Lipedema: Abnormal female fat that just won’t go away Overview with treatment suggestions
By Karen Herbst and Natalie MunguiaIntroduction
Millions of women have a type of excess fat that feels like small pebbles on their arms, lower abdomen, hips, buttocks and legs (gynoid area) that is painful, and resists extreme nutritional (anorexia, weight loss surgery) and physical fat loss attempts. Located under the skin, normal subcutaneous adipose (fat) tissue or SAT and its underlying fascia and freely moving connective tissue fibers is not painful and it is what gives our bodies shape and form. Lipedema SAT, on the other hand has thickened and poorly mobile connective fibers forming a netlike structure under the skin, and applying pressure causes an aching, stabbing, bruise-like feeling. About 50% of women with lipedema have hypermobile skin, joints and blood vessels throughout the body; these vessels are easily damaged and can bleed into the tissue forming a bruise about 75% of the time.1 Lymphatics function well initially, but cannot sustain fluid flow out of SAT causing aneurysms to form in the lymphatics and they leak; the definition of lipedema is “fluid in fat.”
Lipedema begins around puberty but can develop or worsen after childbirth or at menopause - times of hormonal change.
Lipedema is often mistaken for obesity,
TABLE 1
TABLE 1
Conditions that may be confused with lipedema
Condition Similarity to lipedema
Obesity Excess gynoid SAT
Difference from lipedema
• Excess abdominal SAT
• Metabolic syndrome or diabetes
Madelung’s disease Excess SAT arms/back/neck Legs not affected
Familial multiple Masses in the SAT Lipomas dominate on lipomatosis abdomen, flanks, arms and legs
Dercum’s disease Painful gynoid SAT; more women Fibromyalgia-type signs and than men symptoms; more lipomas
Lipodystrophy Gynoid SAT can be in excess Distinct loss of SAT
Fibromyalgia Pain in tissues
SAT = subcutaneous adipose tissue
lymphedema and other SAT disorders (Table 1). Confusing the matter is that lymphedema can develop as a comorbidity of lipedema, requiring not only complete decongestive therapy (CDT), but also deeper treatments to reduce the lipedema structure that inhibits flow through tissue. The SAT fluid - located in the interstitium, the area between and around cells, triggers adipocytes to grow. Lipedema SAT can grow to great extents damaging
Karen L. Herbst PhD, MD is a board-certified endocrinologist and Associate Professor at the University of Arizona (UA). She is the Director of the Treatment, Research, Education, Adipose Tissue (TREAT) program at the UA funded by the Lipedema Foundation.
Natalie Munguia is an undergraduate student at the University of Arizona (UA). She is currently participating in the UA’s graduate college summer program UROCMHD (Undergraduate Research Opportunities Consortium-Minority Health Disparities).
Gel-like masses in muscle/fascia
blood, lymphatic vessels, skin and muscle, potentially resulting in significant disability. The pebbly feel to lipedema SAT comes from nodules the size of a grain of sand, grain of rice or a frozen pea that can feel soft or firm. For quick assessment, nodules are easily found around the cubit nodes (inside elbow) and the medial knee, areas near lymph nodes;1 nodules are more prevalent the more extensive the lipedema SAT.1 The nodules in the tissue can be found by placing the fingertips into the lipedema SAT then gently rolling them; lipedema SAT cannot be found by simply looking for the disproportion between the lower and upper body. The arm should be raised and the area next to the armpit squeezed to reveal congestion and pain in the SAT. The hands and feet are affected in about 22% of women, a sign of more extensive disease; the greater extent,
the higher chance of numbness, blood clots and shortness of breath.1 When the hands are affected, the head should be palpated to assess for lipedema SAT.2
Types and stages of lipedema
Type refers to the location of lipedema SAT on the body (Figure 1). Stage refers to the appearance of lipedema SAT and skin; the stages of lipedema are not necessarily progressive. In Stage 1 painful nodular SAT expands under smooth skin in the gynoid distribution, and arms in 80%. The SAT can balloon over the ankle and wrist forming a “cuff” (Figure 1). Lymphedema may develop in Stage 1 but its occurrence is more prominent in Stages 2 and 3.1 In Stage 2, the skin’s appearance is that of a mattress, due to contraction of SAT fibers and fascia pulling down the skin and changing the structure of the lipedema SAT. The SAT nodules can coalesce to form larger masses, similar to a lipoma, often found on the anterior ankle. In Stage 3, the skin can separate from the
underlying SAT forming “bubbles” in the skin most notable on the thigh. Lipedema SAT stretches and folds into lobules slowing blood flow into and lymphatic flow out of SAT (Table 2). Walking becomes difficult due to heavy legs, pain, and SAT lobules. The SAT on the inner legs pushes the lower legs apart causing a knock-knee stance (genu valgum). Fibrosis can develop in the SAT on the upper buttocks, hips and along the groin area and should be treated by deep therapy. Women can rapidly progress to Stage 3 lipedema especially if they have polycystic ovarian syndrome, obesity, lymphedema, or hypermobility such as Ehlers Danlos Syndrome HypermobilityType.1
Lipedema SAT structure
Lipedema SAT has enlarged and/or contracted fibers. A decrease in blood flow causes the skin to feel cold.5 The adipocytes grow to huge sizes (hypertrophic) and break open, becoming surrounded by macrophages that eat the fat inside the dead cells;6 growth of new fat cells distorts the tissue further. Fluid is found in the tissue and appears to be trapped or held between cells (Table 2).
TABLE 2
Lipedema SAT Structure 6,37,38
Component Issue
Large adipocytes Hypertrophic (large); dead or dying
Small adipocytes Replicating producing excess SAT
Capillaries Leaky; thickened walls; dilated
Venules Thickened walls; dilated
Lymphatics Thick walls; hyperfunction early, leak later
Interstitium Edematous; fibrous
Immune cells Macrophages; lymphocytes
Type III
Complications: Lipedema can affect mobility, reduce quality of life and affects many areas of the body such as the musculoskeletal, vascular, and soft tissues systems (Table 3).
Standard treatment of lipedema
• Type I: Present in the hip and buttocks region; ankle cuff is not present.
• Type II: SAT is found in hips down to knees; ankles cuff is not present.
• Type III: Hips, buttocks thighs and lower leg down to ankles; cuff is usually present.
• Type IV: Arms are affected either only the a) upper arm, b) only the lower arm or c) both and a cuff is often present.
• Type V: Knees to ankles are affected.
• The arrowhead points to nodular lipedema tissue that is usually present in the area below the umbilicus.
Treatment of lipedema requires a multipronged approach including manual lymphatic drainage (MLD) as part of CDT especially when lymphedema is present, but also requires deeper therapies to break down lipedema SAT structure such as myofascial release, Astym® or Quadrivas Therapy®. The lack of deeper treatments of the lipedema SAT may explain why half of women with lipedema did not respond to MLD in a German study.7 When affected, areas to be treated include the scalp,2 neck, all four limbs, trunk, back and abdomen, but also mons, labia and vagina by pelvic MLD if needed.
n Compression garments keep fluid in SAT to a minimum, reduce pain, and protect when skin elasticity is reduced. If lymphedema is not present in lipedema, the strength of the compression garment can be light, from 8-15 mmHg, though women
commonly wear 20-30 mmHg for additional support. Women with very painful lipedema may not tolerate high compression levels. High waisted leggings are preferred to prevent fluid moving up from the legs and accumulating in the abdomen. The ankle cuff may require special compression to prevent damage in this area. Women with lipedema SAT in their hands should be offered gauntlets to reduce hand swelling. Whole body compression can be difficult, especially in warm seasons. Thinner or net-like breathable fabrics, kinesiotaping or sleeping in compression at night should be considered during these months.
n Sequential pneumatic compression pumps (pumps) reduce fluid and pain in lipedema SAT and empower women to improve self-care at home. Pump garments encircle the abdomen and pelvis when the legs are treated, and chest with arms to reduce SAT growth in these areas.8
Wounds improved and capillaries were less fragile after pump therapy for women with lipedema.9 Basic pumps for prevention of deep venous thrombosis where leg garments rise only to the groin are risky for lipedema; if used, pressures must be kept low to prevent lymphatic vessel damage, and a bike short-type compression must be worn to prevent growth of abdominal SAT.
Lifestyle treatment
Daily exercise and healthy food are standard of care for lipedema despite the fact that lipedema SAT does not shrink in response to lifestyle changes.
n Exercise: Movement of any kind, from chair exercises, to swimming or whole body vibration, improves lymphatic and venous pumping by the action of muscle contraction.10
n Healthy eating: Healthy eating is recommended for lipedema treatment, despite a lack of published diet studies in this population. In our practice, we have observed that some women have maintained a low level of lipedema SAT by following plant-based diets and daily movement. Food choices should provide optimal nutrition while lowering generation of inflammation. Patients who eat rainbow colored fruits and vegetables, fats from nuts, beans, avocados, olives, seeds and who lower their consumption of processed foods and carbohydrates, meats and refined grains11 have less pain and more energy.11 Women with lipedema who eat an Atkins type diet also have success with weight loss and pain reduction.
TABLE 3
TABLE 3
Complications and co-morbidities of lipedema
Complications and co-morbidities of lipedema
Musculoskeletal Vascular Soft Tissue Other Gait disturbance Lymphedema Obesity Pain
Change in posture; Easy bruising Decreased skin elasticity Psychological lordosis Thinning of the skin distress/anxiety
Arthritis (knees) Edema Lipomas Shortness of breath
Hypermobile tissue Varicose veins Cellulite Slow metabolic rate
Weakness/ Sluggish lymphatics; Cysts Cellulitis muscle atrophy leaky lymphatics
Non-standard treatment of lipedema Supplements and medications are used in clinical practice and by patients to reduce limb heaviness, pain, and fibrosis. n Lymphagogues: Medications such as amphetamines increase norepinephrine and other nerve transmitters in the sympathetic nervous system (SNS) that bind and activate adrenergic receptors augmenting pumping of lymphatic vessels.12 Phentermine is a medication with similar pharmacologic activity to amphetamine, and is useful in reducing SAT. Plant derivatives such as citrus peelderived diosmin,13 Butcher’s broom (Ruscus
aculeatus) and quercetin14 also stimulate the SNS pathway15 increasing lymphatic pumping, as well as venous tone. 16
n Vascular system anti-inflammatories: Certain plant-derived compounds have anti-inflammatory properties that maintain the integrity of veins and lymphatics in women with lipedema including L-arinine, an amino acid that reduces lymphatic vessel leakage 17 , Pycnogenol (French maritime pine bark extract) that improves venous insufficiency and tone,18 and beta aescin from horse chestnut seeds and other seeds.19, 20 Anti-inflammatory products reduce pain and SAT fluid in lipedema, but there is need for formal testing.
n Selenium: A mineral that inhibits matrix metalloproteinases, enzymes important in lymphatic vessel remodeling and damage,21, 22 selenium is recommended for excess fluid in lymphedema and lipedema. The maximum safe daily intake for selenium is 600 mcg daily (US National Research Council)23, 24 or three Brazil nuts daily. Blood levels can be checked as high levels of selenium have been associated with diabetes.25
n Metformin: Women with lipedema who develop signs or symptoms of metabolic dysfunction should consider metformin treatment to improve inflammation,26 reduce weight, improve gut bacteria,27 and support longevity.28
side of the knee. Stage 2: The SAT is pebbly and tender but now the skin has indentations where the connective tissue has thickened and contracted pulling down the skin. The pad of fat below the knee has become very noticeable. Stage 3: The SAT feels pebbly, is tender but the fat has enlarged to such a great extent that it has completely covered the knee; the pad of fat below the knee has engulfed the entire lower leg and the surface of the skin has large divots, lobules and a bubble-like appearance in some areas.
n Whole Body Vibration (WBV): During WBV, vibrations and oscillations excite stretch receptors, tendon reflexes and muscle contraction. WBV improves blood circulation and increases lymphatic flow, lessening the risk for edema formation in the legs which may be important in lipedema.29
Signs and Symptoms of Lipedema
Signs
• Unexplained weight gain – often rapid
• Symmetric SAT deposits on left and right side of body
• Disproportion of SAT on lower > upper body
• Fat unaffected by diet or exercise
• Lower skin temperature (hypothermia) in some areas
• Pain and tenderness to touch over affected areas of SAT
• Varicose veins
• Blood clot especially in later stage
• Pitting edema especially Stage 4 but also heaviness (lymphedema) of other areas of the body: thighs, hips, buttocks, upper arms
Symptoms
• Muscle/joint aches
• Easy bruising
• Palpitations
• Flu-like symptoms
• Sleep deprivation (due to pain)
• End of day leg heaviness
• Stress, fatigue and anxiety
• Reduced cognitive/concentration
• Numbness (greater in Stage 3>2, 1)
• Shortness of breath (Stage 3>2, 1)
SAT = subcutaneous adipose tissue
These can vary per patient and can be affected by stage 1, 3, 4 and season (i.e.: worse during warmer weather.)
Imaging
n Lymphangioscintigraphy (LAS): Whole body LAS is first-line imaging for visualization of lymphatic vasculature and flow in lipedema. Lymph transport through lymphatics can be normal,30 but lipedema SAT causes lymphatics to become tortuous and they can leak. Clinical phenotyping and correlation with LAS is needed to understand the impact of lipedema on the lymphatic system.
n Dual Energy X-ray Absorpitometry (DEXA) scans: DEXA scans provide bone mineral density, but additional software allows assessment of fat and muscle of the whole body; in women with lipedema, higher amounts of fat are noted in the gynoid region.31
n Magnetic Resonance Imaging (MRI): MRI in Stage 3 lipedema demonstrates circumferential enlargement of fibrotic SAT lobules as well as microaneurysms in lymphatic vessels.32
n Indirect lymphography: Indirect lymphography demonstrates over-extensibility of lipedema SAT and normal lymph collectors.33
Surgical treatment of lipedema
Liposuction of lipedema SAT has been performed in Europe since the 1990s, resulting in significant improvement of quality of life. Indications for liposuction in lipedema include loss of mobility, reduced quality of life, joint damage, altered gait, chronic pain, and failure of conservative therapy to improvement signs and symptoms associated with lipedema. During power-
assisted liposuction, tumescing SAT with saline, analgesia (e.g., lidocaine) and buffer firms up the turgor of the tissue to allow tiny, rapid vibrations of the microcannulae to break up adipocytes, which are suctioned out of the tissue. Water jet assisted liposuction (WAL) uses strong sprays of saline and analgesia to knock off fat for suction into microcannulae.34 Laser assisted liposuction is reserved for fibrotic tissue. Liposuction can be performed on circumferential areas, or in sections. An average of 2-3 surgeries depending on the stage, are used to treat lipedema. Patients who are awake during liposuction can change their position to assist in the procedure. Some patients and physicians choose twilight sedation which can decrease spontaneous activity of the lymphatic vessels,35 or general anesthesia which slows lymphatic pumping and prolongs recovery.36 Some women note growth of SAT on the abdomen, breast, chest or arms after liposuction of lipedema SAT, which needs to be studied further.
Conclusion
Lipedema is a common SAT disorder in women causing pain, altered shape, impaired mobility and reduced quality of life. Medications and supplements can temper symptoms and liposuction can reduce the amount of SAT, but the long-term consequences of any of these treatments for women in Canada or the US are not known. Studies are needed for all aspects of lipedema care and to better understand this lifestyle resistant SAT disorder. LP
A full set of references can be found online at www.lymphedemapathways.ca
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