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Lipedema

Condition involving excessive deposition of fat in the legs

Field	Value
name	Lipedema
image	Lipedema, type III.jpg
caption	Lipedema, type III, stage 1.
synonyms	Lipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome
field	Vascular medicine
symptoms	Increased fat deposits under the skin in the legs, increased extracellular fluid, inflammation, easy bruising, pain
causes	Unknown
risks	Family member with the condition
differential	Lipohypertrophy, chronic venous insufficiency, lymphedema
treatment	Physiotherapy, exercise compression stockings, emollients, liposuction
frequency	Up to 11% of women

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. 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.

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. 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 risk 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. It is estimated to affect up to 11% of women. Onset is typically during puberty, pregnancy, or menopause.

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. 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.{{citation | access-date=April 13, 2024 | access-date=April 13, 2024 Having obesity doesn't cause lipedema, but more than half of people with this condition have a BMI higher than 35. | access-date=April 13, 2024

Diagnosis

Differential diagnosis

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	Swelling in one or more limb, including hands and feet	Fat deposits
Male/female:	F	F	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
Effects of diet:	Restricting calories ineffective	Restricting calories ineffective	Restricting calories ineffective	Diets and weight loss strategies often effective
Presence of edema:	Non-pitting edema	Much edema; some pitting; some fibrosis	Pitting edema at first, later non-pitting edema with fibrosis	No edema
Presence of Stemmer Sign:	Stemmer's Sign negative	Stemmer's Sign positive	Stemmer's Sign positive	Stemmer's Sign negative
Presence of pain:	Pain in affected areas likely	Pain in affected areas	No pain initially	No pain
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
Presence of cellulitis:	No history of cellulitis	Likely history of cellulitis	Possible history of cellulitis
Family history:	Likely	(Of Lipedema) Likely	Not likely (unless primary lymphedema)	Likely

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.

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.

Treatment

Several treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system. 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.

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.

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.

References

Herbst KL, Kahn LA, Iker E, Ehrlich C, Wright T, McHutchison L, Schwartz J, Sleigh M, Donahue PM, Lisson KH, Faris T, Miller J, Lontok E, Schwartz MS, Dean SM, Bartholomew JR, Armour P, Correa-Perez M, Pennings N, Wallace EL, Larson E. Standard of care for lipedema in the United States. Phlebology. 2021 May 28:2683555211015887. doi: 10.1177/02683555211015887. Epub ahead of print. PMID 34049453.
(2 October 2020). "Lipoedema".
(March 2018). "Lipedema: friend and foe". Hormone Molecular Biology and Clinical Investigation.
Herbst, K. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin 33, 155–172 (2012). https://doi.org/10.1038/aps.2011.153
Fat Disorders Research Society [http://www.fatdisorders.org/fat-disorders/lipedema-lipoedema-description Lipedema Description] {{Webarchive. link. (2015-07-31)
(2010). "Lipoedema: Presentation and management". British Journal of Community Nursing.
(2006). "Földi's Textbook of Lymphology". Elsevier.
(2013). "Edema: Diagnosis and management". American Family Physician.
(2012). "Rare adipose disorders (RADs) masquerading as obesity". Acta Pharmacologica Sinica.
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
Buso G, Depairon M, Tomson D, Raffoul W, Vettor R, Mazzolai L. (2019). "Lipedema: A Call to Action!". Obesity (Silver Spring).
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
(February 2017). "FDRS Diagram".
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
Hardy, Denise. (October 2016). "Best practice guidelines for the management of lipoedema". British Journal of Community Nursing.
(2017). "Liposuction in the Treatment of Lipedema: A Longitudinal Study". Archives of Plastic Surgery.
(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.
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
(2010). "Lipedema". Advances in Skin & Wound Care.
(2009). "Lipoedema: From clinical presentation to therapy. A review of the literature". British Journal of Dermatology.
(November 12, 2019). "Cosmetic and Reconstructive Services of the Trunk and Groin".
(2012). "Lipedema: An overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review". Clinical Obesity.
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
(1 May 1951). "Lipedema of the legs: a syndrome characterized by fat legs and edema". Annals of Internal Medicine.
(2 January 1952). "Lipedema and physiologic edema". Proceedings of the Staff Meetings of the Mayo Clinic.

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