Lipedema is a disorder where there is enlargement of both legs due to deposits of fat under the skin. Typically it gets worse over time, pain may be present, and people bruise easily. In severe cases the trunk and upper body may be involved.
The cause is unknown but is believed to involve genetics and hormonal factors. It often runs in families. Risk factors include being overweight or obese. Other conditions that may present similarly include obesity, lipohypertrophy, chronic venous insufficiency, and lymphedema.
A number of treatments may be useful including physiotherapy and exercise. While surgery can remove fat tissue it can also damage lymphatic vessels. Treatment does not typically result in complete resolution. It is estimated to affect up to 11% of women. Onset is typically during puberty, pregnancy, or menopause.
Video Lipedema
Signs and symptoms
People 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 lipedema include disproportionately large, column-like legs, legs unusually large to the knees, disproportionate 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.
As the disease process progresses, there is fibrosis within the fatty tissues. The condition becomes painful, movement is restricted and painful leading to orthopedic problems, and limited mobility. Pathological examination of the abnormal tissues reveals the development of fibrosis which complicates treatment and interferes with mobilization or removal of the abnormal tissues. The diagnosis of "lipedema" is misleading and leads to confusion with other conditions mentioned. A better diagnosis may be FIBROSING LIPODYSTROPHY. There are many documented cases in which the condition is familial, and present in women who are not overweight and progresses in spite of strict dietary management.
Maps Lipedema
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.
Lipedema is usually 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.
Diagnosis
Lipedema is commonly misdiagnosed. At this time, only Germany and the Netherlands have standardized ways of diagnosing lipedema. Other countries do 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
Lymphedema
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. Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.
Lipedema / Dercum's Disease Differentiation These conditions may co-exist. Dercum's Disease is characterized by painful lipomas around the body.
Prevention
There is no known way to prevent lipedema. Some treatments, both conservative and surgical, may help some people postpone or prevent symptom worsening sometimes. 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.
Treatment
A number of treatments may be useful including physiotherapy and exercise. While surgery can remove fat tissue it can also damage lymphatic vessels. Treatment does not typically result in complete resolution.
Physical therapy
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.
Surgery
The use of surgery to treat the condition is controversial. Options include liposuction and lipectomy.
The studies of highest quality involve tumescent or super tumescent anesthesia and vibrating liposuction, powered, cannula used with lymph sparing techniques. The treatment of lipedema with tumescent liposuction requires multiple procedures. In the United States Health Insurance do not generally pay for liposuction for lipedema, making it expensive. Liposuction under general anesthesia, without tumescent anesthesia, can be damaging and is not recommended for the 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 (adiposis dolorosa).
Complications
Complications include depression, anxiety, and pain.
Epidemiology
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. "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-Indian Asian races.
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, Australia and Europe. Lipodema is also used occasionally.
In recent years growing awareness of lipedema has come from internet sources. LASS ( Lipoedema Australia Support Society Inc) formed and set up a secret Facebook support page in 2012, from this original group, international members left to set up groups in UK, USA and Canada. Grass roots patient-led change has continued to push lipedema. Conferences in the Netherlands, Germany, England Australia 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 published the lipedema handbook entitled Lipedema: Help, Hope, and Healing written by Maggie McCarey and Tatjana van der Krabben.
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
- Lymphedema
- Steatopygia
- Adiposis dolorosa
- Lipodystrophy
References
External links
Source of the article : Wikipedia
0 Comments