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.




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    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.

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