The diagnosis of Lipedema is made based on a clinical evaluation from a physician with specific knowledge and experience of the disease and with supporting tests that rule out other diagnoses. Lipedema is a poorly recognized and under-appreciated disease in the United States. It is estimated that 11% of the female population has this condition. However, the diagnosis is rarely made by US physicians, which is in large part a result of poor awareness and understanding. The recognition of characteristic features of Lipedema early stages and elimination of other conditions that can be confused with lipedema is the key to the proper diagnosis of lipedema.
Currently, there is not a specific code for lipedema in the coding system the US is currently using. The current coding system is called the ICD-10 International Classification of Diseases. There are no specific codes for lipedema in the ICD 10 diagnosis coding system.
However, there are several different non-specific ICD-10-CM codes: R60.9, Q82.0 [hereditary lymphedema, and E82.0 [lipomatosis] that can be used. Lipedema is only found in the ICD-10-CM index crosslinked to R60.9: Edema. None of these codes are specific or adequate, ultimately the necessary changes should reflect a specific code for proper diagnosis and treatment of Lipedema.
Dr. Herbst, Dr. Wright, Dr. Shapira, and Dr. Steve Dean, with the support of the American Venous and Lymphatic Society (AVLS), have submitted to a division of CDC the National Center for Health Statistics. NCHS is set to add the code, however, the code application was tabled.
Abnormal Fat Deposition
One of the key distinguishing features of lipedema is the characteristic way affected individuals deposit fat. In people without lipedema excess calories are predominantly deposited in visceral [ Intra- abdominal] fat stores and a very small amount is deposited in the skin of the extremities. However, in individuals with lipedema this is reversed so that when fat accumulates, it predominantly accumulates in the extremities and less inside the abdomen. Moreover, the fat accumulates in the extremities in characteristic areas in a symmetrical manner. The subcutaneous fat gathers around the upper buttocks, inner thighs, inner part of the knees, in a cuff around the ankles/Lipedema ankles, and around the elbow.
Not all individuals with lipedema have fat collected in the same areas; it depends on the type of lipedema. There are 5 types of lipedema and the types are not mutually exclusive. Type I fat collects on the buttocks, Type II fat collects on the buttocks, hips, and thighs, Type III fat collects on the thighs, calves, and ankles, Type IV on the arms, and Type V on just the calves and ankles.
Stage 1 Lipedema
Stage 2 Lipedema
Stage 3 Lipedema
Lipedema Symptoms and Signs
In addition to the characteristic disproportionate fat accumulation, there are specific physical signs and symptoms of lipedema:
- Tender skin and soft tissue in the affected areas that are sensitive to pressure and walking.
- The skin and soft tissue in the affected area initially have a fluffy or slightly rubbery feel. As the disease progresses the skin becomes harder and feels nodular like beans in a bean bag or rubber super balls.
- As the disorder advances, the skin loses elasticity, and the skin surface becomes uneven, with protruding hills and fibrous valleys developing.
- Decreased skin temperature or a relative coolness of the skin.
- Leg heaviness and fatigue, which can lead to overall fatigue – Lipedema pain in legs.
- Swelling in the affected area. Initially, the swelling worsens during the day and resolves at night with elevation. As the disease progresses the swelling becomes persistent and does not resolve with elevation overnight. At the more advanced stages, the swelling affects the whole limb so that the feet and or hands swell.
- Easy bruising and visible veins.
- Disproportionate fat accumulation, which is not affected by calorie restriction.
- Feet and hand relatively spared of swelling.
Lipedema Related Conditions
Below are conditions that can be confused with lipedema and can affect Lipedema. Because there can be so much overlap in appearance and Lipedema symptoms and signs, only clinicians with the training and experience to diagnose and treat these conditions can differentiate them. *1
|Lymphatic Fluid is created by the pressure difference at tissue level. Higher the venous pressure more lymph is created|
All but the mildest cases of lipedema have some swelling; however, lipedema in its earlier stage spares the feet and hands, whereas lymphedema always has to swell in the feet and hands. Lipedema is symmetric affecting both legs equally where lymphedema usually affects one leg or arm. While they are separate diseases, there is quite a bit of overlap as well. As lipedema progresses, secondary lymphedema often occurs where the feet and or hands swell.
Both lipedema and lymphedema benefit from compression and both affect the lymphatic circulation, but they do it differently. Lipedema early stages show some irregularity in the lymphatic collecting system but have normal flow and uptake to the regional lymph nodes.
Lymphedema typically has delayed lymph flow and uptake at the regional lymph nodes. It can sometimes be difficult to separate the two disorders, especially in more advanced cases, because lymphedema can develop a secondary lipo-lymphedema as fat accumulates in limbs that have poor lymph circulation, which is responsible for clearing fatty acid from the tissue. In the more complex cases, it is necessary to evaluate the lymphatic system with lymphoscintigraphy to separate lymphedema and lipedema.
Obesity is much more common than lipedema. Lipedema is often confused with obesity, but as mentioned above, obesity is the accumulation of excess fat stored centrally inside the abdominal cavity or belly. Whereas, lipedema is the excess accumulation of fat out of proportion on the extremities. What can make it confusing is that early on the conditions may be distinctly different but in more advanced cases both lipedema and obesity may be present. In fact, sometimes obesity develops as a secondary condition due to the mobility problems caused by lipedema. So, not only is lipedema often misidentified as just obesity, but obesity can also complicate lipedema. Often a vicious cycle develops where increasing weight gain accelerates the lipedema fat deposition and the lipedema causes fatigue and hinders mobility which worsens obesity.
Venous Insufficiency and Veno-Lipo-Lymphedema:
Venous insufficiency is a very common disease affecting between 10 – 40% of the adult population. More advanced cases of venous insufficiency, class three and above, have associated edema, veno-lymphedema, in the affected limbs. Venous insufficiency leads to increased venous pressure, which overwhelms local lymphatics causing swelling in the affected limb. Lipedema symptoms and signs are similar to venous insufficiency. They both cause heaviness, tenderness, fatigue, and swelling. They often both have discoloration in the shins, easy bruising, and prominent veins. In more advanced cases of venous insufficiency, not only does venous lymphedema develop but secondary Veno- Lipo- Lymphedema develops.
With the overwhelmed lymph circulation, the ability to clear fatty acids from the affected tissue is compromised and a secondary fat accumulation occurs. This secondary fat accumulation can look very much like lipedema. In many cases, the best way to differentiate between lipedema and venous insufficiency and veno- lipo-lymphedema is to have a specialized standing venous Doppler ultrasound to check for venous reflux.
Table of Differences for Lipedema Diagnosis
|Location||Fat deposits/swelling in legs and/or arms NOT hands/feet. Lipedema leg pain||Fat deposits / swelling in one limb including hands/feet||Fat deposits central> limbs widespread||Fat deposits / swelling widespread in legs/arms/torso||Swelling near ankles; brownish discoloration of the legs. Minimal swelling possible.|
|Onset||Around hormonal shifts (puberty, pregnancy, menopause)||After infection, injury affects lymphatic system or rarely at birth||Any age||After injury to lymphatics||Any age but tends to occur in 30-50’s|
|Effect of diet||Restricting calories ineffective||Restricting calories ineffective||Diets and weight loss strategies often effective||Restricting calories ineffective||No relation to caloric intake|
|Edema type||Non-pitting edema||Pitting edema later non-pitting||No edema||Lots of edema; some pitting; some fibrosis||Often edema, but can also occur without edema in earlier stages|
|Stemmer’s sign*||Stemmer’s Sign negative **||Stemmer’s Sign positive||Stemmer’s Sign negative||Stemmer’s Sign positive||Stemmer’s Sign positive or negative|
|Pain||Pain in affected areas likely||No pain initially||No pain||Pain in affected areas||Pain is likely|
|Prevalence||Best estimate is 11% adult women (study done in Germany)||Low||>=30% of US adults||Unknown; best estimate is a few percent of adult women||>30% of US adults|
|Infection history||No history of cellulitis||Possible history of cellulitis||Likely history of cellulitis||Often itching +/- discoloration mistook for cellulitis|
|Family History||Family history likely||Family history not likely unless primary lymphedema||Family history likely||Family history of lipedema likely||Very likely family history|
*Stemmer’s sign is the inability to pinch skin between the toes or fingers
**Lipedema in advanced stages is complicated by secondary lymphedema and will have a stemmer’s sign.
Stemmer’s Sign Test
Tests That Help Diagnose Lipedema
There is no single test to diagnoses lipedema. Tests, however, are important to rule out a related disorder.
|Standing Venous Doppler Ultrasound|
Venous Doppler Ultrasound:
As explained above a venous Doppler ultrasound is a very useful test for the diagnosis of Lipedema. Lipedema has many similarities to venous insufficiency as explained above. A standing venous Doppler ultrasound can readily detect venous insufficiency if done properly. Most hospital vascular labs do venous Doppler ultrasounds with the patient lying down. This is the usual way to find Deep Vein Thrombosis (DVT) but it is definitely not a good way to detect venous insufficiency. A standing venous Doppler ultrasound is a painless, non-invasive, and not very expensive test that provides a lot of useful information about venous circulation.
The Doppler ultrasound not only helps determine the presence of underlying venous problems, but it also gives critical information for managing lipedema. If venous insufficiency is present, it is important that treatment is given as the resulting increased venous pressures can greatly aggravate lipedema.
This is generally only used in complicated cases, where clinical diagnosis is not clear. This is a nuclear scan that assesses the lymph system, can be normal in lipedema or the test will show characteristic corkscrew changes with lymphedema. In most cases of lymphedema, lymphoscintigraphy shows delayed uptake. So lymphoscintigraphy can be very helpful to determine if and how significant a role lymphedema is playing in an individual’s symptoms, especially when there are more swelling problems.
Lipedema Lymphoscintograph 2-D Ultrasound of the Subcutaneous Fat: A fast quick and easy test just placing an ultrasound probe on top of the skin will show the fat below the skin and can provide lots of useful information. Lipedema fat is more fibrous and is often swollen. This can give a good deal of information and is a quick non-invasive test
During the height of the COVID pandemic, there were lockdowns and prohibitions on in-person medical visits. That necessitated a virtual visit via Zoom and Skype. During that time some doctors cut corners and made lipedema diagnoses without an exam. That time has largely passed.
Lipedema diagnosis is completely reliant on a physical examination.The physical signs and characteristics that are instrumental to diagnosing lipedema (such as lobules, ankle and wrist cuffs, subcutaneous nodules negative stemmers signs) can only be properly identified with a physical exam. More importantly, lymphedema and obesity can cause lobules and cuffs which can mimic lipedema. So without an exam to properly assign the signs to the correct disease or condition you, run the risk of diagnostic error or improper diagnosis.
NOTE: Lipedema is pronounced and spelled in several different ways. It is pronounced as lip-edema and lipo-edema – it can also be spelled lipedema, lipoedema, lipodema and Lipödem. The spellings are all correct but one or two spellings are predominantly used in different countries.