Lipedema

{{Short description|Condition involving excessive deposition of fat in the legs}}

{{Infobox medical condition (new)

| name = Lipedema

| image = Lipedema, type III.jpg

| width =

| alt =

| caption = Lipedema, type III, stage 1.

| pronounce =

| 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,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. easy bruising, pain

| complications =

| onset =

| duration =

| types =

| causes = Unknown

| risks = Family member with the condition

| diagnosis =

| differential = Lipohypertrophy, chronic venous insufficiency, lymphedema

| prevention =

| treatment = Physiotherapy, exercise compression stockings, emollients, liposuction

| medication =

| prognosis =

| frequency = Up to 11% of women

| deaths =

}}

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{{cite web | title=Lipoedema | website=nhs.uk | date=2 October 2020 | url=https://www.nhs.uk/conditions/lipoedema/ | access-date=1 April 2021}} and the fat is resistant to traditional weight-loss methods.{{cite journal |vauthors=Torre YS, Wadeea R, Rosas V, Herbst KL |title=Lipedema: friend and foe |journal=Hormone Molecular Biology and Clinical Investigation |volume=33 |issue=1 |pages= |date=March 2018 |pmid=29522416 |pmc=5935449 |doi=10.1515/hmbci-2017-0076 |url= }} 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. It often runs in families.{{cite web|title=Lipedema|url=https://rarediseases.info.nih.gov/diseases/10542/lipedema|website=rarediseases.info.nih.gov|accessdate=30 December 2016|archive-url=https://web.archive.org/web/20210318010747/https://rarediseases.info.nih.gov/diseases/10542/lipedema|archive-date=18 March 2021|url-status=live}} 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 emollients 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.{{cite journal |last1=Anne Warren Peled |first1=Anne |last2=Kappos |first2=Elisabeth |title=Lipedema: diagnostic and management challenges |journal=International Journal of Women's Health |date=August 2016 |volume=8 |pages=389–395 |doi=10.2147/IJWH.S106227 |pmid=27570465 |pmc=4986968 |doi-access=free }} 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.Herbst, K. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin 33, 155–172 (2012). https://doi.org/10.1038/aps.2011.153 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

| title=Lipedema—Pathogenesis, Diagnosis, and Treatment Options

| date=June 1, 2020

| pmc=7465366

| last1=Kruppa

| first1=P.

| last2=Georgiou

| first2=I.

| last3=Biermann

| first3=N.

| last4=Prantl

| first4=L.

| last5=Klein-Weigel

| first5=P.

| last6=Ghods

| first6=M.

| journal=Deutsches Ärzteblatt International

| volume=117

| issue=22–23

| pages=396–403

| doi=10.3238/arztebl.2020.0396

| pmid=32762835

}} Lipedema has been described in familial clusters, suggesting a genetic component.

{{citation

| url=https://www.medicalnewstoday.com/articles/lipedema-stages

| title=Lipedema: Diagnosis, causes, and types

| date=March 15, 2022

| access-date=April 13, 2024

}} It often appears around times of hormonal change such as puberty, pregnancy, and menopause, suggesting a potential hormonal component.

{{citation

| url=https://www.jobst-usa.com/patients/lymphcare/what-is-lipedema.html

| title=What Is Lipedema?

| 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.

{{citation

| url=https://my.clevelandclinic.org/health/diseases/17175-lipedema

| title=Lipedema

| date=June 1, 2023

| access-date=April 13, 2024

}}

Diagnosis

=Differential diagnosis=

Fat Disorders Research Society [http://www.fatdisorders.org/fat-disorders/lipedema-lipoedema-description Lipedema Description] {{Webarchive|url=https://web.archive.org/web/20150731052510/http://fatdisorders.org/fat-disorders/lipedema-lipoedema-description |date=2015-07-31 }}{{cite journal |doi=10.12968/bjcn.2010.15.Sup3.47363 |pmid=20559170 |title=Lipoedema: Presentation and management |journal=British Journal of Community Nursing |volume=15 |issue=4 |pages=S10–6 |year=2010 |last1=Todd |first1=Marie |s2cid=22897427 }}{{cite book |editor1-first=Michael |editor1-last=Földi |editor2-first=Ethel |editor2-last=Földi |year=2006 |chapter=Lipedema |pages=417–27 |title=Földi's Textbook of Lymphology |location=Munich |publisher=Elsevier |isbn=978-0-7234-3446-7 }}{{cite journal |pmid=23939641 |year=2013 |last1=Trayes |first1=K. P. |title=Edema: Diagnosis and management |journal=American Family Physician |volume=88 |issue=2 |pages=102–10 |last2=Studdiford |first2=J. S. |last3=Pickle |first3=S |last4=Tully |first4=A. S. }}{{cite journal |last1=Herbst |first1=Karen L |author-link=Karen Herbst |year=2012 |title=Rare adipose disorders (RADs) masquerading as obesity |journal=Acta Pharmacologica Sinica |volume=33 |issue=2 |pages=155–72 |doi=10.1038/aps.2011.153 |pmc=4010336 |pmid=22301856}}

class="wikitable"
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 is 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 edemaMuch 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:Likely(Of Lipedema) LikelyNot likely (unless primary lymphedema)LikelyVery 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.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–116Schmeller 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.{{cite journal| author=Buso G, Depairon M, Tomson D, Raffoul W, Vettor R, Mazzolai L| title=Lipedema: A Call to Action! | journal=Obesity (Silver Spring) | year= 2019 | volume= 27 | issue= 10 | pages= 1567–1576 | pmid=31544340 | doi=10.1002/oby.22597 | pmc=6790573 }}

==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{{Cite web |url=http://fatdisorders.org/fat-disorders/diagram |title=FDRS Diagram |access-date=2015-07-09 |archive-date=2017-10-11 |archive-url=https://web.archive.org/web/20171011231752/http://fatdisorders.org/fat-disorders/diagram |url-status=dead }}{{full citation needed|date=February 2017}}

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.{{Cite journal |last=Hardy |first=Denise |date=October 2016 |title=Best practice guidelines for the management of lipoedema |url=https://doi.org/10.12968/bjcn.2017.22.Sup10.S44 |journal=British Journal of Community Nursing |volume=22 |issue=Sup10 |pages=s44–s48 |doi=10.12968/bjcn.2017.22.Sup10.S44 |pmid=28961048 |via=CINAHL}}

The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies{{cite journal |last1=Dadras |first1=Mehran |last2=Mallinger |first2=Peter Joachim |last3=Corterier |first3=Cord Christian |last4=Theodosiadi |first4=Sotiria |last5=Ghods |first5=Mojtaba |title=Liposuction in the Treatment of Lipedema: A Longitudinal Study |journal=Archives of Plastic Surgery |date=2017 |volume=44 |issue=4 |pages=324–331 |doi=10.5999/aps.2017.44.4.324 |pmid=28728329 |pmc=5533060 }}{{cite journal |last1=Baumgartner |first1=A. |last2=Hueppe |first2=M. |last3=Schmeller |first3=W. |title=Long-term benefit of liposuction in patients with lipoedema: a follow-up study after an average of 4 and 8 years |journal=British Journal of Dermatology |date=May 2016 |volume=174 |issue=5 |pages=1061–1067 |doi=10.1111/bjd.14289 |pmid=26574236 |s2cid=54522402 }} 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.{{cite journal |doi=10.1097/01.ASW.0000363503.92360.91 |pmid=20087075 |title=Lipedema |journal=Advances in Skin & Wound Care |volume=23 |issue=2 |pages=81–92 |year=2010 |last1=Fife |first1=Caroline E. |last2=Maus |first2=Erik A. |last3=Carter |first3=Marissa J. |s2cid=14350132 }}{{cite journal |doi=10.1111/j.1365-2133.2009.09413.x |pmid=19785610 |title=Lipoedema: From clinical presentation to therapy. A review of the literature |journal=British Journal of Dermatology |volume=161 |issue=5 |pages=980–6 |year=2009 |last1=Langendoen |first1=S.I. |last2=Habbema |first2=L. |last3=Nijsten |first3=T.E.C. |last4=Neumann |first4=H.A.M. |s2cid=30001846 |doi-access=free }} 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.{{cite web |title=Cosmetic and Reconstructive Services of the Trunk and Groin |url=https://www.anthem.com/dam/medpolicies/abc/active/policies/mp_pw_a050277.html |date=November 12, 2019 }} 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.{{cite journal |doi=10.1111/j.1758-8111.2012.00045.x |pmid=25586162 |title=Lipedema: An overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review |journal=Clinical Obesity |volume=2 |issue=3–4 |pages=86–95 |year=2012 |last1=Forner-Cordero |first1=I. |last2=Szolnoky |first2=G. |last3=Forner-Cordero |first3=A. |last4=Kemény |first4=L. |s2cid=45550292 }}

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, GermanyReich-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.{{cite journal |last1=Wold |first1=LE |last2=Hines |first2=EA |last3=Allen |first3=EV |title=Lipedema of the legs: a syndrome characterized by fat legs and edema |journal=Annals of Internal Medicine |date=1 May 1951 |volume=34 |issue=5 |pages=1243–50 |doi=10.7326/0003-4819-34-5-1243 |pmid=14830102 |s2cid=12401140 }}{{cite journal |last1=HINES |first1=EA |title=Lipedema and physiologic edema |journal=Proceedings of the Staff Meetings of the Mayo Clinic |date=2 January 1952 |volume=27 |issue=1 |pages=7–9 |pmid=14900206 }} 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

References

{{Medical resources

| ICD10 = {{ICD10|R60}}, {{ICD10|E88.2}}

| OMIM = 614103

| DiseasesDB = 7491

| MeshID = D065134

| Orphanet = 77243

}}

{{Medicine}}

Category:Diseases of veins, lymphatic vessels and lymph nodes

Category:Women's health