Sandy is a 58-year-old woman referred to your clinic with a diagnosis of lymphedema of the lower extremities. She first noticed this problem 25 years ago after the birth of her first child. During the examination, the physical therapist noted that she has pain to palpation in both lower extremities, which were equally enlarged. There is no edema in the feet and the patient has a negative Stemmer sign, which should be positive in the later stages of lymphedema. The PT questions the original diagnosis and contacts the referring physician to discuss the case and the initial PT examination findings in more detail.

What Is Lipedema?

Lipedema is an often overlooked disease that primarily affects women. It is characterized by a gradual and chronic progressive symmetrical accumulation of fat in the subcutaneous tissue, typically seen in the buttocks and lower extremities.¹ Lipedema is often confused with lymphedema, which is a mechanical failure of the lymphatic system resulting in the abnormal accumulation of water and proteins mainly in the subcutaneous tissues.² Very little information is available concerning the incidence of lipedema. One study performed in 2001 in Germany determined that 11% of females had lipedema, or what non-professionals term "cellulite".³ While no comparable study on lipedema has been done in the United States, if the percentage of affected women in the U.S. is even close to that of Germany, then at a minimum many thousands of American women may have lipedema.

Causes and Clinical Manifestations of Lipedema

Lipedema, described in 1940 by two U.S. physicians, was said to affect only females.⁴ They discussed how lipedema was different than obesity or lymphedema and was not associated with a history of acute cellulitis. Lipedema progresses gradually and often appears at puberty, pregnancy or menopause.³ Other authors suggest an increased incidence after pregnancy.¹ Lipedema is extremely rare in men and if present, involves severe hormonal functional disorders, such as testosterone deficiency or liver dysfunction.³ A genetic component may be involved with lipedema since it often affects more than one woman in a given family.¹

While lipedema may be challenging for the therapist to identify in its early stages, patients with lipedema should exhibit some characteristic signs. The first stage of lipedema seems to involve the disruption of the small blood vessels (microangiopathy) of the adipose tissue, which results in increased permeability to proteins and increased fragility of the blood capillaries.³ Thus the physical therapist may observe blue spots, or hematomas in the subcutaneous adipose tissue during the PT examination. These hematomas can be caused by minor trauma to the fragile capillaries. The PT may also note a localized protein-rich edema around the adipose tissue, which is due to increased capillary permeability.³

Lipedema also affects the skin by reducing the skin’s elasticity, the ability of the skin to return to its original size and shape.³ There is also an increase in skin compliance, a measure of how much the skin alters its size and shape in response to application of pressure. These skin changes, coupled with increased blood capillary permeability, lead to a very high interstitial pressure that reduces the effectiveness of the lymphatic system and increases the protein-rich edema.³

Another key characteristic to note during the PT examination is that areas affected by lipedema are typically sensitive to pressure and touch, and tend to bruise easily.^1,4 As a result of the increased capillary permeability, orthostatic edema often develops in the shins and ankles. The edema is more pronounced later in the day or in warm weather. It usually resolves with elevation early in the course of the disease process, but not in later stages as the disease progresses.^1,4 In addition to pressure sensitivity, patients often complain of aching and pain in the affected limbs.⁵

Although the upper extremities can be involved, the most common presentation of lipedema is in the lower extremities.³ Two different forms of lipedema occur: a column-shaped, cylindrical form in which the leg contour is even, and a lobar form in which the leg contour is uneven and forms creases between lobules.³ Each of these forms can present either in a descending or ascending manner in the lower extremities.³ Unlike lymphedema, lipedema does not extend into the feet or hands. In fact, with lipedema, a sharp demarcation at the ankle occurs between the normal and abnormal tissue in a pattern similar to wearing "riding breeches" or "Turkish pants."^1,5

Perhaps the most prominent characteristic of lipedema for the PT to note is that even though the legs are enlarged, the feet appear normal. Since the feet are of normal size in lipedema, these patients (as well as normal healthy people) have a negative Stemmer sign. A negative Stemmer sign is when a normal skin fold thickness is present at the dorsal base of the second toe or finger that can be easily lifted away from the bone. In contrast, patients with advanced stages of lymphedema have a positive Stemmer sign, where one cannot lift the skin or only lift it with difficulty away from the bone due to the abnormal thickness of the tissue.^3,6

Classic lipedema (not combination forms of lipedema and lymphedema that will be discussed later), presents in a symmetrical and bilateral manner. This distribution of enlarged extremities is primarily due to the location of the pathological fat deposits and not to a deficiency of the lymphatic system. What is easily noticeable in these patients is the striking difference in size between the abnormally large lower extremities and the normally sized upper extremities.³

Lipedema is not the same as being overweight or obese. Even though a large number of patients with lipedema are overweight, not all are. Unfortunately, diet and exercise are not effective in reducing the size of the lipedematous extremities (though they can reduce the unaffected extremities’ size), and these women may become frustrated because no matter what they do, their affected extremities remain large and unattractive.^1,3,4,7 Since women with lipedema often do not understand the cause of their disproportionately large legs or know where to get proper lipedema treatment, they often experience significant emotional distress.^3,4

Diagnosis of Lipedema

Lipedema can be diagnosed based on clinical criteria. As already mentioned, the main characteristics of lipedema are the presence of disease almost exclusively in women, the time of onset (often occurs during puberty, pregnancy, or menopause), the long-term course of symptoms (often decades), the symmetrical distribution of fat in the buttocks and lower extremities that does not involve the feet, orthostatic edema, sensitivity to pressure, and easy bruising.¹ Additionally, lipedema can be graded according to a three-stage scale: stage I – skin surface normal with a small nodular fatty tissue structure; stage II – skin surface uneven (peau d’orange) with a big nodular fatty tissue structure; and stage III – lobular deformation due to increased fatty tissue.¹

Obesity is often confused with lipedema. However, with obesity, a soft accumulation of fatty tissue occurs either in the trunk or over the entire body in normal proportions.¹ The fatty tissue is not painful, doesn’t bruise easily, and is present in both men and women.⁴

Lipohypertrophy is another condition that is similar, but not identical to lipedema. Lipohypertrophy can be found in both sexes. In women it presents as symmetrical fat deposits in the hips and legs. The trunk and arms typically remain normal size and there is no orthostatic edema, pain, or hypersensitivity to touch.¹ Lipohypertrophy often develops later in life.⁸ It should be noted that the exact relationship between lipohypertrophy and lipedema is unclear and that lipohypertrophy may later develop into lipedema.¹

Differential Diagnosis of Lipedema and Lymphedema

Perhaps the biggest challenge in correctly diagnosing lipedema is to properly distinguish lipedema from lymphedema (see Table 1). Lipedema is commonly misdiagnosed as lymphedema.⁹ Lymphedema is a protein-rich edema that results from a mechanical failure of the lymphatic system;² its major characteristics include nonpitting edema, a positive Stemmer sign in the feet or hands, and occurrence in both sexes. The edema, unlike lipedema, is not painful.

Table 1: Major characteristics distinguishing lipedema from lymphedema
Clinical Feature	Lipedema	Lymphedema
Gender	Almost exclusively in women	Women > men
Family history	Common	Rare
Distribution	Bilateral lower extremities, symmetric involvement	Unilateral, or bilateral with one leg affected more severely (asymmetric)
Cellulitis	Rare	Common
Pain on pressure	Present	Absent
Easy bruising of affected area (hematoma)	Present	Absent
Distal edema in the foot	Absent	Present
Stemmer sign	Absent (negative)	Present (positive)

 

Lymphedema may be classified as either primary or secondary. Primary lymphedema is considered to be a congenital or hereditary abnormality or dysfunction of the lymphatic system. One way of classifying primary lymphedema is by the age at which it first presents. Lymphedema can occur at birth (congenital), between the ages of 1 and 35 (lymphedema praecox), or after age 35 (lymphedema tarda).² Lymphedema praecox is the most common form, and in females, lymphedema praecox often occurs around menarche.¹⁰

Another way to classify lymphedema is by the nature of the abnormal lymphatic system (dysplasia).² Hypoplasia is the most common form of primary lymphatic dysplasia and describes a lymphatic system that has not developed completely. The lymph vessels, for example, can be smaller than normal. Hyperplasia is another form of dysplasia in which the lymph vessels are larger than normal size, and are not able to pump efficiently and give proper direction to the lymph flow. Finally, aplasia is the absence of lymph vessels or lymph nodes, which may also lead to primary lymphedema.²

Secondary lymphedema results from a known cause, such as surgery, radiation, trauma, infection, malignant tumors, and chronic venous insufficiency.² Malignant tumors, surgery, and radiation are the most common causes of secondary lymphedema and often involve disrupting the lymph flow through the lymph vessels and lymph nodes. If the lymphatic pathways are disrupted, then lymphedema can result if the remaining undamaged lymphatic pathways are not able to handle the extra demands placed on them.²

Lymphedema, like lipedema, can be graded using a three-point scale.² In stage 1, the edema is reversible with overnight elevation, and the skin may still be soft and pliable. The Stemmer sign at this stage is negative. It is difficult to differentiate lymphedema from other types of edema at this stage. Stage 2 lymphedema has the classic signs of a positive Stemmer sign and nonpitting edema that persist even when the extremities are elevated. The lymphedema is very noticeable at this stage. Additionally, the skin and underlying tissue become fibrotic and thick, and infections (cellulitis) are common due to the decreased health of the tissues. In stage 3, also known as elephantiasis, the classic signs of lymphedema are more pronounced and large skin folds may be present. The amount of lymphedema is greater, and skin alterations, such as papillomas, cysts, mycosis, and ulcers, are often found.²

One of the biggest clues to differentiating lymphedema from lipedema is that in lymphedema the edema is typically asymmetric. Even in primary lymphedema, where both extremities are involved, the PT should obtain a detailed patient history to determine if one limb developed lymphedema first and should also record if one of the extremities is presently larger than the other. In many instances, patients with lymphedema have only one affected limb, while the other remains normal. Secondary lymphedema only affects the extremity or extremities where damage has been done to the lymphatic system, such as lymph node removal or trauma. Only the affected side will be at risk of developing lymphedema.²

While the clinical presentation can usually determine the diagnosis of lymphedema and lipedema, if needed, diagnostic imaging methods can also be used to diagnose both lymphedema and lipedema.^2,11 Lymphoscintigraphy (lymphoscintigraphic function test) is a common method of diagnosis for lymphedema. A radioactive tracer is injected into the dorsum of the hand or foot, and the patient then exercises the involved extremity or extremities.

For example, if the legs are being examined, the patient may walk on a treadmill for 60 minutes. A gamma camera measures the time it takes for the radioactive tracer to be transported from the injection site to the regional lymph nodes, along with the exact pathway the tracer takes through the lymph vessels and lymph nodes.¹¹ Computed tomography (CT) provides cross-sectional views of the body; the resolution is not sufficient for diagnostic evaluation of the lymphatic system, but is successful in distinguishing between lymphedema and lipedema.¹¹ Ultrasound is not practical to assess lymph vessels, but is an excellent choice for diagnosing certain lymph node disorders.¹¹ Magnetic resonance imaging (MRI) is not typically used to diagnose lymph vessel abnormalities due to its high cost. However, when MRI is used, it can help the physician distinguish between lymphedema and lipo-lymphedema.¹² New methods using MRI and nanoparticles do hold much promise in helping to determine lymph node abnormalities and metastases.¹¹

Combination Forms of Lipedema and Lymphedema

Untreated lipedema can also cause the formation of lymphedema in what is known as lipo-lymphedema. It is believed that the abnormal subcutaneous fatty tissue distribution that occurs in lipedema compresses the lymph collectors, thus leading to impaired drainage of the lymphatic system.² This combination form of lipedema and lymphedema is frequently seen in lymphedema clinics. One study reports that lipo-lymphedema occurs on average 17 years after the initial onset of lipedema in patients who do not receive proper treatment for their lipedema.³ It is critical that the specially trained physical therapists recognize this form of edema to help develop realistic treatment plans and goals and to provide proper patient education to prevent the development of this condition.

Treatment of Lipedema—Conservative Methods

Physical therapists can treat patients with lymphedema and lipedema with manual lymph drainage (MLD) along with the other components of complete decongestive therapy (CDT). A number of lymphedema schools offer certified lymphedema therapist (CLT) training programs to healthcare professionals, such as physical therapists and physical therapist assistants. The skills taught in these programs are invaluable tools for treating patients with lymphedema and/or lipedema. The National Lymphedema Network (NLN) is a nonprofit organization that is an excellent source for patients with these conditions as well as for healthcare professionals interested in learning more about these diseases and about CLT training programs.¹³

CDT consists of MLD, compression therapy, decongestive exercises, and skin care. MLD is a gentle manual treatment technique that uses certain strokes in specific patterns on the body based on the location of lymph vessels and lymph nodes. Even though MLD is gentle and is well-received by almost all patients, there are contraindications to MLD such as cardiac edema, renal failure, and malignancies, which the CLT learns during training.²

Emil Vodder was a massage therapist and PhD who lived in Europe in the 20th century and is considered a pioneer in the field of lymphology. He developed the four basic strokes commonly used in MLD today: the stationary circle, pump, rotary, and scoop.² MLD is different from traditional massage techniques in that MLD involves less pressure than the typical massage strokes and also has a directional component. Additionally, the MLD strokes consist of two phases, working and resting, which help move lymph in the desired direction. Not only is MLD very comfortable to the patient, but MLD has been shown to increase the uptake of fluids into the lymphatic system as well as increase the volume of lymph that the lymphatic system can transport.²

Compression therapy is a key component of CDT, and the success of CDT is often determined by how conscientious the patient is with the compression phase of treatment. Since there is no cure for lymphedema or lipedema, it is critical that the patient participates in the compression therapy on a consistent, lifelong basis. In order to have a successful outcome, the patient must wear some type of compression almost constantly.

A frank discussion during the initial patient examination outlining the nature and benefits of CDT and, in particular, the time and cost commitment involved with compression therapy, will greatly help to increase patient compliance and decrease frustration. The therapist must also inform the patient that compression garments lose their elasticity over time and need to be replaced after about 6 months. The patient should also be advised to purchase two compression garments at a time so that one can be worn, while the other is washed and dried. Unfortunately, the ongoing lifetime cost of garment procurement is a barrier for effective treatment to many patients because many health insurance plans do not pay for garments. In the initial phase of CDT, the patient will undergo intensive MLD for weeks to months depending on the particulars of the case. At this stage of CDT, compression therapy consists of using short-stretch bandages that are applied over special padding. Short-stretch bandages differ from long-stretch (Ace) bandages in that their elasticity is woven into the bandage material itself, which can be called textile-elastic. Short-stretch bandages can only stretch about 60% more than their original length.²

In contrast, long-stretch bandages contain polyurethane fibers that allow this type of bandage to stretch more than double its original length. Short-stretch bandages are much more effective in providing resistance when the muscles contract (“working pressure”); however, at rest they have a relatively lower “resting pressure.” Short-stretch bandages form a strong support when muscles contract, but if properly applied, they avoid the “tourniquet effect” at rest that can lead to unwanted compression of the lymphatic vessels.

The opposite is true for long-stretch bandages, which have a high resting pressure and a low working pressure. Short-stretch bandages are typically used to provide compression for lymphedema, lipedema, and lipo-lymphedema. They are applied so that a pressure gradient is formed from the higher pressure distal end to the lower pressure proximal end of the extremity.²

Once the patient’s symptoms have improved and the reduction in edema has reached a plateau, the patient enters the second and final phase of CDT. During this phase the frequency of MLD will be reduced or discontinued and compression therapy will transition to using a combination of compression garments and short-stretch bandages. The typical pattern is that the patient will wear the compression garment during the day, and then wrap the affected extremity or extremities with short-stretch bandages at night. Compression garments come in a variety of types and only specially trained healthcare professionals should measure and select the appropriate compression garment.

Garments are designed to exert higher pressures on the distal extremity parts than on the proximal parts. Compression garment variations may include the area of the body to cover, size, style, color, knitting type (circular or flat), and compression level. The garment can be custom-made or standard. Recently, in addition to the traditional short-stretch bandages and compression garments, a number of adjustable-compression devices that provide gradient compression by using non-elastic adjustable bands are now on the market.²

While the basic treatment for lipedema is similar to that for lymphedema, important differences do exist in the anticipated outcomes. Depending on the type and severity of lymphedema, the CLT would expect a patient who has pure primary lymphedema to respond to CDT faster than one who has a combination of lipo-lymphedema or pure lipedema. Some patients with lipedema, the slowest to benefit from treatment, do not respond at all to the conservative methods of CDT.² Since patients with lipedema or lipo-lymphedema usually have pain and hypersensitivity in the lipedematous limbs, the CLT will likely need to use lighter pressures during MLD, and either defer or reduce bandaging pressures with increased padding during the initial treatments until the hypersensitivity resolves, as it usually does.²

Treatment of Lipedema—Surgical Methods

Considerable debate exists about the wisdom of removing the fat deposits in patients with lipedema using surgical methods, such as liposuction. A number of authors have indicated the potential risks of liposuction, which can damage the lymphatic system, resulting in lymphedema postsurgically.^2,3 In the past, liposuction was performed under general anesthesia, using a “dry” technique, meaning that the subcutaneous tissue was not infiltrated with fluid. Additionally, large, sharp cannulas were used to remove the fat deposits, which could cause further damage to the lymphatic system.¹⁴

Recently clinicians have developed tumescent liposuction to reduce the damage to surrounding tissue, including the lymphatic system.^1,14 Tumescent refers to abnormal distention, especially by fluids.¹⁵ This newer type of liposuction differs from traditional liposuction in that it is performed using local anesthesia, is a “wet” technique using a combination of lidocaine and prilocaine to infiltrate and swell up subcutaneous tissue, and uses a blunt microcannula. These refinements allow the surgeon to remove the abnormal fat deposits from the patient with lipedema in a manner that minimizes damage to the lymphatic system. However, even those advocating this newer liposuction technique maintain that the conservative treatment techniques of MLD and CDT are still necessary postsurgery, though at reduced frequency compared to before surgery.^1,14

Lipedema Education of the Public, Patients, and Healthcare Providers

Compared to lymphedema, much less has been written about lipedema in lymphology textbooks targeting healthcare providers, though the major lymphedema textbooks in English (some translated from the original German) do have sections on lipedema.^1-3 There is relatively more information concerning lipedema available to German-speaking healthcare providers, but not all of this information is always considered accurate.¹⁶ This confusion also exists in the U.S., as one study found that 9 of the 250 patients with a physician diagnosis of “lymphedema” actually had lipedema.¹⁷

Even though lipedema is a little-known disease, it is likely to be widespread. One study conducted in Germany found that 11% of German women have some form of lipedema, which would imply that at a minimum, hundreds of thousands of American women may have it.³ If lipedema is not treated properly, it can develop into the combination form lipo-lymphedema after an average of 17 years.³ Thus proper lipedema management and education to the public is of critical concern to prevent progression of lipedema to lipo-lymphedema.

The nonprofit organization National Lymphedema Network, the lymphedema training schools, and the thousands of CLTs credentialed by these schools collectively have helped to raise awareness of lymphedema and lipedema.¹³ While lipedema management is a relatively small part of the emphasis of lymphedema certification training, the CLT is qualified to educate and treat both those patients with pure lipedema and with the combination form, lipo-lymphedema. Clearly, in spite of recent advances, much work needs to be done since lipedema has been often overlooked and misdiagnosed.⁹

Proper education of the public, patients, and healthcare providers such as that provided by the NLN is crucial to the effective treatment of lipedema. There is much CLTs can do to educate physician and patient groups about the benefits of treating lipedema with the conservative methods of CDT and MLD and about the ineffectiveness of diet and exercise.^1,3,4,7 The recent introduction of tumescent liposuction holds much promise as physicians trained in this technique and CLTs work together to provide American women with the best combination of surgical and conservative treatments to help minimize the effects of this little-known but widespread disease.