Lipedema

Not to be confused with dyslipidemia.

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The three stages of lipedema: Stage 1, Stage 2, and Stage 3 (left to right)

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Accumulation of lipedema fat tissue on legs of 28-year-old woman. The feet are not affected, the fat can form a cuff of tissue at the ankles.

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Lipedema fat tissue accumulates in the upper arms in a significant number of cases.

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Advanced stage lipolymphedema, primary lipedema with severe secondary lymphedema

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Pronounced lipedema of the right leg (the knee pointing to the right and is concealed by the overhanging lipedema). This is a very advanced case, possibly Stage 4 with lymphedema as well - lipolymphedema.

Lipedema or lipoedema is a chronic disorder of adipose tissue and lymphatic dysfunction generally affecting the legs, which causes the legs and sometimes the arms to accumulate distinct patterns of fatty tissue. It is distinguishable by six characteristics:

1. it can be inherited;
2. it occurs almost exclusively in women;
3. it can occur in women of all sizes, from the seriously underweight to the morbidly obese;
4. it involves the excess deposit and expansion of fat cells in an unusual and particular pattern – bilateral, symmetrical and usually from the waist to a distinct line just above the ankles;
5. unlike normal fat, lipedematous fat generally cannot be lost through diet and exercise;
6. the adipose tissue may present as painful, or, there may be pain when pressure is applied to the lipedematous areas;
7. a pathognomonic indicator of lipedema is that the feet are spared.

Lipedema usually is triggered at puberty, but can trigger or worsen during or after pregnancy, at peri-menopause, and following gynecological surgery, i.e., surgery of the uterus, ovaries, or fallopian tubes or any surgery with general anesthesia. Lipedema can also be triggered by an extremely stressful situation such as a death in the family or a divorce because cortisol levels increase, causing an inflammation cascade, almost always misdiagnosed as simple weight gain. If lipedema is diagnosed early, which currently is very rare, it is possible to prevent a significant expansion of lipedematous fat cells and to alert patients to their heightened risk factors so they can take appropriate action.

It is commonly misdiagnosed. Estimates of the incidence of lipedema vary widely, and range as high as 11% of the post-pubertal female population, with estimates of 17 million women in the US, and 370 million women worldwide affected.

Causes

The cause is unknown. There are likely genetically inherited risk factors as it is more likely to occur in the first and second degree relatives of affected individuals. It appears to be related to estrogen and progesterone hormonal influences as it occurs exclusively post puberty and largely in females. There is a strong inflammation component; some patients self-report an improvement of symptoms by moving to anti-inflammatory diets and/or eliminating foods that exacerbate inflammation.

Diagnosis

At this time, only Germany and The Netherlands have standardized ways of diagnosing lipedema. The US does not currently have a standardized diagnosis protocol, and therefore the diagnosis is typically made clinically via physical inspection (palpating the adipose tissue). Some trained clinicians and therapists can feel the physical differences in the adipose tissue, characterized as nodules with "beans in a bag" feeling. When the tissue has excess fluid the nodules are no longer easily palpable. Testing of the major components of the lymphatic system can be done through tools such as lymphoscintigraphy, but lack of noticeable lymphatic impairment does not indicate lipedema is not present, only that the major lymphatics are not (yet) affected.

Differential Diagnosis,

	Lipedema	Lipo-lymphedema	Lymphedema	Obesity	Venous Insufficiency/Venous Stasis
Symptoms:	Fat deposits / swelling in legs and/or arms NOT hands/feet	Fat deposits / swelling widespread in legs/arms/torso	Fat deposits / swelling in one limb including hands/feet	Fat deposits widespread	Swelling near ankles; brownish discoloration of lower legs (hemosiderin deposits). Minimal swelling possible.
Male/Female:	F	F	F/M	F/M	F/M
Onset:	Around hormonal shifts (puberty, pregnancy, menopause)	Around hormonal shifts	After surgery that affects lymphatic system, or at birth	Any age	Around onset of obesity, diabetes, pregnancy, hypertension
Effects of Diet:	Restricting calories ineffective	Restricting calories ineffective	Restricting calories ineffective	Diets and weight loss strategies may be effective	No relation to caloric intake
Presence of Edema:	Non-pitting edema	Lots of edema; some pitting; some fibrosis	Pitting edema	No edema	Often edema, but can also occur without edema in earlier stages
Presence of Stemmer Sign:	Stemmer's Sign negative	Stemmer's Sign positive	Stemmer's Sign positive	Stemmer's Sign negative	Stemmer's sign may or may not be present in Lymphedema/Lipolymphedema
Presence of Pain:	Pain in affected areas likely	Pain in affected areas	No pain initially	No pain	Pain is likely
Affected Population:	Best estimate is 11% adult women (study done in Germany)	Unknown; best estimate is a few percent of adult women	Low	>=30% of US adults	>30% of US adults
Presence of Cellulitis:	No history of cellulitis	Likely history of cellulitis	Possible history of cellulitis		Often itching +/- discoloration mistaken for cellulitis
Family History:	Family history likely	Family history of lipedema likely	Family history not likely unless primary lymphedema	Family history likely	Very likely family history

Lipedema/Lymphedema Differentiation

Lymphedema is usually asymmetrical and can be either acquired (through surgery, trauma or infection damaging the lymphatic system) or congenital (hereditary changes in the lymphatic system). However, symmetrical enlargement of both lower limbs, from waist to ankles (i.e. gynoid fat) is a hallmark of lipedema. As the swelling continues and spreads from lower extremities to other parts of the body, the swelling is likely caused by slower lymphatic flow and changes in the lymphatic vessel structure caused by pressure in the lipedematous limbs. This is known as lipo-lymphedema. Lipo-lymphedema may also develop in combination with chronic venous insufficiency and other vascular disorders.

Lipedema can be underdiagnosed due the difficulty in differentiating it from other edemas and obesity. Some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas can be found in the article "Edema: Diagnosis and Management." (3)

Lipedema / Dercum’s Disease Differentiation These conditions may co-exist. Dercum’s Disease is characterized by painful lipomas around the body. CITATION: http://fatdisorders.org/fat-disorders/diagram

Prevention

There is currently no known way to prevent lipedema. Some treatments, both conservative and surgical, may help some people postpone or prevent symptom worsening sometimes.

Complications

Complications include depression, anxiety and pain.

Signs and symptoms

Patients tend to gain weight in lipedematous areas and lose it in non-lipedematous areas, though there are cases where weight loss has resulted in improvement of the condition. Obese lipedema patients who undergo bariatric surgery lose fat primarily from the waist up. While lipedema presents itself in various ways, diagnosis is possible as early as pre-puberty when inner thigh pads present and at any age when fat gathers and drapes at knees. Symptoms of lipdemia include disproportionately large, column-like legs, legs unusually large to the knees, disproportinate hips, stomach or buttocks. As lipedema progresses, patients become increasingly heavy in the lower body. The additional, expanding fat cells interfere with the pathways of lymphatic vessels, and patients can develop secondary lymphedema, a condition known as lipo-lymphedema. Many lipedema patients cannot tolerate the compression garments associated with conventional lymphedema treatment because the underlying lipedematous fat is very painful, and those patients therefore are at risk for the side effects of uncontrolled lymphedema, including recurring blood infections and fibrosis. If not kept in check through a healthy lifestyle, lipo-lymphedema can worsen, and patients will become progressively less mobile.

Treatment

There is no consensus on a uniform treatment plan. Treatments range from mild lifestyle changes, to medications and supplements, to surgical.

Medical treatment is designed primarily to address the secondary lymphedema part of the lipedema patient's condition. This treatment includes a course of manual lymphatic drainage and bandaging by a lymphedema therapist, followed by the wearing of custom-fitted compression garments or devices — usually stockings, and sometimes biker shorts and/or arm compression. Compression prevents recurrence of lymphedema, and in some lipedema patients can reduce the pain of lipedematous fat. There is currently no known uniform medical procedure to cure lipedema. It is, however, successfully managed through a variety of consistently applied techniques to improve the health of the legs and prevent the condition from returning in more difficult to manage levels. Management involves reducing dietary sodium intake, frequent, gentle exercise to promote circulation in the legs, such as rebound exercise, and treatments typical for lymphedema treatment.

Conversely, a growing number of women claim to have succeeded in reversing lipedema through elimination diets, low impact exercise, and an herbal protocol that addresses inflammation of the legs rather than edema of the legs. This group claims to eliminate inflammatory pain in legs by

1. changing food choices;
2. and, modulating the tumor necrosis factor (TNF) antagonists or TNF blockers in the adipose tissue.

This movement is gaining recognition in European and Australian medical circles.

Though there is debate about the merits of surgical intervention, there are surgeons in USA, Germany, the Netherlands and the U.K. achieving success with lymphatic-sparing gentle water-jet-assisted liposuction. This is different from conventional liposuction, and is usually performed with tumescent local anesthesia; one of its merits is that WAL liposuction preserves the integrity of the lymphatic system while removing lipedematous adipose tissue.

It should be remembered that lipedema is a childhood disease that progresses throughout adulthood. Early diagnosis makes possible the best treatment.

Prognosis

There is no uniform prognosis across all patients. Some patients have success managing the symptoms with conservative and/or surgical treatments. Some patients progress to lipo-lymphedema, a condition where lipedema and lipo-lymphedema both occur. Some patients develop Dercum's Disease.

Epidemiology

The epidemiology is unknown although it is believed by knowledgeable clinicians to be quite common. "11% of adult women" is often cited but that is unsubstantiated at this time. The "11% of adult women" comes from a small, unpublished study done in Germany at the Foeldi Clinic in 2006. It has been reported that it occurs in multiple races including Caucasian, African American and people from India, but likely is less represented in non-Indiad Asian races. Additional research is needed.

Research

2 clinical trials have been done or are in progress. Clinical Trials

The TREAT Program (Treatment, Research and Education of Adipose Tissue Program) at the University of Arizona and its $1.5M inaugural funding commitment was announced in 2015. This program is the ultimate in integrative medicine providing patient care and research under one program. TREAT Program

Over 150 papers published on lipedema. Research Papers

Many books mention or discuss lipedema. Books

4 postdoctoral fellowships are being sponsored by the Fat Disorders Research Society and the Lipedema Foundation for research into lipedema and Dercum's Disease. Research Grants

Research Agenda

In April 17–18, 2015, The Lipedema Project convened the 1st International Symposium on Lipedema held in NYC. Events were co-sponsored by The Friedman Center for Lymphedema Research & Treatment at Mount Sinai Beth Israel Medical Center, Lipedema Simplified, LLC and Lymphatic Education & Research Network (LE&RN). CME's(Continuing Medical Education credits) were available for healthcare professionals. Keynote speakers included Prof Etelka Földi, MD, Mark L Smith, MD, FACS, Stanley Rockson, MD, Karen Herbst, MD, and many others. Additional to the Symposium there were two tracks, an invitation Think Tank for top clinicians, surgeons, researchers, scientists, therapist and lipedema advocates; and a Solutions Forum for patients, therapists and supporters. The focus was Setting the Research Agenda for Lipedema: STEPS TOWARDS A CURE. Video of several presentations is available at the Lymphatic Education & Research Network website - http://livestream.com/LymphaticRF/lipedema Online courses are being developed and will be available in the Fall 2015. The documentary film, The Disease They Call FAT, premiered at the Symposium and is now available live streaming, as a download, or on DVD http://DiseaseTheyCallFAT.tv

This is the first Symposium of its kind and spearheaded a collaboration between healthcare professionals and patients in order to move the research agenda forward. The Think Tank produced 5 groups moving forward in the following areas: 1. Tissue issues (research to understand the differences between normal fat & lipedema fat) 2. Diagnostic Criteria (defining diagnostic criteria) 3. Centers of Excellence (CoE - establishing benchmarks and protocols across centers) 4. Epidemiology & Genetics (creating an international Lipedema patient databank & studies to determine the genetic markers of hereditary lipedema) 5. Outcomes Research (focus on understanding the efficacy of different treatment modalities, i.e. conservative treatment, surgical intervention)

(http://lipedemaproject.org/symposium2015)

History

Although first identified in the United States, at the Mayo Clinic in 1940, lipedema is barely known in that country – to physicians or to the patients who have the disease. Lipedema often is confused with obesity, and a significant number of patients currently diagnosed as obese are believed to have lipedema, either instead of or in addition to obesity.

The word lipedema has multiple spellings. Lipedema is the American spelling, while lipoedema is used in Britain and Europe. Lipodema is also used occasionally.

In recent years growing awareness of lipedema has come from internet sources. Conferences in the Netherlands, Germany, England and the USA have convened, in part initiated by groups initially only organized through social media.

In 2008, the Fat Disorders Research Society, Inc. was formed to aid lipedema awareness and research, and in 2009 achieved US non-profit 501(c)(3) status. They held conferences in 2010, 2011, and 2014.

In 2012, Lipese, LLC published the first co-authored English lipedema handbook entitled: Lipedema: Help, Hope, and Healing written by Maggie McCarey, USA, and Tatjana van der Krabben, the Netherlands,

In November 2012 the UK's Health service the NHS first listed lipedema as the abnormal build-up of fat cells in the legs, thighs and buttocks. This national acceptance of lipedema is due largely to The Lymphoedema Support Network and the administrators and members of Lipoedema Ladies who are an important part of the grassroots movement. The NHS listed the only treatment that appears to be effective for lipedema is a procedure called tumescent liposuction which is now routinely performed in the Netherlands and Germany.

In June 2015, the Fat Disorders Research Society was granted member organization status to NORD, The National Organization for Rare Disorders, representing lipedema and related conditions such as Dercum's Disease.

See also

• Steatopygia
• Adiposis dolorosa
• Dercum's Disease
• List of Books Discussing Lipedema
• List of Papers Discussing Lipedema
• Educational Videos

Bibliography

1. Allen E, Hines EA Lipedema of the legs: a syndrome characteried by fat legs and orthostatic edema. Proc. Staff Meet. Mayo Clin. 1940; 15184-187.
2. Wold LE, Hines EA Allen EV. Lipedema of the legs: a syndrome characterized by fat legs and edema. Ann. Intern. Med. 1951; 34(5): 1243-1250. doi:10.7326/0003-4819-34-5-1243.
3. Cornley ME. Fatter Through Lipids or Water: Lipohyperpalsia Dolorosa Versus Lymphedema. Am. Journal of Cosmetic Surg. 2014; 31: 189-195.

External links

• Dr. Karen Herbst's page
• Fat Disorders Research Society's Lipedema Description
• Royal College of General Practioners' course on lipedema
• Lipoedema Australia
• Talk Lipoedema