Lipedema is a condition that is almost exclusively found in women and results in enlargement of both legs due to deposits of fat under the skin.[ Women of any weight may be affected] and the fat is resistant to traditional weight-loss methods. There is no cure and typically it gets worse over time, pain may be present, and people bruise more easily.[ Over time mobility may be reduced, and due to reduced quality of life, people often experience depression.] In severe cases the trunk and upper body may be involved.[
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The cause is unknown but is believed to involve genetic and hormonal factors that regulate the lymphatic system, thus blocking the return of fats to the bloodstream.[ It often runs in families.] Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.[ It is commonly misdiagnosed.]
The condition is resistant to weight loss methods; however, unlike other fat it is not associated with an increased risks of diabetes or cardiovascular disease. Physiotherapy may help to preserve mobility. Exercise may help with overall fitness but will not prevent the progression of the disease.[ Compression stockings can help with pain and make walking easier.] Regularly moisturising with protects the skin and prevents it from drying out. Liposuction can help if the symptoms are particularly severe. 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.][
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Signs and symptoms
Associated conditions
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 treatments for lipedema although they may help associated conditions.[
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/ref> Joint pain, arthritis, dry skin, fungal infections, cellulitis and slow wound healing are also associated with lipedema.
Cause
The cause of lipedema is still unknown. There are various hypotheses about its pathophysiology, including altered adipogenesis, microangiopathy, and damage to the lymphatic system disturbing its microcirculation. Lipedema has been described in familial clusters, suggesting a genetic component.[
] It often appears around times of hormonal change such as puberty, pregnancy, and menopause, suggesting a potential hormonal component.[
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Having obesity doesn’t cause lipedema, but more than half of people with this condition have a BMI higher than 35. [
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Diagnosis
Differential diagnosis
[Fat Disorders Research Society Lipedema Description ]
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Fat deposits
widespread
| Swelling near ankles; brownish discoloration of lower legs (hemosiderin deposits). Minimal swelling is possible.
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F/M |
Around onset of obesity, diabetes, pregnancy, hypertension |
No relation to caloric intake |
Often edema, but can also occur without edema in earlier stages |
Stemmer's sign may or may not be present in lymphedema/lipolymphedema |
Pain is likely |
>30% of US adults |
Often itching +/- discoloration mistaken for cellulitis |
Very likely |
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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.[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][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]
Similar conditions
Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas.
Lipo-lymphedema
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.
Dercum's disease
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.[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]
Treatment
Several treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system.[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] The two most common conservative treatments are manual lymphatic drainage (MLD) where a therapist gently opens lymphatic channels and moves 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 regarding lymph-sparing liposuction and lipectomy.[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]
The studies of the 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. 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 regarded the procedure as reconstructive and medically necessary and did reimburse. 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 as blood vessels.
Prognosis
There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor quality of life, depression, anxiety, and pain.
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.[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][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]
History
Lipedema was first identified in the United States, at the Mayo Clinic, in 1940. Most attribute the original identification of lipedema to E. A. Hines and L. E. Wold (1951).[ Despite 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.]
See also