Lipedema is a condition that is almost exclusively found in women[3] and results in enlargement of both legs due to deposits of fat under the skin.[2] Women of any weight may be affected[2][3] and the fat is resistant to traditional weight-loss methods.[4] There is no cure and typically it gets worse over time, pain may be present, and people bruise more easily.[2] Over time mobility may be reduced, and due to reduced quality of life, people often experience depression.[3] In severe cases the trunk and upper body may be involved.[2]
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.[2] It often runs in families.[2][3] Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.[2] It is commonly misdiagnosed.[5]
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.[4]Physiotherapy may help to preserve mobility. Exercise may help with overall fitness but will not prevent the progression of the disease.[2] Compression stockings can help with pain and make walking easier.[3] Regularly moisturising with emollients protects the skin and prevents it from drying out.[3]Liposuction can help if the symptoms are particularly severe.[3] While surgery can remove fat tissue it can also damage lymphatic vessels.[2] Treatment does not typically result in complete resolution.[6] It is estimated to affect up to 11% of women.[2] Onset is typically during puberty, pregnancy, or menopause.[2]
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.[7] Joint pain, arthritis, dry skin, fungal infections, cellulitis and slow wound healing are also associated with lipedema.[7]
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.[8] Lipedema has been described in familial clusters, suggesting a genetic component.[9] It often appears around times of hormonal change such as puberty, pregnancy, and menopause, suggesting a potential hormonal component.[10] Having obesity doesn’t cause lipedema, but more than half of people with this condition have a BMI higher than 35. [11]
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 is 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:
Likely
(Of Lipedema) Likely
Not likely (unless primary lymphedema)
Likely
Very 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.[17][18]
Similar conditions
Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas.[19]
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.[17]
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.[20][21]
Treatment
Several treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system.[22] 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.[23]
The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies[24][25] regarding lymph-sparing liposuction and lipectomy.[26]
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.[14][27] 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.[28] 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.[29]
Prognosis
There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor quality of life, depression, anxiety, and pain.[5]
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.[30][31]
History
Lipedema was first identified in the United States, at the Mayo Clinic, in 1940.[32][33] Most attribute the original identification of lipedema to E. A. Hines and L. E. Wold (1951).[32] 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.[5]
↑ 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.
↑ Trayes, K. P.; Studdiford, J. S.; Pickle, S; Tully, A. S. (2013). "Edema: Diagnosis and management". American Family Physician. 88 (2): 102–10. PMID23939641.
1 2 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
↑ 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
↑ 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. PMID26574236. S2CID54522402.
↑ 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
↑ 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. PMID25586162. S2CID45550292.
↑ 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
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