setting, even when controlling for comorbid diabetes and tobacco use. As morbid obesity continues to become more prevalent, it becomes imperative that primary care physicians have better antibiotic dosing guidelines to account for the physiologic effects of obesity to minimize the risk of increased morbidity, health care costs, and antibiotic resistance.

Thursday, June 18, 2015

Lipedema, Part 3: Types of Fat Distribution and Diagnosis

We have been discussing Lipedema, a "Rare Adipose Disease" (RAD) that most people assume is just fat.

In this condition, an abnormal accumulation of fat occurs in the legs and lower trunk, sometimes also including the arms or upper body as well.

Lipedema (also spelled Lipoedema, as to the left) is rarely recognized by doctors. Often it is thought to be "simple obesity," or it is confused with lymphedema, the accumulation of lymph fluid in the interstitial areas.

In Part One of the series, we discussed the symptoms and typical presentation of lipedema, and we pointed out the differences between lipedema and lymphedema.

In Part Two, we discussed the stages and progression of the lipedema.

Today, in Part Three, let's talk about fat distribution types and the process of diagnosing lipedema.

In future posts, we will also discuss possible causes of lipedema, associated conditions, treatment options, and how to live proactively with it.

Stages vs. Types of Lipedema

Stage 1, Stage 2, and Stage 3 of Lipedema
Before we go any further, let's review again the distinction between Stages and Types of lipedema. This is important because people new to the topic can get confused between them.

Stages of lipedema refers to how far the condition has progressed (worsened). Types of lipedema refers to the specific pattern of fat distribution in lipedema. 

In other words, when we talk about Stages of lipedema, we are asking whether a woman has developed various characteristics of advancing lipedema:
  • Stage One - Disproportionate pear shape, with somewhat increased fat. Normal skin surface which feels smooth and soft. Leg still has shape but may be considered somewhat larger or thicker than average by others. There can be some swelling (edema) during the day but it usually resolves overnight or with rest and elevation
  • Stage Two - Skin texture begins to change from smooth to uneven with indentations ("orange peel" or "mattress" skin). Fatty deposits start to grow around knees and thighs, and some also develop large arms or chest. Legs begin to thicken more and lose their shape, and "cankles" start to develop. Skin is rubbery or spongy and begins to feel nodular in places, like little beans under the surface. Edema can occur but doesn't resolve as easily as it has in the past
  • Stage Three - Skin texture in hips and thighs has more of an "orange peel" look now, and fat nodules are easier to detect. Large masses of tissue can form folds and ridges, especially around the knees and on the thighs. Cankles get worse and begin to "overshoulder" the ankle. Swelling becomes more consistent and does not resolve with rest and elevation. If lymphedema starts to develop, hardening of connective tissues can start to occur, and skin starts feeling tougher
  • Stage Four - Lipo-lymphedema develops (lipedema with secondary lymphedema). Larger masses of skin and fat overhang, making many complex folds and ridges with consistent swelling. Mobility can be affected. Skin can become harder and discolored. In severe cases, lymph fluid can begin to leak from lymphatic vessels
On the other hand, Types of lipedema refers to where the majority of fat is located on the woman ─ only the hips to the ankle, the whole leg, etc.

So Stage is about progression of the condition, whereas Type is about fat distribution pattern.

Although we will talk first about Type today, we will be evaluating both Type and Stage in sample pictures at the end of the post.

Types of Lipedema

Although online resources mainly discuss the Type of lipedema that involves the whole leg, there are other variations of lipedema that do not involve the whole leg. This may lead to some cases of lipedema being missed. That's why it's important to understand that there are different patterns of fat distribution, and that all still qualify as lipedema.

According to one site which specializes in treating lipedema, there are 5 Types of lipedema, based on the distribution of the fat:
  • Type I - Pelvis, buttocks, and hips ("riding breeches" type, but does not extend to knees)
  • Type II - Buttocks to knees, with some folds of fat around the knees (can also be called "riding breeches" type, but goes further)
  • Type III - Buttocks to ankles (like "harem" pants or "pantaloons")
  • Type IV - Arms are affected, usually along with legs
  • Type V - Lower legs only (knees to ankles)
Some resources list a slightly different set of types. One resource breaks Type I into 1a and 1b, and Type V is sometimes listed as lipo-lymphedema (in my opinion, this is a Stage, not a Type).

You can have more than one type at once if you have arm involvement. For example, nearly every woman with Type IV (arms) will also have some leg type as well. One research article estimates that only about 3% of women with lipedema will have it only in the arms.

One site notes that each type also has a subcategory, A (without pain) and B (with pain). However, most resources don't seem to make this distinction. Indeed, pain on touching the legs (especially the shins) is one of the classic symptoms usually listed, although the implication is that there is less pain early in the progression of lipedema, and far more pain as it advances.

Obviously, if you dig around enough, you can find variations, but the majority of resources list the 5 Types above, so this is the model I am using. Below is a brief video with visual descriptions of the different Types.



Clothing Metaphors

Sometimes lipedema Types are described with clothing metaphors, and this is one way to remember the different types of fat distribution in Types I-III.


For example, "jodhpurs" are breeches that were popular in history for riding or for military uniforms. They were cut extra wide in the seat and thigh to give extra room for the moves needed in horse-riding. "Riding Breech" lipedema, or Types I/II, has most of the extra fat in a pattern much like jodhpur breeches.


On the other hand, "pantaloons" or "harem" pants were cut full all the way down to the ankle, then gathered there. The example above are "bloomers," named for Amelia Jenks Bloomer, who promoted them as a more sensible clothing for women in the late 1800s. The most common form of lipedema, Type III, involves fat distribution that goes down to the ankles but stops abruptly there, much like pantaloons.

Although not all lipedema resources reference clothing when discussing Types of lipedema, it can be an easy way to remember the differences. To help clarify the Types for those new to lipedema, we will include clothing comparisons in our descriptions below.

Pictures of Types

Below you will find a more detailed description of each lipedema Type, along with pictures illustrating that type. Some might find the pictures disturbing, but remember that not all women with lipedema progress to the same level of severity and that treatment exists that can help many cases.

Regardless of the level of progression, no criticism of any woman's body should be inferred from these pictures or descriptions. ALL bodies are beautiful and deserve kindness and love.

Also note that some pictures will be used more than once in this series, as there are only so many pictures available of lipedema. Some come from various lipedema resources, some come from Wikimedia, some come from research papers freely available online, some from lymphedema or liposuction treatment sites, and some come from women's websites or blogs.

Many thanks to the brave women who have been willing to share their pictures online to help educate others about this condition! I know this was critical in me realizing that I have lipedema, and I share these here with the hope that it will be helpful to others, both women with lipedema and the care providers who are learning how to identify and care for women with lipedema.

Type I - Pelvis, Hips and Buttocks


In Type I, the pelvis, hips and buttocks are affected but not the legs as much. The knees are not involved. This is one type of "riding breech" lipedema.


The woman above clearly has "saddlebags." She has disproportionate fat deposits around her hips and behind but it doesn't really go much into her legs.

She is fairly early in progression. She has substantial deposits of fat, and her skin is mostly smooth but starting to change. She is probably going into Stage 2.

At least one resource further separates Type I into Type Ia and Ib. It's an interesting distinction so I am including it here, but readers should know that most resources do not make this distinction. Type Ia involves the area of the pelvis, hips and buttocks only ("saddlebags"), and Type Ib involves the area of the pelvis, buttocks, hips, and upper thighs ("riding breeches")


Type Ia has most of the fat concentrated in the buttocks and hips. It really is a saddlebag type of look. Typically they do not have much leg involvement, or it doesn't go down very far.

The woman above is probably a Type Ia, in that her saddlebags involve mostly just her hips/behind. Notice how her hips "overshoulder" her legs.

She is probably going into Stage 2 in that her skin is still mostly smooth but changes are beginning to happen.


In Type Ib, the saddlebag look is still there, but the fat goes down into the upper thighs as well.  It does not extend all the way to the knees, however.

This woman is probably an example of Type Ib. Her fat is not just her hips, but extends a bit into her legs. However, it does not go down to her knees. Notice how her hips/upper thighs overshoulder the rest of her thighs. That is classic Type I.

She clearly is in Stage 3, because she has folds and ridges happening, as well as some orange peel texture to the skin. She may have some degree of lipo-lymphedema as well but it doesn't seem so strong that I would put her in Stage 4.

Notice the disproportion between her average-sized upper body, her larger abdomen/hips/thighs, and then average-sized legs. This can make finding nice clothes very difficult. Still, she is not letting that hold her down, and she is rocking her swimsuit!

Type II - Buttocks to Knees


In most women with the "riding breeches" type of lipedema, the fat actually extends from the hips all the way down to the knees, but stops just at or just beyond the knees, like many jodhpur riding breeches.


This woman's fat distribution clearly stops at her knees in the classic jodhpur or riding breeches silhouette. Although her hips are by far her largest part and they overshoulder the legs, she does have quite a bit of fat on her thighs down to her knees, so I would call it Type II rather than Type I.

She is likely entering Stage 4. She has severe enlargement and the folds so typical of Stage 3, but she doesn't have the massive number of folds or lobes so typical of Stage 4. We can't see her from the front, so it's possible that we are missing more folds there, but she is definitely seems like she's going into Stage 4.


Many women with Type I or II lipedema develop extremely disproportionate hips as lipedema progresses. The woman above has a much smaller upper body, quite disproportionate hips and legs to the knee, and much smaller lower legs. There seem to be a number of women with "riding breech" lipedema that have this extreme presentation.


Here is another Type II woman with extreme size difference between her upper body, her hips to her knees, and then much smaller legs. I would guess that she has Stage 4 lipo-lymphedema, and that is why things are so disproportionate.

Sadly, women with riding breeches lipedema are often very cruelly hassled in public and online, even though the extreme disproportion of fat distribution clearly shows that there is more going on than just "simple obesity."


Here is a link to a story about Mikel Ruffinelli, a woman who probably has extreme Type II lipedema also. She mentions that most of the weight around her hips was put on with each of her pregnancies, a classic symptom of lipedema. She is an excellent example of a woman who has embraced her look, is very empowered, and has a loving family and a good life. Lipedema presents many challenges and these must not be discounted, but it does not have to keep you from having a good life!

Type III - Buttocks to Ankles


Type III is the "pantaloon" type of figure. The fat starts at the abdomen/hips/buttocks but doesn't stop at the knees. Instead it goes all the way down to the ankles. In later stages, it can droop over the ankles like the elasticized or cuffed bottom of a pair of pantaloons or harem pants.


Type III seems to be the most common form of lipedema. The typical bracelet of fat becomes more marked as the lipedema progresses in severity. Notice that this woman's cankles are starting to droop down onto the skin below.

The woman above is probably transitioning into Stage 3. Her condition is significant but not extreme.


Here is an extreme example of Type III. You can see why some people compare this to pantaloons. The extreme size difference between leg and foot is its hallmark.

This woman appears to be in Stage 4, with severe lipolymphedema. Her swelling has caused her ankles to severely overshoulder the foot. She desperately needs some Manual Lymph Drainage work.

Type IV - Arm Involvement


Some women have extremely heavy upper arms, much larger than the rest of their arm. This is Type IV lipedema, probably in Stage 3. Notice how disproportionate this woman's upper arm is from her lower arm. It's very common for it to hang down like that...not the normal "bat wings" that many women get as they age, but much more extreme, and often at younger ages too.

One research paper suggests that of women with lipedema in the legs, about 30% also have lipedema in the arms as well. From my observation, the most common combo seems to be Type III and IV (full leg plus arm involvement).

It is possible to have lipedema of the arms only, although that's rare. This same paper notes that about 3% of women with lipedema have it only in the arms.


If Type IV lipedema advances into lipo-lymphedema (Stage 4), then lower arms may also be affected. Instead of having the overshouldering bracelet of fat around the ankle, they have the bracelet of fat around the wrist, which you can just see in the picture above. The hand can sometimes be affected as well, but usually much less so.

Type V - Lower Legs Only


Type V seems to be a pretty rare type of lipedema; it's very hard to find images of this one online. In this type, the lipedema is only in the bottom half of the legs (calves). In the picture above, the calves are quite a bit heavier than the hips and thighs.

This woman is probably only in early Stage 2; while there is a bit of mattress skin look, mostly her legs are fairly smooth and she doesn't have major cankles yet.

Summary

Although it's interesting to trace the different patterns of fat deposition in lipedema, most experts seem to feel its distribution is probably not as important as its stage of progression.

On the other hand, while it is important to realize that a bracelet of fat is highly characteristic of lipedema, there are forms that do not go to the ankle.

You can see an illustration of the various types of lipedema and how they progress here. [However, do note that this source has slightly different delineations of "Type" than most sources.]

Variations on Stage and Type

The moral of the story is that not all lipedema cases are alike. While certain patterns are typical, there are variations. Here are a few examples of different types of lipedema at differing stages of severity to show the wide range of possibilities.

Keep in mind that these only represent my best guess for evaluation, but I am not a medical professional nor an expert in lipedema. I could very well be wrong and lipedema experts might have a very different take on some of these. Insert caveats.

Clear Examples



This woman has Type III lipedema, which is probably the most common type. Notice the typical pantaloon look. Her whole leg is involved and she has the "stepped" look at the ankles.

She is in Stage 3 ─ notice the size and clear orange peel texture of her legs. She may be experiencing some secondary lymphedema, especially around her ankles, but she lacks the severe folds, lobes, and swelling that most Stage 4 women get.



This woman also has Type III lipedema. Notice the full leg involvement, the fat pads on the inside of the knees, the column-like look of the leg and its relative lack of shape.

She seems to be in Stage 4, with significant overshouldering of the ankles because of secondary lymphedema. She does not have the severe folds and lobes that occur in severe Stage 4, but she has a lot of swelling. Her ankles are clearly much larger than her feet and she has some lymphedema in the feet starting to occur, too.


Here is a woman who is also clearly a Type III. Notice the pantaloon shape. She is not disproportionate above the knee and smaller below; the whole leg is strongly affected down to her ankles.

She is in severe Stage 4 (lipo-lymphedema). She has the "Michelin Man" folds that can develop with severe secondary lymphedema. Notice the tremendous overshouldering of the ankles yet relatively unaffected feet. Also notice that her skin texture is beginning to change and get the rougher appearance that can come with severe lymphedema. Hopefully she got some good Manual Lymph Drainage treatment and compression garments after this picture was taken.


This woman clearly has the riding breeches form of lipedema. Notice the disproportion between her upper body and lower body, and between her hips and her lower legs. She probably has Type II lipedema.

Her Stage is harder to evaluate because her clothes cover her skin. Given the disproportion, however, she is likely Stage 3, possibly going into Stage 4.


Here is another picture of a Type II lipedema case who has developed lipo-lymphedema. Again, notice that the lipedema is much more severe in the thighs to the knees, with a little bit just below the knees. Notice that she doesn't have cankles at all.

She is in Stage 4 with significant secondary lymphedema. Notice all the folds and lobes that have formed. Notice also how the fat pads around her knees are severely swollen and droop down, much like the severe cankles that Type III cases get. (The lines drawn on her legs are a surgeon's markings prior to lymph-sparing liposuction.)


This is a classic case of Type IV lipedema. Notice the upper arm size compared to the lower arm. We can't know for sure what other type she may have without seeing her legs, but based on what we can see of her, I'd guess she also has Type III legs.

It is hard to judge stages from just an arm picture because arms typically don't develop the same orange peel texture as the legs. However, given her younger age, her young child, and relative tightness in the arm skin, I'd guess that she is probably in Stage 2.


This woman obviously has Type IV. Notice how disproportionate her upper arm is to her lower arm. We can't tell what other type of lipedema she has, if any.

It is hard to judge stages but given the amount of fat in the arm and its relative droop, I'd guess that she is probably Stage 3.

Less Clear Examples

In some women, it's hard to tell what Type they are. Let's take a look at a few pictures that are less obvious than the previous ones, or where the fat distribution seems to be a bit of a hybrid.


The woman above is a bit hard to define because we are only seeing her from the side. However, it looks like she has most of her lipedema fat between her abdomen and knees but not a lot of lower leg involvement. Note that her thighs are disproportionately heavy compared to her calves. Although she has "cankles" to some extent, they are not that significant compared to her thighs. I would guess that she is a Type II.

She has substantial fat deposits, disproportion, and the orange peel texture to her thighs, so I'd guess she is early in Stage 3. She does have a little bit of edema but it's not extensive. 


This case is also not easy to determine. Some might call it a Type II, since the majority of the fat distribution is in the abdomen, hips, and thighs. However, her fat deposits clearly extend down into her lower legs a bit past the knees, so some might call it Type III. Yet she has only a very minimal bracelet of fat around her ankles, nothing like the level of fat above. Therefore, I personally would probably lean towards calling it Type II based on looking at where the majority of fat is, but you could make a case for Type III.

Her lipedema is definitely in Stage 3, with significant mattress texture to her skin and folds and lobes of fat forming. However, she does not appear to have significant secondary lymphedema.


Here's another case that looks like Type II but extends a bit below the knees. Yet although she has some swelling in her ankles and feet, they clearly are much less affected than the area from the hips to just below the knees. So even though this woman has some fat below the knee, I would classify this as riding breeches, or Type II.

Obviously, this woman is in Stage 4, with a severe case of lipo-lymphedema. There are many folds and bulges and lobes of fat. Some edema is present in the ankles and foot as well, but most of it is limited to the riding breeches area. It is my observation that many of the worst cases of lipo-lymphedema I have seen in pictures are from the women with Type II lipedema, but that could be just coincidence. However, if it's true, women with the riding breeches presentations may want to be extra vigilant about treatment options to try to avoid Stage 4.


This woman looks like she has Type III lipedema but it is early in its development. She has the classic pear shape (note how much smaller her waist is than her hips) but not the saddlebag or riding breeches look. It's hard to tell because of the early stage, but it looks like her whole leg will be involved.

She is probably Stage 1, just going into Stage 2. Her legs still retain their shape and her skin is only beginning to show signs of orange peel texture. If you look closely, her ankles seem a little thickened but not that much yet. She doesn't really have cankles yet but there is the suggestion of them beginning.


The woman above has lipedema on her whole leg (Type III) but her lower leg seems to be a bit more affected (Type V). Still, I'd call her a Type III, because she does seem to have whole leg involvement, even if the lower leg is more involved.

She is probably Stage 3 because she has the orange-peel appearance and the fat pads by the knees with a bit of a cankle.


This woman's complete legs are involved, so she is a Type III. Some care providers might object to diagnosing lipedema at all because she doesn't really have a distinct pear shape; she is fairly large on the top too. However, her waist is smaller than her hips, she has bilateral and mostly symmetrical legs, and the classic cankle swelling. Her arms are clearly involved too, so she also has Type IV.

She is in Stage 4 and has developed secondary lymphedema. The extra folds and swelling at the knees and ankles suggest that Manual Lymph Drainage and compression garments would be helpful.


This one is more arguable. The heaviest amount of weight is to just below the knees, so Type II springs to mind first. However, her lower leg is somewhat affected and she does have some cankles, so some might argue for Type III. Still, her profile tapers down from wide to narrower at the ankles, and most of the folds and lobes are above or just below the knee, whereas a Type III with lipo-lymphedema usually has a severe pantaloon look all the way to the ankle. Personally, I'd call this Type II, but some might argue with me.

Clearly, she is in Stage 4 with lipo-lymphedema. Multiple folds and lobes are present, but although significant, her case is not as severe as some of the ones we have seen earlier.

Diagnosis

There is no particular blood test or definitive lab test for lipedema. Rather, it becomes a clinical diagnosis, based on observation of clinical symptoms and a process of elimination of other possible conditions, before arriving at an official diagnosis of lipedema. Lipedema is usually diagnosed if a patient meets certain clinical criteria, although it should be remembered that variations on the typical presentation can occur.

Physical History

The first step should be a comprehensive physical history. The provider should ask when your symptoms began and how they presented. If the condition arose late in life, they will usually rule out lipedema, since most of the time it starts in puberty or early adulthood.

The care provider should also ask how they have developed over time. There can be a slow progressive increase in fatness in the legs, hips, and possibly arms, but often there are periods of explosive increases, usually around hormonal events (puberty, pregnancy, perimenopause, possibly birth control or hormone replacement) or severe stress. All of this is information that should be shared with the care provider.

Care providers should also ask about symptoms such as a history of easy bruising or pain with minimal pressure on the legs. They will want to know if you experience swelling in the summer heat or after a long day, and whether that swelling goes down with rest and elevation of your legs.

Exam

Next up is a physical exam. The provider will be looking to see if what they see in your body matches up with the history you gave and typical presentation of lipedema.

The first thing a provider will be looking for is a disproportionate body, one where the body parts don't match in size (a pear shape where legs or hips are much fatter than the rest of the body, or where the upper arm is much larger than the lower arm, etc.). They will then look for the classic bilateral (both legs) and symmetrical pattern of fat deposits.

Then the provider will look closer, watching for unexplained bruises, whether the skin has the characteristic "mattress" or "orange peel" look, and whether their touch on the affected parts is painful. They may palpate the fat, trying to see if they can feel nodules of fat (like a doughy bag of small beans or peas) under the skin. The cubital area (area around the inside of the elbow) is one that some providers look at first, as well as the ankles and legs.

The provider should also look carefully around the knee area to see if there are extra pads or folds or fat. They will check around the ankle area to look for the characteristic bracelet of fat and whether any excess tissue or swelling continues into the foot. If the swelling continues into the foot, then the person either has lymphedema alone, or possibly Stage 4 lipo-lymphedema.

The bracelet of fat around both ankles ("inverse shouldering") and lack of involvement of the foot are considered classic signs of lipedema. However, as we have seen, there are some types of lipedema that don't necessarily involve a bracelet of fat at the ankle, and in Stage 4 secondary lymphedema the swelling may continue into the foot. Care providers need to be well aware of the variety of presentations that can occur with lipedema or they may inadvertently rule out lipedema cases that are a variation of the usual.

Providers should also examine the woman's arms. If both arms are involved, symmetrical, and much larger than average, than she probably also has Type IV lipedema. This is usually limited to the upper arms, but sometimes it can extend down to the lower arm with lipo-lymphedema. If so, they will look for the bracelet of fat around the wrist to see whether it overshoulders a normal-sized hand, or whether secondary lymphedema has gone into the hand as well.

Tests

There really aren't a lot of tests a provider can do to diagnose lipedema. There are no blood tests or genetic tests that will reveal its presence definitively. It's really about physical observation and patient history.

Some providers will do ratios to document disproportion in the body. For example, a document from Germany suggests that providers should do a waist-hip ratio and a waist-height ratio, although most U.S. doctors seem to omit this.

One clinical test that should be done is Stemmer's Sign. Since lipedema and lymphedema can overlap, part of the physical exam is to do tests that will help differentiate them. The classic test for this is Stemmer's Sign.

Stemmer's Sign
Stemmer's Sign is done in the following way:
Stemmer's Sign: A diagnostic test that involves tenting (pinching) the skin on the upper surface of the toes. In a negative result, which is characteristic of lipoedema, it is possible to grasp a thin fold of tissue. In a positive result, which is characteristic of lymphedema, it is only possible to grasp a lump of tissue.
In addition, care providers may also press into the leg to see if the skin rebounds back up or stays depressed after the finger is removed (pitting edema). With lipedema, the skin usually bounces back pretty quickly and does not stay "pitted," whereas pitting edema is common in lymphedema.
Pitting Edema. Most women with
lipedema will not have this
However, if expanding fat cells have interfered with the flow of lymph and severe swelling develops, it may be difficult to distinguish between lipedema and lymphedema as a diagnosis. The key is that lipo-lymphedema is usually found on both sides and lymphedema is typically found only on one side.

Most authorities agree that unless advanced lymphedema has developed, there is no need for invasive tests or imaging procedures. However, a few European providers will order an ultrasound of the affected areas fairly early in the diagnostic process, though most do not. One research article sums up the use of diagnostic imaging for lipedema this way:
Imaging techniques, such as lymphoscintigraphy, computed tomography, magnetic resonance, or ultrasound, are not routinely needed to establish a diagnosis for lipedema. As discussed, the diagnosis of lipedema is a clinical one. In some cases of lipolymphedema, where the extent of the lymphedema component is not obvious from clinical examination, and delineation would be helpful in terms of treatment planning, magnetic resonance lymphangiography is the recommended imaging modality of choice as it is the least invasive procedure and provides both anatomical location and severity assessment of any dysfunctionality within the lymphatic system.
Once a diagnosis of lipedema has been made, providers should consider ordering blood tests to check thyroid levels (which are often borderline; treating subclinical thyroid disease often helps lipedema patients) and levels of certain nutrients such as Vitamin D and B12, which can be low.

Exclusion Criteria

The research study from Germany suggests the following exclusion criteria. In other words, this author feels that if you have any of the following, you probably do not have lipedema: 
  • Lack of disproportion between upper and lower body
  • Asymmetry of both legs/ arms
  • Manifestation in late adulthood
  • Waist-hip ratio >0.85 in women/ >1.0 in men
  • Waist-height ratio: <40 years: >0.5 pathological; 40 to 50 years: 0.5–0.6 pathological; >50 years: >0.6 pathological
  • Lack of step formation in the ankle region
  • Lack of pressure-induced pain of tissue
  • Lack of tendency to develop hematomas
  • Subcutis thickness <12 mm (6–8 cm above the malleolus)
For the most part, this is probably pretty accurate, except that if the lack of a bracelet of fat at the ankle  ("step formation") were used as an absolute exclusionary criteria, then Type I and II women would be excluded, even though they clearly seem lipedemic otherwise.

Not all women have a "step formation" (bracelet of fat) around the ankle, and not all women feel pressure-induced pain in the early stages. In addition, some women with lipedema have hourglass figures instead of pear figures, but clearly have lipedemic fat.

Although most women with lipedema will have the classic shape and signs of lipedema, some women have a variation in shape or presentation that make diagnosis a bit more challenging. Although other diseases do have to be considered and ruled out (i.e., Madelung's Disease, Dercum's Disease, lipo-hypertrophy, etc.), lipedema variations should also be considered.

Summary

Although most lipedema resources emphasize the Stages of progression of the condition, it is also important to know the Types of fat distribution that can occur.

Although most women with lipedema have the Type III (full leg "pantaloon") look, there are some who have the Type I or II ("riding breeches") look, and Type IV women also have arm involvement in addition to the legs. In a very few, only arms are involved, and in another very small group, only the lower legs (Type V) are involved.

It is important to know about the Types and Stages possible so that care providers are aware of the variations that can present when trying to diagnose lipedema.

Most women do not learn about their lipedema from their local family doctor because most doctors do not know of this condition. Many women learn about it from the internet after having googled "fat legs" or something similar, and then have to search to find a doctor that knows what lipedema is and how to confirm or rule it out.

If you think you might have lipedema but have not been diagnosed, you will want to print out a few of the resources listed below (the ones from medical authorities). Print one out that it a good quick-glance summary of the condition and its most important features, then print out additional resources that are more scholarly in nature so your doctor can dig deeper if desired.

It may be that your family doctor does not feel qualified to diagnose lipedema; in that case, you can ask to be referred to a specialist. Be sure to choose one that knows about lipedema. Check the lipedema websites to see if anyone has a recommendation for specialists in your area. A doctor who treats lymphedema might be your best bet to find someone who has heard of lipedema.

*Next post: Possible causes of lipedema, and conditions often associated with it


References and Resources


Resources

*Trigger Warning: Many of these sites are not size-friendly. However, because they also contain valuable information about lipedema and its treatment, they are included here.
Blogs, Websites, and Facebook Pages from Those Dealing with Lipedema
General Information about Lipedema

Med Hypotheses. 2014 Aug 23. pii: S0306-9877(14)00295-3. doi: 10.1016/j.mehy.2014.08.011. [Epub ahead of print] Pathophysiological dilemmas of lipedema. Szél E1, Kemény L2, Groma G2, Szolnoky G2. PMID: 25200646
Lipedema is a common, but often underdiagnosed masquerading disease of obesity, which almost exclusively affects females. There are many debates regarding the diagnosis as well as the treatment strategies of the disease. The clinical diagnosis is relatively simple, however, knowledge regarding the pathomechanism is less than limited and curative therapy does not exist at all demanding an urgent need for extensive research. According to our hypothesis, lipedema is an estrogen-regulated polygenetic disease, which manifests in parallel with feminine hormonal changes and leads to vasculo- and lymphangiopathy. Inflammation of the peripheral nerves and sympathetic innervation abnormalities of the subcutaneous adipose tissue also involving estrogen may be responsible for neuropathy. Adipocyte hyperproliferation is likely to be a secondary phenomenon maintaining a vicious cycle. Herein, the relevant articles are reviewed from 1913 until now and discussed in context of the most likely mechanisms leading to the disease, which could serve as a starting point for further research.
    Diagnosis

    J Dtsch Dermatol Ges. 2013 Mar;11(3):225-33. doi: 10.1111/ddg.12024. Epub 2012 Dec 11. Thick legs - not always lipedema. Reich-Schupke S1, Altmeyer P, Stücker M. PMID: 23231593  Full text at: http://onlinelibrary.wiley.com/doi/10.1111/ddg.12024/full
    Due to its increased presence in the press and on television, the diagnosis of lipedema is on the way to becoming a trendy diagnosis for those with thick legs. Despite this, one must recognize that lipedema is a very rare disease. It is characterized by disproportional obesity of the extremities, especially in the region of the hip and the legs, hematoma development after minimal trauma, and increased pressure-induced or spontaneous pain. Aids for making the correct diagnosis are (duplex) sonography, the waist-hip index or the waist-height index and lymphoscintigraphy. Important differential diagnoses are constitutional variability of the legs, lipohypertrophy in obesity, edema in immobility, edema in chronic venous insufficiency and rheumatic diseases. The symptom-based therapy of lipedema consists of conservative (compression, manual lymphatic drainage, exercise) and surgical treatments (liposuction). Until now there is no curative therapy. Obesity is an important risk factor for the severity and prognosis of lipedema. Further studies for a better understanding of the pathogenesis of lipedema and in the end possible curative treatments are urgently needed.