Lipedema

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Lipedema
Other namesLipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome
Lipödem.jpg
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 factorsFamily member with the condition,[2]
Differential diagnosislipohypertrophy, chronic venous insufficiency, lymphedema[1]
TreatmentPhysiotherapy, exercise[1] compression stockings, emollients, liposuction [2]
FrequencyUp to 11% of women[1]

Lipedema is a condition that is almost exclusively found in women[2] and results in enlargement of both legs due to deposits of fat under the skin.[1] Women of any weight may develop lipedema.[1][2] There is no cure and typically it gets worse over time, pain may be present, and sufferers bruise easily.[1] Over time mobility may be reduced and due to reduced quality of life suffers often experience depression.[2] In severe cases the trunk and upper body may be involved.[1] Lipedema is commonly misdiagnosed.[3]

The cause is unknown but is believed to involve genetics and hormonal factors.[1] It often runs in families, with having a member of the family with the condition in the family being a risk factor for developing it.[1][2] Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.[1] It is estimated to affect up to 11% of women.[1] Onset is typically during puberty, pregnancy, or menopause.[1]

Initial treatment is generally basic health care approaches of a healthy diet, exercise trying to maintain a healthy weight.[2] 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] Compression stockings can help with pain and make walking easier.[2] Regularly moisturising with emollients protects the skin and prevents it from drying out.[2] Liposuction to remove the fat can help if the symptoms are particularly severe.[2] While surgery can remove fat tissue it can also damage lymphatic vessels.[1] Treatment does not typically result in complete resolution.[4]

Many clinicians either are unaware of the disease or have a hard time differentiating it from obesity or other types of edema.[5]

Diagnosis[edit]

Differential diagnosis[edit]

[6][7][8][9][10][3]

Lipedema Lipo-lymphedema Lymphedema Obesity Venous 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/torso Fat deposits / swelling in one limb including hands and 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 Much 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 stages[edit]

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.[11][12]

Similar conditions[edit]

Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas. Many clinicians are unaware of the disease.[5]

Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.[10]

Lipo-lymphedema[edit]

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.[13]

Dercum's disease[edit]

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.[14][15]

Conditions associated with lipidema[edit]

Depression and anxiety are very common for a variety of reasons, particularly the fact that diagnosis usually takes a long time and patients have received much advice on diet and exercise in the meantime, neither of which are effective treatment for the lipedema although they may help associated conditions.[16] Joint pain, arthritis, dry skin, fungal infections, cellulitis and slow wound healing.[17]

Treatment[edit]

A number of treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system.[18] 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.[citation needed]

The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies.[19][20] regarding lymph-sparing liposuction and lipectomy.[21]

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.[8][22] 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.[23] 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. [24]

Prognosis[edit]

There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor Quality of Life (QOL), depression, anxiety, and pain.[3]

Epidemiology[edit]

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.[25] [26]

History[edit]

Lipedema was first identified in the United States, at the Mayo Clinic in 1940.[27][28] Most attribute the original identification of lipedema to EA Hines and LE Wold (1951).[27] 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.[3]

See also[edit]

References[edit]

  1. ^ a b c d e f g h i j k l m n o p "Lipedema". rarediseases.info.nih.gov. Retrieved 30 December 2016. CS1 maint: discouraged parameter (link)
  2. ^ a b c d e f g h i j "Lipoedema". nhs.uk. 2 October 2020. Retrieved 1 April 2021.
  3. ^ a b c d 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.
  4. ^ 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.
  5. ^ a b 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
  6. ^ Fat Disorders Research Society Lipedema Description Archived 2015-07-31 at the Wayback Machine
  7. ^ 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.
  8. ^ a b 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.
  9. ^ Földi, Michael; Földi, Ethel, eds. (2006). "Lipedema". Földi's Textbook of Lymphology. Munich: Elsevier. pp. 417–27. ISBN 978-0-7234-3446-7.
  10. ^ a b 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.
  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. ^ 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
  13. ^ 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
  14. ^ 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
  15. ^ http://fatdisorders.org/fat-disorders/diagram[full citation needed]
  16. ^ Herbst, K. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin 33, 155–172 (2012). https://doi.org/10.1038/aps.2011.153
  17. ^ Herbst, K. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin 33, 155–172 (2012). https://doi.org/10.1038/aps.2011.153
  18. ^ 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
  19. ^ 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.
  20. ^ 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.
  21. ^ 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
  22. ^ 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.
  23. ^ "Cosmetic and Reconstructive Services of the Trunk and Groin". November 12, 2019.
  24. ^ 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.
  25. ^ 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
  26. ^ 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
  27. ^ a b 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.
  28. ^ HINES, EA (2 January 1952). "Lipedema and physiologic edema". Proceedings of the Staff Meetings. Mayo Clinic. 27 (1): 7–9. PMID 14900206.

External links[edit]

Classification
External resources