Wednesday, June 10, 2015

Lipedema, Part 2: Stages and Progression of Lipedema

This documentary about lipedema is free during
all of June, which is Lipedema Awareness Month
We have been discussing Lipedema, a "Rare Adipose Disorder" (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 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. We also looked at a number of pictures that show typical features of lipedema and lymphedema.

Today, let's talk about the stages and progression of the condition.

In the next post, we will talk about fat distribution types and diagnosis of lipedema.

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

Stages of Lipedema

Stage 1, Stage 2, and Stage 3 of Lipedema,
as seen from left to right
Lipedema tends to be a progressive condition, unfortunately. It typically appears around puberty and progresses throughout life, depending on hormonal changes, stress, surgeries, and other circumstances. However, not every woman progresses to all stages. 

Different sources stage lipedema differently. Some list 2 stages, some 3, and some 4. Most sources use the 3-Stage model (like the picture shown above), but I think the 4-Stage model makes more sense.

The following summary is adapted from Dr. Herbst's article on Rare Adipose Disorders, Catherine Seo's video on her experience with lipedema, plus other resources and my own observations:
  • 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 larger 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, and fat nodules are easier to detect. Large masses of tissue can form folds and ridges ("lobular deformations"), especially just above and below the knees and on the thighs. Cankles get worse and may 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. Large gains in weight can occur, and mobility can be affected. Skin can become harder and/or discolored. In severe cases, lymph fluid can begin to leak from lymphatic vessels
Let's look at each one of these stages a bit more closely. We will use pictures to help show the differences between stages, but be warned that some pictures may be distressing to look at. However, remember that not every woman develops every stage of lipedema, and those that develop lipo-lymphedema have treatment available to them that can help lessen its severity.

Stage One


In Stage 1, the legs and hips are just somewhat heavier than normal, what some people think of as "thunder thighs" or a distinct pear shape. The woman's behind, thighs, ankles, and legs may be a little thicker than average but not too far outside the range of normal.


Generally, women are much heavier on the bottom than the top. Some may wear quite different sizes in pants versus shirts. In the picture above, notice the size of the woman's waist compared to her hips. This is the classic "pear" shape, but a little exaggerated.


Hips and thighs are usually a bit heavier than average and may flare or bulge out a bit. If the waist is much smaller than the hips and thighs, it can be hard to buy pants that fit well. Often the waist of pants must be taken in with darts, or the woman ends up buying elastic-waist pants so there is not a large revealing gap at the waistline.


At this point, the skin still looks quite normal. It is still smooth. Underneath the skin nodules of fat are starting to form ─ but the effect is not very pronounced yet. Although the legs are a little thicker than average, "cankles" have not yet really formed, and fat pads on the insides of the knees are minimal or just starting to form. However, the woman may experience a fair amount of swelling in heat or at the end of the day, which typically resolves overnight or with rest and elevation. Generally there is no big impact on the woman's health yet, and her mobility remains very good.

Stage Two

Stage 1 into Stage 2
In Stage 2, there is an increase in the amount and size of fat deposits. The nodules under the skin get a little bigger and are more noticeable, and fibers form around them and pull down to the tissue below.


As a result, the skin can now start to look uneven, with indentations (called "indurations"). This is the "cottage cheese," "mattress," or "orange peel" texture. Some people have this strongly early on, while others retain a smoother skin surface longer, even as fat pads and cankles begin to form. Usually it's worst on the thighs; the lower leg is not that affected.


Pockets of fluid may begin to develop but are not pronounced yet. The skin begins to feel more rubbery or spongy. The leg begins to lose its shapeliness and "cankles" start to appear. A bump of fat may appear above the ankles, which is sometimes called "inverse shouldering" because it can look like upside-down shoulders.


The ankles may get thicker, and a bump of fat above the ankle becomes more noticeable. There is a "stepped" appearance, which means that a care provider trying to move their hands from the foot to the leg would get momentarily stopped at this bump of fat above the ankle.


Sometimes just under that bump of fat there is a ring that looks like too-tight socks have been worn. At this point the ankles do not tend to "overshoulder" (droop onto) the foot much. Mostly there is just a ridge where the bracelet of fat ends.


Some larger mounds or ridges of fat can start to appear. Often this starts with larger fat pads on the inside of the knees, or a bit of a fold just above the knee.


Certain types of lipedema that are more concentrated around the hips instead of the legs can start to get "saddlebags." These are bumps of fat in the behind or upper thighs that tend to overshoulder the legs.


In the picture above, the woman has some saddlebags, her hips are larger than her waist, she is beginning to get the fat pad on the inside of the knee, and the skin texture is starting to change, with just a touch of the "orange peel" look. She is in Stage 2.


However, not every woman experiences lipedema in the same way. For some, stage 2 is more about losing the shapeliness of their legs and developing "column-like" legs. The woman above does not have a pear shape, but she does have the "stove-pipe" legs, some cankles, a little bit of fat pad by the knees, some change in skin texture, and edema (swelling).

In this stage, significant leg edema is still mostly transitory and resolves somewhat with elevation, rest, or treatment. Often edema and unexplained weight gain is what brings women with lipedema to the doctor to get help, but at this stage, many women are told to just eat less and exercise more because few doctors recognize their condition as lipedema.

Stage Three 


In Stage 3, more uneven fat deposits develop, and the "mattress" or "orange peel" texture becomes much more marked.


You begin to see strange folds and ridges ("lobes") of fat deposits around the thighs and knees. This fat folds over onto tissue below it, creating increased risks for skin infections.


There are often ridges of fat just over the knee, and a mound of fat just below the knee is common too. Pockets of fluid form in the lobes of fat and can deform the skin even more.


In some cases, the legs can become quite disproportionately large. This courageous woman, Claire Tickler, appeared on a TV show to raise awareness about lipedema. Notice the size of her legs compared to the host's legs, the difference in shape, as well as the bracelet of fat at the ankles. This is classic lipedema. [Bravo to Claire for raising awareness like this!]


In some cases, the legs simply become more column-like and lose most of their shape. Notice the lack of curves in the leg, the orange peel texture above the knee, the fat pad on the inside and above the knee, and the fat bracelet at the ankle.


The bracelet of  fat around the ankle can start to become very distinctive now. Notice how swollen the legs appear to be.


Again, there can be a big "stepped" difference in size between the foot and leg. Often the skin will start to droop down over the ankle and onto the foot (overshouldering) as lipo-lymphedema develops, but the foot remains normal-sized unless lipo-lymphedema becomes severe.


The texture of the skin can turn rougher and have extreme folds and fibrous lumps. Spider veins and varicosities can develop, especially as women age. Easy bruising is common.

Stage Four


In Stage 4 (or Stage 3b in some resources), secondary lymphedema develops, called lipo-lymphedema. At this stage, weight gains, edema, and fat folds can vary from significant to extreme. Notice the swelling, the orange peel texture of the skin, the overshouldering of the ankles, and the bruising on the skin ─ all typical lipedema symptoms, but more severe than in Stage 3.


The secondary lymphedema can become so significant in a few cases that it really does look like the woman has a pantaloon fat suit on over her legs, as in the picture above. Notice the extreme difference between the legs and the feet. This swelling does not resolve without treatment anymore, but treatment can make a tremendous difference if it's received in time. The woman above got Manual Lymphatic Drainage on her legs and lost 65 lbs. in 14 days. That's a lot of swelling.


Sometimes, massive swelling adds even more folds, ridges, and lobes of fat. These can deform the leg significantly. Many "super-obese" people actually have Stage 4 lipo-lymphedema that has gone undiagnosed and untreated for years.


Although most of the time lipo-lymphedema still spares the feet, the feet can also be affected now. In addition, sometimes the lipo-lymphedema is not completely symmetrical between legs either. Both of these effects can be seen above.


Occasionally the fat folds and ridges that develop can become quite severe and disfiguring, like the Michelin Man on steroids. As you might guess, this can make walking difficult. As a result, many women with severe lipo-lymphedema experience significant mobility issues. Many need wheelchairs and scooters, only to be shamed for using them as a fat person.

This lipedema patient has developed a 
secondary infection (see the redness on the lower legs)
In addition, the extreme expansion and swelling of the fat cells in late-stage lipedema makes it hard for lymph to flow normally. In severe cases, the lymph vessels can develop weak areas, break open, or even leak.

The lack of efficient lymph transport and pooling of lymph fluid can lead to a predisposition to infections, delayed wound healing, and other issues. Any redness in the skin on areas affected by lipedema needs to be taken seriously and treated immediately with antibiotics (with weight-based dosing, depending on the type of antibiotic).

Mosquito bites, spider bites, or minor trauma can quickly lead to significant problems and must be watched carefully in women with any stage of lipedema, but especially so in Stages 3 and 4.


Serious skin infections are mainly characterized by reddened, tender skin. Sometimes a fever, tiredness, and swollen lymph glands are present too but not severe, leading patients and caregivers to shrug them off. Nausea and vomiting may also occur, but may be mistaken for the flu. A particularly ominous symptom is red streaks extending from the infected area, as this indicates the infection is spreading via the lymph system.

The two main infections to watch for are erysipelas and cellulitis. Erysipelas ("air-eh-sip-eh-las") is a more superficial infection of the top layers of the skin and lymph system, and usually is marked by a raised rash with sharp, clear boundaries. Cellulitis, on the other hand, is an infection that goes deeper into the skin, subcutaneous fat, and underlying lymph system. The rash is usually not raised and its borders are not nearly as clear-cut.

Both infections are dangerous because if the infections go deep enough, they can spread into the underlying tissues (necrotizing fasciitis) or blood system (sepsis) and become life-threatening. If antibiotic doses are inadequate or if the infection is left untreated long enough, the person can die, which has happened to women with lipo-lymphedema.

Most women with lipedema do not progress to Stage 4 lipo-lymphedema, but for those who do, it can become severely disabling and even life-threatening. This is why lipedema must be taken more seriously by healthcare providers.

Summary

Below is a video comparing a mother-daughter pair at different stages of the disease. The daughter is in Stage 1, while the mother is in Stage 3. The doctor is Dr. Karen Herbst from Arizona, one of the leading specialists on Rare Adipose Disorders.



Conclusion

Lipedema can be a progressive condition, meaning it often gets worse as women age. In the past, women with lipedema were often counseled that the disease was progressive and that lipolymphedema and immobility were virtually inevitable.

 However, more recent knowledge suggests that not everyone progresses through every stage listed above. 

Some resources note that many women's cases stay fairly stable over time or only progress mildly. Anecdotally, some people seem to stay at Stage 1 their whole lives; many progress to Stage 2 but never get worse. Some get to Stage 3 but never develop the lipo-lymphedema of Stage 4. It's difficult to predict what will happen to any one individual. But have courage; lipedema doesn't have to get worse.

Doctors hope that by being aggressive in care early on, later stages and complications like cellulitis can be avoided. However, the course for each patient is different. Sometimes complications develop even when you are only in Stage 2, and some later-stage women never develop any serious complications.

Most women do not learn about their lipedema from their doctors because most doctors do not know about 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.

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: Types of Lipedema and Diagnostic Process


References and 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

Clin Obes. 2012 Jun;2(3-4):86-95. doi: 10.1111/j.1758-8111.2012.00045.x. Epub 2012 Aug 3. Lipedema: an overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review. Forner-Cordero I1, Szolnoky G, Forner-Cordero A, Kemény L. PMID: 25586162
Lipedema is a disproportionate, symmetrical fatty swelling characterized by pain and bruising existing almost exclusively among women. We undertook a systematic review of the available literature about lipedema, given the lack of knowledge and little evidence about this disorder especially among obesity experts. Diagnosis of lipedema is usually based on clinical features. Symmetrical edema in the lower limbs with fatty deposits located to hips and thighs usually appears at puberty and often affects several members of the same family. Main disorders considered for differential diagnosis are lymphedema, obesity, lipohypertrophy and phlebedema. Treatment protocols comprise conservative (decongestive lymphatic therapy) and surgical (liposuction) approaches. Early diagnosis and treatment are mandatory for this disorder otherwise gradual enlargement of fatty deposition causes impaired mobility and further comorbidities like arthrosis and lymphatic insufficiency.
Int J Low Extrem Wounds. 2014 Oct 17. pii: 1534734614554284. [Epub ahead of print] Lipedema: A Review of the Literature. Okhovat JP1, Alavi A2. PMID: 25326446
Lipedema is a disorder of adipose tissue that primarily affects females and is often misdiagnosed as obesity or lymphedema. Relatively few studies have defined the precise pathogenesis, epidemiology, and management strategies for this disorder, yet the need to successfully identify this disorder as a unique entity has important implications for proper treatment. In this review, we sought to review and identify information in the existing literature with respect to the epidemiology, pathogenesis, clinical presentation, differential diagnosis, and management strategies for lipedema. The current literature suggests that lipedema appears to be a clinical entity thought to be related to both genetic factors and fat distribution. While distinct from lymphedema and obesity, there are some existing treatments such as complex decongestive physiotherapy, liposuction, and laser-assisted lipolysis. Management of lipedema is complex and distinct from lymphedema. The role of newer randomized controlled studies to further explore the management of this clinical entity remains promising.
Acta Pharmacol Sin. 2012 Feb;33(2):155-72. doi: 10.1038/aps.2011.153. Rare adipose disorders (RADs) masquerading as obesity. Herbst KL1. PMID: 22301856 Full text available here.
Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.

Posted by at 3 comments:
Labels: , , ,

Tuesday, June 2, 2015

Lipedema, Part 1: Lipedema vs. Lymphedema


Have you ever heard of "lipedema"? Until a few years ago, I hadn't. Yet I quite likely have it, and rather significantly too. I wonder how many other fat women might have it and never realized it's a separate diagnosis from being fat?

However, when I have tried to research the condition, I've been turned off by many lipedema websites. Some pay lip service to being size-friendly but when you dig deeper, there is a whole lot of food neuroses and weight-loss rhetoric, which can be very triggering for those trying to practice Body Love and Health At Every Size®.

After looking around again recently, I decided that it's time for a more size-neutral resource on the topic. Since June is Lipedema Awareness Month, here's a summary of what I've learned about lipedema so far, presented as size-neutrally as possible. I have used a multi-part format for length considerations:
  • Part One - Symptoms and appearance of lipedema; lipedema vs. lymphedema
  • Part Two - Progression and stages of lipedema
  • Part Three - Fat distribution types and diagnostic criteria
  • Part Four - Possible causes; conditions often associated with lipedema
  • Part Five - Possible treatment options, both traditional and alternative
  • Part Six - Practical advice for living and dealing proactively with lipedema, as well as sharing a few women's stories* of lipedema
As always, I'm not a healthcare professional and don't offer medical advice. Consult your own healthcare providers. And remember, there is no one "right" way to treat any condition; inform yourself, consult the experts, and then figure out what works best for you. 

Terminology

Very few care providers in the US are aware of lipedema. Never has ANY care provider mentioned the possibility of it to me, despite my very distinct symptoms of it.

Lipedema is such an under-recognized condition that until just recently, it did not have a MESH search term in the National Library of Medicine. Nor does it yet have an International Classification of Diseases (ICD) code. The condition was only recognized by the National Health Service (NHS) in the United Kingdom in 2012. Awareness of lipedema is emerging, but very slowly in some quarters. 

One problem is terminology. Many care providers confuse lipedema with lymphedema. The spelling and the pronunciation are so similar that it's no wonder many people confuse them. However, while the two conditions can overlap somewhat, they are not the same.

Lipedema is the abnormal deposition of excess fat, usually in the extremities, whereas lymphedema is the collection of protein-rich lymph fluid in tissues, causing swelling. 

Lymphedema is pronounced "limf-eh-deem-ah." Lipedema is pronounced "lip-eh-deem-ah." In Europe and Australia they call it "lipoedema" (lye-poh-eh-deem-ah). Terms in the medical literature include:
  • Adiposis dolorosa (painful fat syndrome)
  • Lipalgia
  • Adiposalgia/Adipoalgesia
  • Lipomatosis dolorosa of the legs
  • Lipohypertrophy dolorosa
  • Painful column leg
  • Painful lipedema syndrome
  • Lipedema (U.S.), lipoedema or lipodema (U.K.), lipödem (Germany)
Here in this post, we'll just use "lipedema" for ease of use.

What is Lipedema?

In layman's terms, lipedema is an abnormal storage and distribution of fat deposits, usually in the lower body but sometimes also in the arms.

Lipedema is considered a R.A.D, a Rare Adipose Disorder. The medical definition, as defined by one source, is:
A chronically progressive, symmetrical accumulation of fat in the subcutaneous tissue with orthostatic edema occurring almost exclusively in women. 
Wikipedia defines it as:
...a chronic disorder of adipose tissue generally affecting the legs, which causes the legs and sometimes the arms to accumulate fatty tissue.
Once in a while it's called "Riding Breeches Syndrome." Some women have described it as like being held prisoner inside a fat suit on their lower body, a fat suit shaped like pantaloons (when the leg to the ankle is affected) or riding breeches (when the waist to the knees is affected). 

According to Rebecca Morris, the founding president of the National Lipedema Association (NLA), lipedema is distinctive in four ways: 
  1. It can be inherited
  2. It occurs almost exclusively in women
  3. It occurs in women of all sizes, from the anorexic to the very heavy
  4. Lipedemic fat generally is not mobilized by diet and exercise
Wikipedia adds two more defining characteristics:
  1. It involves the excess deposit and expansion of fat cells in an unusual and particular pattern – bilateral, symmetrical and usually from the waist to a distinct line just above the ankles
  2. a pathognomonic [definitive] indicator of lipedema is that the feet are spared
What Does Lipedema Look Like?

Here are some pictures to help illustrate the most important characteristics of lipedema. Be warned ─ pictures of the more severe presentations are hard to look at.

Don't panic; remember that not everyone progresses to the severe stages and that even if you do, treatment is available. The pictures merely serve as warning of why early diagnosis and effective treatment are so important.

Disproportionate Body

In the beginning, lipedema is often just a significantly disproportionate "pear" shape. Often women wear a different size on top than on the bottom.

One part of the body (often the hips and upper thighs at first) tends to be much larger than the rest of the body, as in the picture to the left. Some people think of this as "thunder thighs" but it's more than just legs that rub together a bit.

 As time goes on, more and more fat begins to be preferentially deposited in the legs and hips. Often the area around the knees begins to develop extra fat pads, and the area just above the ankles starts to get thicker. If you look closely at the picture on the left, you can see the beginnings of these deposits.

In lipedema, the lower half of the body typically is disproportionately larger than the rest of the body and can become even more disproportionate with time.

There are some exceptions to this; some women with lipedema also have significantly larger arms and sometimes chest too. Sometimes there's a lot of fat between the waist and the knees but the lower legs are not nearly as affected. However, the majority of women with lipedema start off with the classic "pear" shape and a lower body that is larger than the rest of her body.

Bilateral and Symmetrical

Lipedema is characterized by really heavy legs which are bilaterally and symmetrically affected. 

Bilateral means BOTH legs are affected (not just one). In the picture to the left, notice that both legs are indeed pretty equally affected.

Symmetrical means the fat deposits are fairly even, not all lumped up in one area. The picture shows this as well. The fat is pretty evenly distributed down the leg until it reaches the ankle, and the two legs look quite alike. 

Bilateral and symmetrical means the same even pattern of fat deposition is seen on both legs. That's very important in distinguishing lipedema from lymphedema, as you will see below.

Does Not Involve the Feet

One of the most defining characteristics of lipedema is that it ends at the ankle and does NOT involve the feet.

It's not just fat legs; it's disproportionately fat legs without fat feet too.

Notice that the legs in the picture fit the description. Both legs are affected pretty equally and evenly (bilateral and symmetrical), but the feet are not affected. That's one of the key symptoms doctors will look for when diagnosing lipedema.

However, it should be noted that if severe secondary lymphedema develops, sometimes the feet can also develop swelling. Thus, in the early to middle stages of lipedema, feet will not be affected, but in the later stages, feet can sometimes be affected too. 

Legs That Lose Their Shape

In early stages of lipedema, there is still some shape to the legs and ankles, but as the condition progresses, the shapeliness of the legs begins to disappear.

Lower legs and ankles start to get thicker and the curves begin to disappear.

Sometimes this is called by the lovely nicknames of "columnar" legs, "piano legs," or "stove-pipe" legs.

This is because as the fat deposition progresses, the leg shape is lost and the legs become more like a column, going mostly straight up and down.



"Cankles On Steroids"

 Society likes to call thick ankles "cankles" (i.e. calf-ankles), but lipedema involves a much more severe presentation ─ basically, cankles on steroids.

In the early stages of lipedema, the lower legs become slightly thicker than normal but you don't really see cankles.

In the picture to the left, the woman still has shape to her legs but her lower legs are starting to get a bit thicker and you can just begin to see the suggestion of deposits of fat above the ankle. This is an early stage of lipedema.



Little by little (or sometimes in a fairly short period of time in response to a major hormonal change or severe stress), more and more fat begins to be deposited by the ankles.

As the leg becomes more column-like and less curvy, a "stepped" appearance from the feet to the legs begins to appear.

As some doctors describe a "stepped" appearance, if you ran your hands around the feet and then up to the ankles your hand would get stuck momentarily by the increase of fat at or just above the ankles. You would not have a smooth transition from the foot to the leg because of the significant increase at the ankles.


As lipedema progresses, it often looks like you've worn socks that were too tight and this has left a ring on the skin. This "ring" around the ankles is a classic sign of middle-stage lipedema.  If you look carefully, you can see that "ring" in these two pictures.

In time many women with lipedema develop a bump of fat just above the ankles, as you can see in this close-up just above the "ring." This is what gives the leg the "stepped" appearance.

In the later stages of lipedema, the bump gets large enough that it is very distinctive. Many lipedema resources talk about this characteristic "bracelet" of fat just above the ankles on both legs. Some doctors call this bracelet of fat "inverse shouldering" or "overshouldering."  

As the condition worsens, the shape of the ankles begins to disappear. Eventually, the "shoulders" droop down to hang over the ankles.

This makes it extremely difficult to get socks or boots that fit.

When it's severe, the "overshouldering" of the ankles can droop way down past the ankles. Sometimes the swelling extends nearly to the floor. The skin can become quite prone to infections.

In the picture to the left, the woman obviously has developed secondary lymphedema and her legs are severely affected. However, note that the feet are not involved and that both legs are pretty equally affected. This is probably lipo-lymphedema rather than straight lymphedema. At this point, it really is like having a "pantaloon" fat suit over your legs.

Obviously, this is an extreme stage and most women with lipidema do not experience this level of overshouldering. At this point, some major Manual Lymph Drainage treatment and compression wrapping is needed to reduce the size of the ankles and legs and to restore comfortable functioning.

 "Mattress" Skin

In lipedema, the skin starts smooth in the early stages, but as the condition progresses, the skin often develops an appearance some people compare to cottage cheese, a mattress, or an orange peel, as in this picture. Beneath the skin, you may feel little nodules or lumps of fat that feel like little peas or beans under the skin. As the condition progresses, these may start to feel larger. This is probably what causes the "cottage cheese" appearance.

Big Hips, Behind, and Abdomen

Although lipedema is usually thought of as happening primarily in the legs, it can occur from the waist down (including abdomen and hips).

Often the person has a lot of "junk in the trunk," with a very large behind. Sometimes it tends to jut out and become what some people crudely call a "shelf butt." Or the lower abdomen can grow quite large, especially with repeated bouts of weight loss/weight regain, after pregnancy, or with secondary lymphedema.

This can make finding clothes very difficult. Often, women must purchase pants that are far too large in the waist in order to find some that fit in the hips, abdomen, or thighs.

Arms Can Be Affected Too

Sometimes the arms develop lipedema too. One well-known expert in the field states that about 30% of people with lipedema also have arms that are affected.

In the picture to the left, notice that the upper arm is very heavy but that the lower arm is not. This is a common lipedema pattern.

These extremely heavy upper arms can make it very difficult to find shirts that fit. Shirts that are wide enough to fit the upper arms are often far too loose on lower arms. Most short-sleeve shirts don't begin to cover the whole upper arm and can cut uncomfortably into the arm.

The proximity of the arms to the face means that upper-body lipedema is more noticeable to others, and women with affected arms often get more remarks and negative attention. Heavy legs can be covered up by loose pants and long skirts, but lipedema arms are more noticeable, covered or not.

Although most women with arm lipedema experience it only in the upper arm, sometimes the lower arm is heavy too, as in this second arm picture. In these cases, the "bracelet of fat" that is usually seen around the ankle can also be seen around the wrist, as in the picture.

Interestingly, for whatever reason, in lipedema the arms do not usually develop the typical "cottage cheese" skin texture as badly as the legs often do. They can have the nodules of fat that the legs get, but they may not be as noticeable.

Folds and Ridges of Fat

As lipedema worsens, more and more fat is deposited on the legs. This begins to deform the leg in different ways with harmful side effects.

Frequently there is a big pad of fat on the inside of the knees, as in the picture to the left. This can begin to alter the woman's gait; an altered gait can in turn lead to issues with the feet, hips, and back.

The extra fat pads and altered gait can make exercise more difficult and painful, leading to decreased fitness as the lipedema worsens.

In addition, fluids from the abnormal fat deposits can leak into the knee itself, making it very prone to arthritis at earlier ages than normal. This, too, increases the pain associated with exercise and makes it difficult to keep up fitness.

In time, large lumps, folds, and ridges can begin to appear. Often these folds and ridges develop around the knees first.

It is very common to develop a mound of fat tissue just below the knees, as you see in the picture to the left. Notice there is also some folds and ridges of fat above the knee as well. The upper leg  has the classic orange peel look, and the abdomen is also enlarged.

This picture shows a variation in lipedema in that there is less of the typical bracelet of fat around the ankles. This woman has a slightly different type of lipedema, the "riding breeches" version, where the excess fat extends from the waist down to just below the knee and mostly spares the ankles. Anecdotally, this type seems to be particularly prone to secondary lymphedema.

As lipedema progresses into more severe stages, even larger masses of tissue can develop and the leg can become quite large.

This woman probably also has the "riding breeches" form of lipedema that is heaviest from waist to just below the knee. She does have a small bracelet of fat around the ankle, but she is much larger in the abdomen and top two-thirds of her leg.

Secondary lymphedema has developed here, causing additional folds and ridges of fat to appear. Notice the fold on the outside above the knee, the fold and ridge below the knee, the fold on the inner thigh, and the "saddlebag" fold near where the hip and thigh meet. This kind of deformation is common with lipo-lymphedema and can often be reduced considerably with treatment.

Summary

 Lipedema presents with certain key characteristics, including a disproportionate body shape, bilateral and symmetrical fat deposition on the legs, dimpled "mattress" skin, and a bracelet of fat around the ankle in many (but not all) cases. Probably the most definitive characteristic of lipedema is the lack of involvement of the foot. This is one feature that doctors particularly look for when trying to differentiate lipedema from other possible diagnoses.

If lipedema worsens, folds and ridges of fat can occur. If secondary lymphedema develops, these can become extremely large, severely deforming the leg and making it difficult to walk. Sometimes the lymphedema also spreads into the foot at this stage.

The good news is that treatment may be able to help slow down or even prevent progression of the condition into lipo-lymphedema.

Other Symptoms of Lipedema

Although the strongest characteristics of lipedema are the ones described above, there are other symptoms commonly found among many people with lipedema. These include:
  • High sensitivity to pain in the legs
  • Tendency towards easy bruising on the legs
  • Cold legs and feet
  • Edema/swelling at times
  • Spider veins/varicose veins
  • Tendency towards unexplained weight gain
  • Difficulty losing weight, especially in the lower body
Let's discuss each one of these a bit more closely.

Pain in the Legs

 One classic sign of lipedema (but not lymphedema) is high sensitivity to pain with even minimal pressure on the legs, especially on the shins and calves. This sensitivity to touch does not extend to the whole body. Lower leg massage may be too painful, but massage on the back is fine (unless there is fibromyalgia). The pin-point pressure of a cat walking up the leg can cause extreme pain, whereas the same cat walking on the abdomen is fine.

In addition, many women with lipedema report a deep, aching pain from inside the legs, which is why it is sometimes called Painful Fat Syndrome. Some sources believe that this is a separate condition (Dercum's Disease), while others believe that it is part of the spectrum of lipedema. But because Dercum's Disease can occur in several places in the body and onset is usually around menopause (lipedema mostly occurs in the lower body and onset is usually puberty), they are usually considered separate conditions.

Easy Bruising

People with lipedema also tend to bruise easily due to increased capillary fragility. Although women with lipedema are not usually covered in bruises, they do tend to bruise more easily than others, and often have bruises on their legs they can't explain away easily.

Cold Legs and Feet

Many women with lipedema experience cold legs and feet (hypothermia of the skin), although this symptom tends to get overlooked a lot. The woman may have cold feet or legs even when others around her are comfortable, or the skin on her legs may be much colder than on her upper body. This can be a significant source of discomfort for some.

Anecdotally, some women find that it takes significant outside heat sources (hot pads, other people, etc.) to warm up their feet or legs in cold weather. The best guess is that the increased adiposity in the legs interferes with blood and lymph flow, making it harder to regulate temperature.

Swelling

Many women with lipedema experience swelling in the extremities during hot weather or after a long day on their feet. They may find that their fingers swell so much during a walk on a hot summer's day that they cannot tolerate wearing rings at all in the summer.

At first, this swelling resolves pretty easily with rest and elevation, but as lipedema progresses, the swelling tends to not resolve as well due to the lymph system becoming increasingly compromised.

Spider and Varicose Veins

Spider veins and varicose veins are also common in people with lipedema. Spider veins ("telangiectasias") are tiny red veins you can see on the surface of the legs. This is probably due to damage to capillary blood vessels. Varicose veins are also often found.

Unexplained Weight Gain

Many women with lipedema have a history of large, unexplained weight gains, especially associated with major hormonal changes (puberty, pregnancy, perimenopause, and perhaps hormonal birth control) or stress (which increases cortisol and can trigger an inflammation cascade).

Some experience sudden weight or fat gains even when their habits have not changed. Sometimes this gain is mild, sometimes it is extreme. Often it occurs despite aggressive attempts to stop it via diet and exercise.

Difficulty Losing Weight

Many women with lipedema have great difficulty losing weight, especially in the lower half of their bodies. Most have tried very hard to diet away the extra fat. Sometimes weight loss occurs, but is lower in amount than what would be expected by caloric count, and it often returns with a vengeance afterwards. Some even gain weight while on a diet and stringent exercise plan.

Even when weight is lost in the upper body, very little is lost in the lower body, including after bariatric surgery like gastric bypass. All this suggests strongly that there is something going on here beyond simplistic "calories in, calories out" calculations.

Summary

All of this is part of the symptom spectrum of lipedema. You don't have to have all of these symptoms to have lipedema, but if you have most of them, you probably have lipedema. If in doubt, consult your doctor ─ just make sure he/she is well-educated about lipedema first so you can get an accurate evaluation.

Lipedema vs. Lymphedema

Lymphedema is much different than lipedema. Lipedema is caused by the abnormal deposition of fatty tissue. Lymphedema has to do with impaired flow of lymph fluid and the resulting swelling.

Again, although people with latter-stage lipedema often develop lymphedema as a complication because fatty deposits can impair lymph flow, the reverse is never true ─ people with lymphedema do not develop lipedema as a complication.

One definition of lymphedema can be found here:
Lymphedema is an accumulation of lymphatic fluid in the interstitial tissue that causes swelling, most often in the arm(s) and/or leg(s), and occasionally in other parts of the body. Lymphedema can develop when lymphatic vessels are missing or impaired (primary), or when lymph vessels are damaged or lymph nodes removed (secondary). 
When the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid not only causes tissue channels to increase in size and number, but also reduces oxygen availability in the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in lymphangitis (infection).
In contrast to lipedema, lymphedema affects feet as well as legs, as can be observed in the picture to the left. This is one of the most important ways to distinguish between the two conditions.

In lymphedema, the swelling often starts in the toes and continues upwards, whereas with lipedema the fat deposits start near the waist and extend downwards to the ankles.

Occasionally, lipedema patients will also have swelling of the feet, but typically this only happens with either temporary lymphedema due to trauma like surgery, or when secondary lipo-lymphedema has developed.

Onset during puberty is common with lipedema, whereas with lymphedema, onset can occur at any age. Lymphedema is often seen after major cancer surgeries, for example, when the lymph system has been damaged.

Another important difference is that lymphedema often affects only one limb, and is usually not symmetrical all over. Note in the picture to the left the difference in size between the leg. Notice also that one foot is swollen and the other is not. This is clearly lymphedema.

Sometimes lymphedema does involve both legs, but that's unusual, and if it happens, the pattern of swelling almost always involves the feet.
.
Here is another picture of a lymphedema case. Notice that only one leg is affected, and there is a huge difference in size between the two legs. Notice also that while there is "overshouldering" of the ankle on the affected leg, the foot is also affected and is much larger than the foot on the other leg. Nor does the skin have the characteristic "orange peel" or "mattress skin" look that you would expect if the leg on the right were truly from lipedema.

Lymphedema can occur in males or females, whereas lipedema is usually limited to females. There is no increased bruising with lymphedema, and the skin texture is harder or more fibrotic, while it is more rubbery with lipedema. Sometimes there are changes in skin color with lymphedema, whereas this is less common with lipedema (unless secondary lymphedema or an infection has developed).

In addition, people with lymphedema may get pitting edema (where you press down the skin and it stays down), as seen in the picture to the left. Pitting edema is rare in lipedema, however, unless secondary lipo-lymphedema has also developed.

Abnormal retention of fluid and difficulty moving lymph is part of both syndromes. In lymphedema, it's usually caused by trauma to the area (like surgery, radiation, chemotherapy) or from vascular anomalies.


Lipolymphedema
In lipedema, lymph issues are caused by the abnormal deposition of fat cells blocking lymph flow. As noted, this causes severe swelling and deforming ridges and folds of fat.

Notice that while the woman on the left clearly has lymphedema, both legs are affected, the feet are much smaller, and there are characteristic fat pads at the knees and over-shouldering around the ankles. This is a LIPEDEMA patient who has also developed secondary lipo-lymphedema.

When lipedema patients develop significant secondary lymphedema, it can become very extreme and debilitating, even life-threatening. The goal of diagnosis and treatment of lipedema is to keep it from progressing to these severe stages.

Summary

 Lipedema was first described in the medical literature in 1940 by Doctors Allen and Hines of the Mayo Clinic. However, it did not become well-known among U.S. providers. European providers were also slow to recognize lipedema, but generally have more awareness of it today than U.S. providers. Many countries outside of Europe and the U.S. have even less awareness of it.

One important point is that we do not truly know how common lipedema is. It is called a "Rare Adipose Disorder" but it is probably not that rare. The Lipoedema Australia Support Society (LASS) says that 5-15% of women may have it. One epidemiological study estimated that about 11% of women have lipedema. If around 1 in 10 to 1 in 20 women have a condition, that's not "rare."

Since these figures are only guesses, they could be over-estimates, but then again they may not be counting many early cases which are hard to identify. Does every woman with a pear-shaped body have lipedema? Hard to say. Best guess is that the women with true lipedema are those with pronounced "pear" shapes and whose bottom halves put on fat in response to hormonal challenges or major stress. But what percentage of women fit this criteria is hard to say.

Because it is relatively unknown, it is common for women to go for years without being diagnosed. Women with lipedema are simply seen as fat by the public and by most doctors. Often they are pressured into diets, weight loss surgery, and/or extreme exercise to treat it. Many are shamed and mistreated due to obesity stigma and prejudice.

However, the unique pear-shaped silhouette, the sparing of the hands and feet, and the progressive nature of condition despite all attempts at diet and exercise certainly suggest that it is a real medical condition that is more complicated than "just" obesity.

Sometimes, lipedema is shrugged off as lymphedema, but the two conditions have very distinct differences. However, the lines can be blurred in the late stages because lipedema patients can develop lipo-lymphedema.

Often, lipedema patients are only diagnosed because they have been referred to lymphedema specialists who have more recognition of lipedema as a condition. By that time, however, the lipo-lymphedema has often advanced so far that the condition has become quite disabling or even life-threatening.

A significant percentage of fat people probably have lipedema and are going undiagnosed and untreated for it. This is why I want to bring particular attention to it on this blog.

This lack of recognition is probably leading to many cases of severe lipo-lymphedema that might have been prevented by having more awareness and earlier treatment of lipedema.

It is time for more care providers and consumers to learn about lipedema and how to care for it. It is time for the general public to learn about lipedema so there is less stigma attached to it. And it is time for women with lipedema to take back their power and learn to live proactively with it.




*Do you have a Lipedema story you'd like to share? Please email it to kmom AT plus-size-pregnancy dot org. 


Resources and References

 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.

Posted by at 20 comments:
Labels: , , , , , , , ,
Subscribe to: Posts (Atom)