May 30, 2018

What is Lipedema

Pear shaped body shape
Pear shaped body type

Lipedema was first formally identified in the U.S. at the Mayo Clinic in 1940 (1).  However, not much research and diagnosis criteria have been established to battle this adipose tissue disorder.  There is no cure.  Physicians are not readily trained to diagnose Lipedema from Lymphedema and general obesity.  More training and education is needed in the area of adipose tissue and lymphatic disorders to facilitate patients into proper treatment! Let’s learn more about what is Lipedema.

Defining Lipedema

Lipedema (or Lipoedema as it is spelled in the European community) is a progressive chronic disease of fat tissue that affects more than 11 percent woman and less than 1 percent of men worldwide. This adipose tissue disorder is characterized by bilateral symmetrical swelling of the legs, thighs and hips.  While the upper torso usually remains the same, the hips and thighs usually start enlarging disproportionately to the rest of the body thus giving you a pear shaped body appearance.  Clinicians and patients commonly confuse Lipedema with general obesity, which classically has an apple shaped body appearance.

Lipedema Infographic
Lipedema Infographic via Lipedema Simplified

Lipedema is often called the “painful fat syndrome” as abnormal fat cells cluster together and expand under the skin. Interstitial fluid increases between the clusters creating pain to touch or when standing.  People in the later stages often complain about pain when being touched where pockets of fat press on nerves.

They may also easily bruise either from bumping into things or overstretching your limb when capillaries embedded in the fat cluster burst.  Since Lipedema can also be present in the upper arms where touch is painful, people should never have their blood pressure taken in the upper arm.  Tell your clinician to take your blood pressure in your lower arm only!

Cause of Lipedema

The cause of Lipedema is unknown at this point.  There is some data available that genetics could be involved as evidence of mother and daughter emerging withe Lipedema has been seen.  Researchers in this area are also investigating whether metabolics, inflammatory and hormonal changes are responsible for the development of the disease.  Whatever the etiology of this disease it is still not readily recognized by the medical community with many women being undiagnosed for years!

Many women report the most significant changes in adipose fat occur during life changes, such as puberty, childbirth, surgery and menopause when hormones are fluctuating. Mothers should pay careful attention to their daughters during and following puberty for any abnormal fat development or pain when touching the skin.  It is imperative that women be vigilant about changes to their bodies, particularly the hips, thighs and lower legs after a physical life change so that early diagnosis and treatment can begin.

Immune to Diet and Exercise

Lipdema does not discriminate on body size or shape and ethnicity.  Women who are thin and obese are equally at risk of developing this disease.   It is however, a predominately woman based disease, some are surmising it is mostly confined to genetics of the X chromosome, with less than one percent of Lipedema occurring in men.

This fat disorder is also resistant to diet and exercise.  Many women try dieting and exercising so hard and yet there are no changes in their hips, thighs and legs.  They may end up losing some weight in the upper torso area where Lipedema is not present, but nothing below the lower torso is affected.

Imagine the frustration when discussing this problem with medical staff as they often hear that they are not eating a healthy diet, cheating on their diet or they need to exercise more.  This can be so demeaning and frustrating for women searching for answers to feel blamed and shamed for something that it out of their control!  Lipedema is not your fault and as of yet there is no cure for this disease!

Lipedema Phenotypes

Lipedema TypesLipedema can affect one or more areas of the body in people with the disease.  Because not all people present with the same exact affected areas it takes a clinician skilled in adipose tissue disorders to diagnose it properly, especially when the disease is in its early stages.

Type 1 – Buttocks: excess buildup of fat in the buttock area disproportionate to other body areas.  Can cause discomfort when sitting and moving around.

Type 2 – Buttock, Hip and Thigh: excess buildup of fat in buttock, thigh and thigh area with swelling causing pain and discomfort.  More common than Type 1.

Type 3 – Buttock, Hip, Thigh and Calves: excess buildup of fat in the entire lower body area with swelling causing pain and discomfort. Most common type of Lipedema.

Type 4 – Calves: (or “cankles”) less common only calves are involved. Swelling in ankles causing pain and possible mobility issues.

Type 5 – Upper Arms: least common type of Lipedema. Heaviness and swelling in the upper arms cause discomfort.  Can present with any of the other types of Lipedema.

Can you identify what areas of your body are affected by Lipedema?  In addition to body types there are progressive stages of Lipedema.

Stages of Lipedema

Lipedema is a progressive fat disease meaning if no treatment is applied fat cells will increase in size and cluster, As the disease progresses the hips, thighs, lower legs and sometimes the upper arms will increase in size and pain.  The skin texture will change from spongy fat to a dimpling effect as fat cells create bulging in the hips, thighs and legs.  Eventually the lymphatic and vascular systems become compromised causing excessive painful swelling, excess skin pockets that fold over the legs, knees and ankles and severe lymphedema.

Recognizing the stages of Lipedema is critical to getting a diagnosis and a treatment plan to avoid progression to the next stage.  There are four stages of this disease:

Four Stages of Lipedema
Four Stages of Lipedema via the Lipedema Project

Stage 1

This stage is probably the most difficult to diagnose as it may appear like normal weight gain.  This is where you need to be evaluated by someone trained in fat disorders or who can distinguish the difference between normal fat and Lipedema fat.

Here are the most common symptoms:

  • Excess fat in legs that looks disproportionate to the upper body.  Weight loss does not affect the lower body area.  The look and feel of fat is symmetrical and evenly distributed from the hips to the ankles. Padding around knees can make it harder to differentiate normal from Lipedema fat.
  • Skin is generally smooth, healthy in color and not discolored.  May feel spongy to the touch.
  • No excess fat in ankles or feet.
  • Pain may be present with or without pressure.
  • Fat deposit may feel lumpy and be dispersed among affected area, like peas or marbles.
  • May bruise more readily in affected area
  • Stemmer’s sign is negative (when you pinch the skin near the toes you can lift the skin off the foot).
  • No pitting of the skin (pressing in the skin with your thumb does not leave an indent that slowly fills and disappears).
  • May have swelling in the ankles or feet that disappear when legs are elevated.
  • Responds well to treatment.

Stage 2

When left undiagnosed and untreated Lipedema will progress to the next level.

Here are the most common symptoms:

  • All of the stage 1 symptoms
  • Skin starts to show indentations
  • Lipomas may develop (large fat clusters ranging from pea size to fist size located between the skin and muscle layers)
  • Dry itching skin or Eczema in patches across the Lipedema areas
  • Swelling of the legs, especially lower legs, that when elevated does not bring much relief or reduction
  • Should respond well to treatment

Stage 3

Unlike Stage 1 and Stage 2 progression, which may happen in a short period of time, Stage 3 may take several years to develop.

Here are the most common symptoms:

  • Excess skin and fat in the thighs and legs continue to grow in size and eventually create skin folds and creases (also known as saddlebags, shelves or cuffs) that overhang knees and ankles.
  • Skin is largely bumpy in look and touch.
  • Hardened connective fibrotic tissue is present (from stagnation of fluid and protein movement)
  • No excess fat in ankles or feet.
  • Lipomas may increase in number and size and are painful with or without touch.
  • Appearance of varicose and spider veins
  • May present with Livedo reticularis, which is a netlike mesh pattern of capillaries showing just under the skin
  • Bruising happens more readily in affected areas.
  • Lower legs, feet and toes may feel colder than the rest of the body (a form of hypothermia)
  • Stemmer’s sign is still negative.
  • Pitting of the skin is present.
  • Swelling is consistently present and will not be relieved by leg elevation or sleeping.
  • Mobility and gait issues may occur from the location of excess fat interfering with normal daily activities.
  • Treatment is less responsive in this stage

Stage 4

Unlike Stage 1, 2 and 3 progression, Stage 4 may take several more years to develop. This stage incorporates a secondary condition called Lymphedema, which is caused by severe Lipedema fat deposits blocking key areas of the lymphatic system. Even though the lymph system tries to create new pathways there is too much fluid and protein retention to adequately move out the legs, thighs and hips causing extreme swelling.  This stage is also known as Lipo-Lymphedema.

While Lipo-Lymphedema is the most common type of Lipedema at this stage someone may also present with Lipo-Phlebolymphedema, which includes the symptoms per stage below, and a reddish or purplish discoloration in the lower legs between the calves and the ankles. This discoloration, or hyperpigmentation, is a sign of chronic venous insufficiency (CVI) where inflammation of the subcutaneous fat causes blood to pool in the affected area.  If left untreated someone may be looking at the further development of lipodermatosclerosis, which is the hardening of the affected skin area.

Here are the most common symptoms:

  • Excess skin and fat in the thighs and legs continue to grow in size, eventually create skin folds and creases that overhang knees and ankles.
  • Skin increases in its bumpy look and touch. May also present with hyperkeratosis or thickening of the outer layer of skin.
  • Additional massive localized lymphedema lobules may appear on the back of the leg(s) or inner thighs making walking difficult to impossible (immobility).  Legs are no longer symmetrical and could present as elephantiasis.
  • Hardened connective fibrotic tissue is present in multiple areas.
  • Some may have excess swelling in feet.  (Stemmer’s sign is positive.)
  • Appearance of varicose and spider veins
  • May present with Livedo reticularis, which is a netlike mesh pattern of capillaries showing just under the skin
  • Lower legs, feet and toes may feel colder than the rest of the body (a form of hypothermia)
  • Lipomas may increase in number and size with increased pain regardless of touch or not.
  • Venous Insufficiency (inability to maintain good blood supply through the affected area) may occur causing skin dermatitis or varicose veins.
  • Lipedema may spread to both upper arms causing significant swelling and pain from lipomas.
  • Lipedema may spread to abdomen causing creation of large panis (belly that hangs down past private areas) and may be painful from lipomas. (Causes problems with mobility and gait.)
  • Increased occurrence of sores and tears in the skin from cellulitis infection and poor hygiene.
  • Increased bruising happens more readily in affected areas.
  • Pressure ulcers may develop if immobile or sitting/lying down too long
  • Pitting of the skin is present.
  • Swelling is consistently present and will not be relieved by leg elevation or sleeping.
  • Treatment is difficult.  Need to treat for Lymphedema (covered in separate section).

Here is a video from the Lipedema Project demonstrating a mother and daughter with Lipedema:

Anyone experience additional signs of Lipedema in the various stages?  Can you identify where you might fall in the various stages of Lipedema?  I am definitely Stage 4 and have been for many years.  It can be disfiguring and very challenging to walk.  My prayer would be for anyone with Lipedema to be diagnosed early and get treatment so that progression can be halted in Stage 1 or 2 without all the debilitating problems that come in Stage 3 and 4!

Check out our next section on diagnosing Lipedema.

To your improved health!

 

References:

(1) –  Allen E V, Hines E A, Lipoedema of the legs. A syndrome characterized by fat legs and edema. Proc Staff. Meet. Mayo Clinic 1940; 15:184

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