Lipedema

Lipedema is a disorder where there is enlargement of both legs due to deposits of fat under the skin.[1] Typically it gets worse over time, pain may be present, and sufferers bruise easily.[1] In severe cases the trunk and upper body may be involved.[1] Lipedema is commonly misdiagnosed.[2]

Lipedema
Other namesLipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome
A very advanced case of lipedema of the right leg (the knee is pointing to the right and is concealed by the overhanging lipedema).
SpecialtyVascular medicine
SymptomsIncreased fat deposits under the skin in the legs, easy bruising, pain[1]
CausesUnknown[1]
Risk factorsOverweight[1]
Differential diagnosislipohypertrophy, chronic venous insufficiency, lymphedema[1]
TreatmentPhysiotherapy, exercise[1]
FrequencyUp to 11% of women[1]

The cause is unknown but is believed to involve genetics and hormonal factors.[1] It often runs in families,[1] and is hormone related. Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.[1]

A number of treatments may be useful including physiotherapy and exercise. Physiotherapy may help to preserve mobility for a little longer than would otherwise be the case. Exercise, only as much as the patient is able to do without causing damage to the joints, may help with overall fitness but will not prevent progression of the disease.[1] While surgery can remove fat tissue it can also damage lymphatic vessels.[1] Treatment does not typically result in complete resolution.[3] It is estimated to affect up to 11% of women.[1] Onset is typically during puberty, pregnancy, or menopause.[1]

Diagnosis

Differential diagnosis

[4][5][6][7][8][2]

LipedemaLipo-lymphedemaLymphedemaObesityVenous insufficiency/venous stasis
Symptoms:Fat deposits / swelling in legs and arms not in hands or feet; hands and feet may be affected as the disease progresses.Fat deposits / swelling widespread in legs/arms/torsoFat deposits / swelling in one limb including hands and feetFat deposits

widespread

 Swelling near ankles; brownish discoloration of lower legs (hemosiderin deposits). Minimal swelling possible.
Male/female:FFF/MF/MF/M
Onset:Around hormonal shifts (puberty, pregnancy, menopause)Around hormonal shiftsAfter surgery that affects lymphatic system, or at birthAny ageAround onset of obesity, diabetes, pregnancy, hypertension
Effects of diet:Restricting calories ineffectiveRestricting calories ineffectiveRestricting calories ineffectiveDiets and weight loss strategies often effectiveNo relation to caloric intake
Presence of edema:Non-pitting edemaLots of edema; some pitting; some fibrosisPitting edemaNo edemaOften edema, but can also occur without edema in earlier stages
Presence of Stemmer Sign:Stemmer's Sign negativeStemmer's Sign positiveStemmer's Sign positiveStemmer's Sign negativeStemmer's sign may or may not be present in lymphedema/lipolymphedema
Presence of pain:Pain in affected areas likelyPain in affected areasNo pain initiallyNo painPain is likely
Affected population:Best estimate is 11% adult women (study done in Germany)Unknown; best estimate is a few percent of adult womenLow>=30% of US adults>30% of US adults
Presence of cellulitis:No history of cellulitisLikely history of cellulitisPossible history of cellulitisOften itching +/- discoloration mistaken for cellulitis
Family history:Family history likelyFamily history of lipedema likelyFamily history not likely unless primary lymphedemaFamily history likelyVery likely family history

Lipedema Stages

Lipedema is classified by stage: Stage 1: Normal skin surface with enlarged hypodermis (lipedema fat). Stage 2: Uneven skin with indentations in fat and larger hypodermal masses (lipomas). Stage 3: Bulky extrusions of skin and fat cause large deformations especially on the thighs and around the knees. These large extrusions of tissue drastically inhibit mobility.[9][10]

Lipo-lymphedema, a secondary lymphedema, is associated with both lipedema and obesity (which occur together in the majority of cases), most often lipedema stages 2 and 3.[11] Lipedema is often underdiagnosed due the difficulty in differentiating it from lymphedema, obesity, or other edemas. Many clinicians are unaware of the disease.[12] Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.[8]

Lipedema / Dercum’s disease differentiation – these conditions may co-exist. Dercum's disease is a syndrome of painful growths in subcutaneous fat. Unlike lipedema, which occurs primarily in the trunk and legs, the fatty growths can occur anywhere on the body.[13][14]

Treatment

A number of treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system.[15] The two most common conservative treatments are manual lymph drainage (MLD) where a therapist gently opens lymphatic channels and move the lymphatic fluid using hands-on techniques, and compression garments that keep the fluid at bay and assist the sluggish lymphatic flow.

The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies.[16][17] regarding lymph-sparing liposuction and lipectomy.[18]

The studies of highest quality involve tumescent local anesthesia (TLA), often referred to as simply tumescent liposuction. This can be accomplished via both Suction-Assisted Liposuction (SAL) and Power-Assisted (vibrating) liposuction.[6][19] The treatment of lipedema with tumescent liposuction may require multiple procedures. While many health insurance carriers in the United States do not reimburse for liposuction for lipedema, in 2020 several carriers regard the procedure as reconstructive and medically necessary and do reimburse.[20] Water Assisted Liposuction (WAL) is technically not considered to be tumescent but achieves the same goal as the anesthetic solution is injected as part of the procedure rather than before-hand. Developed by Doctor Ziah Taufig from Germany, it is usually performed under general anesthesia and is also considered to be lymph-sparing and protective of other tissues such blood vessels. [21]

Prognosis

Complications include a malformed appearance, reduced functionality (mobility and gait), poor Quality of Life (QOL), depression, anxiety, and pain.[2]

Epidemiology

According to an epidemiologic study by Földi E and Földi M, lipedema affects 11% of the female population, although rates from 6-39% have also been reported.[22] [23]

History

Lipedema was first identified in the United States, at the Mayo Clinic in 1940.[24][25] Most attribute the original identification of lipedema to EA Hines and LE Wold (1951).[24] In spite of that lipedema is barely known in the United States to physicians or to the patients who have the disease. Lipedema often is confused with obesity or lymphedema, and a significant number of patients currently diagnosed as obese are believed to have lipedema, either instead of or in addition to obesity.[2]

See also

References
  1. "Lipedema". rarediseases.info.nih.gov. Retrieved 30 December 2016.
  2. Herbst, Karen L (2012). "Rare adipose disorders (RADs) masquerading as obesity". Acta Pharmacologica Sinica. 33 (2): 155–72. doi:10.1038/aps.2011.153. PMC 4010336. PMID 22301856.
  3. Anne Warren Peled, Anne; Kappos, Elisabeth (August 2016). "Lipedema: diagnostic and management challenges". International Journal of Women's Health. 8: 389–395. doi:10.2147/IJWH.S106227. PMC 4986968. PMID 27570465.
  4. Fat Disorders Research Society Lipedema Description Archived 2015-07-31 at the Wayback Machine
  5. Todd, Marie (2010). "Lipoedema: Presentation and management". British Journal of Community Nursing. 15 (4): S10–6. doi:10.12968/bjcn.2010.15.Sup3.47363. PMID 20559170.
  6. 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. PMID 20087075.
  7. Földi, Michael; Földi, Ethel, eds. (2006). "Lipedema". Földi's Textbook of Lymphology. Munich: Elsevier. pp. 417–27. ISBN 978-0-7234-3446-7.
  8. 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.
  9. Leopoldo Cobos, MD, Karen Herbst, PhD, MD, Christopher Ussery, MS, CSCS, MON-116 Liposuction for Lipedema (Persistent Fat) in the US Improves Quality of Life, Journal of the Endocrine Society, Volume 3, Issue Supplement_1, April-May 2019, MON–116
  10. Schmeller W, Hueppe M, Meier-Vollrath I. Tumescent liposuction in lipoedema yields good long-term results. Br J Dermatol. 2012;166(1):161‐168. doi:10.1111/j.1365-2133.2011.10566.x
  11. Leopoldo Cobos, MD, Karen Herbst, PhD, MD, Christopher Ussery, MS, CSCS, MON-116 Liposuction for Lipedema (Persistent Fat) in the US Improves Quality of Life, Journal of the Endocrine Society, Volume 3, Issue Supplement_1, April-May 2019, MON–116
  12. Vignes S. Lipœdème : une entité mal connue [Lipedema: a misdiagnosed entity]. J Mal Vasc. 2012;37(4):213‐218. doi:10.1016/j.jmv.2012.05.002
  13. Beltran K, Herbst KL. Differentiating lipedema and Dercum's disease. Int J Obes (Lond). 2017;41(2):240‐245. doi:10.1038/ijo.2016.205
  14. http://fatdisorders.org/fat-disorders/diagram%5B%5D
  15. Fetzer A, Wise C. Living with lipoedema: reviewing different self-management techniques. Br J Community Nurs. 2015;Suppl Chronic:S14‐S19. doi:10.12968/bjcn.2015.20.Sup10.S14
  16. Dadras, Mehran; Mallinger, Peter Joachim; Corterier, Cord Christian; Theodosiadi, Sotiria; Ghods, Mojtaba (2017). "Liposuction in the Treatment of Lipedema: A Longitudinal Study". Archives of Plastic Surgery. 44 (4): 324–331. doi:10.5999/aps.2017.44.4.324. PMC 5533060. PMID 28728329.
  17. Baumgartner, A.; Hueppe, M.; Schmeller, W. (May 2016). "Long-term benefit of liposuction in patients with lipoedema: a follow-up study after an average of 4 and 8 years". British Journal of Dermatology. 174 (5): 1061–1067. doi:10.1111/bjd.14289. PMID 26574236.
  18. Sandhofer M, Hanke CW, Habbema L, et al. Prevention of Progression of Lipedema With Liposuction Using Tumescent Local Anesthesia: Results of an International Consensus Conference. Dermatol Surg. 2020;46(2):220‐228. doi:10.1097/DSS.0000000000002019
  19. 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. doi:10.1111/j.1365-2133.2009.09413.x. PMID 19785610.
  20. "Cosmetic and Reconstructive Services of the Trunk and Groin". November 12, 2019.
  21. 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. doi:10.1111/j.1758-8111.2012.00045.x. PMID 25586162.
  22. Foldi, E. and Foldi, M. (2006) Lipedema. In Foldi's Textbook of Lymphology (Foldi, M., and Foldi, E., eds) pp. 417-427, Elsevier GmbH, Munich, Germany
  23. Reich-Schupke S, Schmeller W, Brauer WJ, et al. S1 guidelines: Lipedema. J Dtsch Dermatol Ges. 2017;15(7):758-767. doi: 710.1111/ddg.13036
  24. Wold, LE; Hines, EA; Allen, EV (1 May 1951). "Lipedema of the legs: a syndrome characterized by fat legs and edema". Annals of Internal Medicine. 34 (5): 1243–50. doi:10.7326/0003-4819-34-5-1243. PMID 14830102.
  25. HINES, EA (2 January 1952). "Lipedema and physiologic edema". Proceedings of the Staff Meetings. Mayo Clinic. 27 (1): 7–9. PMID 14900206.
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