Lipedema

Lipedema
Synonyms Lipoedema, Lipödem, Lipalgia, Adiposalgia/Adipoalgesia, Adiposis dolorosa, Lipomatosis dolorosa of the legs, Lipohypertrophy dolorosa, Painful column leg, Painful lipedema syndrome
Pronounced lipedema of the right leg (the knee is 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.
Classification and external resources
Specialty Vascular Medicine
ICD-10 R60, E88.2
OMIM 614103
DiseasesDB 7491
MeSH D065134
Orphanet 77243

Lipedema or lipoedema (European spelling) is an incurable, chronically progressive affliction that occurs almost exclusively in women. It causes a symmetrical accumulation of fat in the subcutaneous tissue that disproportionately affects the lower limbs from buttocks to ankles. The legs may also be sensitive and prone to easy bruising. In some cases, the upper arms can also accumulate distinct patterns of fatty tissue.[1][2]

Lipedema is little known, under recognized, and generally misdiagnosed as simple obesity.[1] Despite the fact that it has been named for over 75 years, it is only recently that interest and education about lipedema is gaining in awareness. Estimates of the incidence of lipedema vary widely, and range as high as 11% of the post-pubertal female population,[3] with estimates of 17 million women in the US, and 370 million women worldwide affected.

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.

Lymphatic vessels

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.

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 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.

Diagnosis

Lipedema is commonly misdiagnosed.[4] 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

[5][6][7][8][9][10]

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 often 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. Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.[9]

Lipedema / Dercum’s Disease Differentiation These conditions may co-exist. Dercum’s Disease is characterized by painful lipomas around the body.[11]

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. 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

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.

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 (WAL). 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.[12] The only proven method to address the characteristic disproportionate fat of lipedema is lymph sparing tumescent liposuction.[13] Most of literature on the effectiveness of these tumescent liposuction treatment are from England, Netherlands and Germany. The studies of highest quality involve tumescent or super tumescent anesthesia and vibrating liposuction, powered, cannula used with lymph sparing techniques.[14][15][7][16][17] Although liposuction is not curative it can improve mobility, improve functionality of daily living, reduce or relieve pain, improve quality of life. The longest follow up on series of patients after tumescent liposuction with vibrating cannulas and lymph sparing techniques is 15 years and no reported reaccumulation of disproportionate fat in the treated areas.[13] The treatment of lipedema with tumescent liposuction requires multiple procedures. In the United States Health Insurance, including medicare and medicaid do not generally pay for liposuction for lipedema, so multiple procedures can be expensive or cost prohibitive. Liposuction under general anesthesia, without tumescent anesthesia, can be damaging and is not recommended for the treatment.[18]

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.

Complications

Complications include depression, anxiety, and pain.[4]

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.[8] "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.[4]

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 lead 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

References

  1. 1 2 Schmeller, W.; Meier-Vollrath, I. (2008). "Chapter 7: Lipedema". In Weissleder, Horst; Schuchhardt, Christian. Lymphedema Diagnosis and Therapy (4th ed.). Viavital Verlag. pp. 294–323. ISBN 978-3-934371-38-5.
  2. ↑ "Lipedema | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 30 December 2016.
  3. ↑ Buck Dw, I. I.; Herbst, K. L. (2016). "Lipedema: A Relatively Common Disease with Extremely Common Misconceptions". Plastic and Reconstructive Surgery - Global Open. 4 (9): e1043. PMC 5055019 Freely accessible. PMID 27757353. doi:10.1097/GOX.0000000000001043.
  4. 1 2 3 Herbst, Karen L (2012). "Rare adipose disorders (RADs) masquerading as obesity". Acta Pharmacologica Sinica. 33 (2): 155–72. PMC 4010336 Freely accessible. PMID 22301856. doi:10.1038/aps.2011.153.
  5. ↑ Fat Disorders Research Society Lipedema Description
  6. ↑ Todd, Marie (2010). "Lipoedema: Presentation and management". British Journal of Community Nursing. 15 (4): S10–6. PMID 20559170. doi:10.12968/bjcn.2010.15.Sup3.47363.
  7. 1 2 Fife, Caroline E.; Maus, Erik A.; Carter, Marissa J. (2010). "Lipedema". Advances in Skin & Wound Care. 23 (2): 81–92. doi:10.1097/01.ASW.0000363503.92360.91.
  8. 1 2 Földi, Michael; Földi, Ethel, eds. (2006). "Lipedema". Földi's Textbook of Lymphology. Munich: Elsevier. pp. 417–27. ISBN 978-0-7234-3446-7.
  9. 1 2 Trayes, K. P.; Studdiford, J. S.; Pickle, S; Tully, A. S. (2013). "Edema: Diagnosis and management". American family physician. 88 (2): 102–10. PMID 23939641.
  10. ↑ Herbst, Karen L (2012). "Rare adipose disorders (RADs) masquerading as obesity". Acta Pharmacologica Sinica. 33 (2): 155–72. PMC 4010336 Freely accessible. PMID 22301856. doi:10.1038/aps.2011.153.
  11. ↑ http://fatdisorders.org/fat-disorders/diagram%5B%5D
  12. ↑ Stutz, J. J.; Krahl, D. (2008). "Water Jet-Assisted Liposuction for Patients with Lipoedema: Histologic and Immunohistologic Analysis of the Aspirates of 30 Lipoedema Patients". Aesthetic Plastic Surgery. 33 (2): 153–62. PMID 18663515. doi:10.1007/s00266-008-9214-y.
  13. 1 2 Rapprich, Stefan; Dingler, Anne; Podda, Maurizio (2011). "Liposuction is an effective treatment for lipedema-results of a study with 25 patients". JDDG. 9 (1): 33–40. PMID 21166777. doi:10.1111/j.1610-0387.2010.07504.x.
  14. ↑ Schmeller, W.; Hueppe, M.; Meier-Vollrath, I. (2012). "Tumescent liposuction in lipoedema yields good long-term results". British Journal of Dermatology. 166 (1): 161–8. PMID 21824127. doi:10.1111/j.1365-2133.2011.10566.x.
  15. ↑ Schmeller, W.; Meier-Vollrath, I. (2007). "Lipödem - Aktuelles zu einem weitgehend unbekannten Krankheitsbild" [Lipoedema - New Facts of a Fairly Unknown Disease]. Aktuelle Dermatologie (in German). 33 (7): 251–60. doi:10.1055/s-2007-966651.
  16. ↑ Langendoen, S.I.; Habbema, L.; Nijsten, T.E.C.; Neumann, H.A.M. (2009). "Lipoedema: From clinical presentation to therapy. A review of the literature". British Journal of Dermatology. 161 (5): 980–6. PMID 19785610. doi:10.1111/j.1365-2133.2009.09413.x.
  17. ↑ Habbema, Louis (2009). "Safety of Liposuction Using Exclusively Tumescent Local Anesthesia in 3,240 Consecutive Cases". Dermatologic Surgery. 35 (11): 1728–35. PMID 19660028. doi:10.1111/j.1524-4725.2009.01284.x.
  18. ↑ Forner-Cordero, I.; Szolnoky, G.; Forner-Cordero, A.; Kemény, L. (2012). "Lipedema: An overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review". Clinical Obesity. 2 (3–4): 86–95. PMID 25586162. doi:10.1111/j.1758-8111.2012.00045.x.

Bibliography

  1. Wold, L. E.; Hines Jr, E. A.; Allen, E. V. (1951). "Lipedema of the Legs: A Syndrome Characterized by Fat Legs and Edema". Annals of Internal Medicine. 34 (5): 1243–50. PMID 14830102. doi:10.7326/0003-4819-34-5-1243. 
  2. Wold, L. E.; Hines Jr, E. A.; Allen, E. V. (1951). "Lipedema of the Legs: A Syndrome Characterized by Fat Legs and Edema". Annals of Internal Medicine. 34 (5): 1243–50. PMID 14830102. doi:10.7326/0003-4819-34-5-1243. 
  3. Cornely, Manuel E. (2014). "Fatter Through Lipids or Water: Lipohyperplasia Dolorosa Versus Lymphedema". American Journal of Cosmetic Surgery. 31 (3): 189–95. doi:10.5992/AJCS-D-12-00026.1. 
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