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Lipedema

Lipedema

Last Updated: November 5, 2023 | Dr Reza Moazzeni

Introduction

Lipedema, often mistaken for obesity, is a unique medical condition that predominantly affects women. Lipedema is an abnormal and often painful accumulation of fat in specific areas of the body, mainly the legs and sometimes the arms. It’s a condition that can significantly impact one’s quality of life. Despite its distinct nature, lipedema remains remarkably underdiagnosed, leading many to suffer from this condition in silence.

Unlike regular weight fluctuations that distribute fat reasonably evenly throughout the body, lipedema has a more targeted and disproportionate fat distribution. The condition can evolve over time and, if left unchecked, can result in mobility challenges and other complications. It is crucial to distinguish lipedema from other causes of lower limb edema, such as chronic venous insufficiency, to avoid unnecessary investigations and ineffective treatments. In this article, we will discuss lipedema’s presentation, diagnosis, and management options.

Lipedema Features

Lipedema primarily affects women and is characterized by an abnormal proliferation and disproportionate distribution of fat cells. It typically occurs in the lower limbs while sparing the feet. The affected areas in lipedema are characterized by pain, firm subcutaneous nodules and easy bruiseability. This disorder is often misdiagnosed as Obesity, lymphedema or even heart failure. Lipedema is a genetic condition passed in an autosomal dominant or X-linked dominant fashion. Therefore, a female child of an affected woman has a 50% chance of inheriting the disorder. Lipedema usually begins during adolescence and is resistant to extreme weight loss strategies.

Fig-1 illustrates a hallmark of lipedema: the sparing of the feet. A distinct fold at the ankles delineates the legs from the feet. However, as the disease advances, secondary Chronic Venous Insufficiency and Lymphedema sets in, which can cause swollen feet. 

Lipedema - spared feet
Fig-1: (Click to enlarge)

Pathophysiology

Lipedema involves an intricate interplay between adipose and vascular systems. In the early stages, abnormal fat deposition is the central pathology. However, as the condition advances, the vascular and lymphatic systems become compromised, adding to the complexity of the disorder. Here is a stepwise breakdown of the events:

Adipocyte Hyperplasia and Hypertrophy:

The hallmark of lipedema is an abnormal increase in the number (hyperplasia) and size (hypertrophy) of fat cells in the affected regions. This disproportionate fat distribution typically involves the legs and, less frequently, hips, buttocks and upper extremities.

Compromised Lymphatic Drainage:

The excessive adipose tissue in lipedema can exert pressure on the lymphatic vessels, impeding their function. Over time, this can result in reduced lymphatic drainage, leading to the accumulation of lymphatic fluid and potential progression to secondary lymphedema.

Microvascular Alterations:

Increased susceptibility to bruising in lipedema patients can be attributed to changes in the microvasculature, called microangiopathy. The expanding adipose tissue can cause fragility in the capillaries, resulting in telangiectasias, easier rupturing and subsequent hematomas.

Inflammation and Tissue Changes:

The adipose tissue in lipedema often exhibits signs of chronic inflammation. The presence of inflammatory mediators can lead to fibrosis or tissue hardening. This inflammatory state contributes to the characteristic pain associated with lipedema and tissue damage.

To put it simply, lipedema is a condition where the body’s normal way of storing and distributing fat is disrupted. This is not caused by overeating or lack of physical activity but by a pathological process that affects cellular growth, lymphatic drainage, and blood vessels. Understanding these underlying mechanisms is crucial for a comprehensive understanding of the condition and its effective management.

Lipedema vs Lifestyle-induced Obesity

Lipedema is often misdiagnosed as general Obesity, leading to misconceptions about the condition. Yet, several distinguishing features set these two apart:

  • Distribution and Proportions of Fat: Lipedema manifests as a disproportionate enlargement of the extremities. This swelling typically concludes sharply at the wrists and ankles, sparing the hands and feet. In contrast, lifestyle-induced Obesity shows a more evenly distributed adiposity, affecting the trunk as well.
  • Resistance to Weight Loss: Conventional weight loss methods, such as dieting, exercise, or bariatric surgery, often have minimal impact on lipedema. Some patients even report a worsening of their lipedema symptoms after these interventions.
  • Rate of Diabetes: Even though both conditions can lead to an elevated BMI, lipedema patients tend to have a lower prevalence of diabetes.
  • Pain and Tenderness: Affected areas in individuals with lipedema often experience pain or tenderness, a feature not commonly seen in general Obesity.
  • Bruising and Telangiectasia: Lipedema can lead to easy bruising in the affected regions, distinguishing it from general Obesity. The prevalence of telangiectasias is higher in Lipedema than in Obesity.
  • Skin Texture: Lipedema may present a “peau d’orange” or orange peel appearance in the skin, not typical of general Obesity. As the disease progresses, changes in the skin become more noticeable. In the initial stages, the skin appears smooth. However, in later stages, the skin surface becomes uneven due to the development of nodules in the subcutaneous fat, with the formation of lipomas. In advanced cases, rolls of adipose tissue with distinct contours form, particularly around the knees and thighs.
Lipedema - peau dorange skin
Click to enlarge
  • Joint Mobility: The pattern of fat distribution in lipedema can strain joints, particularly affecting the legs, leading to specific mobility challenges.
  • Psychological Impact: Lipedema patients might face heightened feelings of frustration and distress, exacerbated by frequent misdiagnosis and societal misunderstandings of their condition.

Fig-2: Lipedema (Click to enlarge)

In this image, the prominent contours of adipose tissue (fat) fold above the knee are evident. The feet remain relatively unaffected, distinguished by a band-like fold at the ankles. Additionally, early signs of Chronic Venous Insufficiency are present, manifesting as mild bilateral Lipodermatosclerosis on the shins and secondary mild pitting edema of the feet.

Fig-3: Lipedema easy bruisability (Click to enlarge)

This image showcases several characteristics of lipedema in the affected arm. Notable features include easy bruisability, a nodular skin appearance, and contoured folds in the upper arm.

Progression to Lipo-lymphedema

Lipedema, in its early stages, is primarily characterized by the disproportionate accumulation of fatty tissue in specific parts of the body, notably the limbs. However, as the disease advances, it can lead to additional complications. One such complication is the development of secondary lymphedema, which, combined with lipedema, is termed “lipo-lymphedema.”

Mechanism of Progression:

    • Over time, the expanding fat deposits in Lipedema can compress and damage the lymphatic vessels in the affected areas. This obstruction impairs the lymphatic system’s ability to drain lymph fluid, resulting in fluid accumulation and swelling. This further exacerbates the swelling initially caused by Lipedema.

Distinguishing Features:

    • Pitting Edema: While lipedema is typically associated with non-pitting edema (where pressing on the swollen skin does not leave an indentation), the presence of lymphedema can introduce pitting edema.
    • Increased Risk of Infections: The compromised lymphatic flow can increase the risk of infections, such as cellulitis, in the affected areas.
    • Hardening of Tissue: As lipo-lymphedema progresses, the affected areas might feel firmer or harder to the touch, a condition known as fibrosis.

Management Implications:

    • The management of lipo-lymphedema requires a combined approach that addresses both the excessive fatty deposits of lipedema and the impaired lymphatic drainage of lymphedema.
    • Interventions like manual lymphatic drainage and compression therapy become even more critical in managing lipo-lymphedema.

Management of Lipedema

While there is no definitive cure for lipedema, several interventions can help manage its symptoms and prevent or slow progression:

Conservative Measures:

    • Compression Therapy: Wearing compression garments or bandages can help reduce swelling, promote lymph flow, and support the affected areas.
    • Manual Lymphatic Drainage (MLD): This specialized form of massage can help stimulate lymph flow and reduce swelling. It’s best done by a trained therapist.
    • Exercise: Low-impact exercises, including aquatic exercises, can help alleviate lipedema symptoms by improving lymphatic drainage, muscle tone, and overall mobility.
    • Skin Care: Regular skin care can prevent complications like infections, especially in areas where skin overlying fatty deposits become thin or stretched.
    • Weight Management: While dieting might not reduce lipedema fat, maintaining a healthy weight can prevent additional health problems such as secondary Obesity and reduce the strain on already affected limbs.

Surgical Interventions:

    • Liposuction: This can help remove lipedema fat. There are various techniques like tumescent or water-assisted liposuction specifically tailored for lipedema patients.
    • Lymphatic-Sparing Procedures: These surgeries avoid damaging lymphatic vessels while addressing fatty deposits.

Medications:

    • Pain Management: Over-the-counter pain relievers and non-steroidal anti-inflammatory drugs (NSAIDs) can help manage pain and discomfort.
    • Supplements: Some patients have reported benefits from supplements like selenium, but evidence is limited.

Psycho-social Support:

    • Counseling & Support Groups: Due to the physical and emotional challenges posed by lipedema, seeking psycho-social support can be beneficial. Joining a support group or undergoing counselling can help patients cope with the condition and build a community with others who understand their experience.

Monitoring & Follow-Up:

    • Regular check-ups with a healthcare provider experienced in lipedema can help monitor the condition, evaluate the effectiveness of treatments, and adjust management strategies as necessary.

Conclusion

Lipedema is a condition that is often not given enough attention in medical education. It is frequently mistaken for other types of swelling in the legs. This misidentification results in unnecessary medical procedures and tests, which can cause emotional and physical stress for patients. It is crucial to raise awareness and improve understanding of Lipedema to prevent misdiagnosis and provide the best possible care for patients.

References and further reading

  • Allen EV, Hines EAJ. Lipedema of the legs: a syndrome characterised by fat legs and orthostatic edema. Proc Staff Meet Mayo Clin. 1940;15:184–187. []
  • Sudduth CL. Study of 700 Referrals to a Lymphedema Program. Lymphat Res Biol 2020; 18:534.
  • Foldi E, an Foldi M. Lipedema. In: Foldi M, Foldi E, editors. In: Foldi’s Textbook of Lymphology. Munich, Germany: Elsevier GmbH; 2006. pp. 417–427. []
  • Herbst KL. Rare adipose disorders (RADs) masquerading as obesity. Acta Pharmacol Sin. 2012;33:155–172. [PMC free article] [PubMed[]
  • Wold LE. Lipedema of the legs: a syndrome characterized by fat legs and edema. Ann Intern Med. 1951;34:1243–1250. [PubMed[]
  • http://www.lipoedema.co.uk. Accessed on July 13, 2015.
  • Rasmussen JC, et al. An abnormal lymphatic phenotype is associated with subcutaneous adipose tissue deposits in Dercum’s disease. Obesity (Silver Spring) 2014;22:2186–2192. [PMC free article] [PubMed[]
  • Pascucci A. Lipedema with multiple lipomas. Dermatol Online J. 2010;16:4. [PubMed[]
  • Van Geest AJ, et al. Lymphatic disturbances in lipoedema. Phlebologie. 2003;32:138–142. []
  • Stallworth JM, et al. The chronically swollen, painful extremity. A detailed study for possible etiological factors. JAMA. 1974;228:1656–1659. [PubMed[]
  • Greer KE. Lipedema of the legs. Cutis. 1974;14:98. []
  • Harwood CA, et al. Lymphatic and venous function in lipoedema. Br J Dermatol. 1996;134:1–6. [PubMed[]
  • Amann-Vesti BR. Microlymphatic aneurysms in patients with lipedema. Lymphology. 2001;34:170–175. [PubMed[]
  • Partsch H, et al. Clinical use of indirect lymphography in different forms of leg edema. Lymphology. 1988;21:152–160. [PubMed[]
  • Greene AK. Liposuction for Swelling in Patients with Lymphedema. N Engl J Med 2017; 377:1788.
  • Bräutigam P, et al. Analysis of lymphatic drainage in various forms of leg edema using two-compartment lymphoscintigraphy. Lymphology. 1998;31:43–55. [PubMed[]
  • Curri SB, Merlen JF. [Microvascular disorders of adipose tissue]. J Mal Vasc. 1986;11:303–309. [PubMed[]
  • Merlen JF. [Cellulitis, a conjunctive microvascular disease]. Phlebologie. 1979;32:279–282. [PubMed[]
  • Schmeller W. Tumescent liposuction: a new and successful therapy for lipedema. J Cutan Med Surg. 2006;10:7–10. [PubMed[]
  • Stutz JJ. Water jet-assisted liposuction for patients with lipoedema: histologic and immunohistologic analysis of the aspirates of 30 lipoedema patients. Aesthetic Plast Surg. 2009;33:153–162. [PubMed[]
  • Rapprich S. Liposuction is an effective treatment for lipedema-results of a study with 25 patients. J Dtsch Dermatol Ges. 2011;9:33–40. [PubMed[]
  • Rapprich S, et al. Treatment of lipoedema using liposuction. Results of our own surveys. Phlebologie. 2015;3:1–13. []
  • Schmeller W, Hueppe M. Tumescent liposuction in lipoedema yields good long-term results. Br J Dermatol. 2012;166:161–168. [PubMed[]
  • Kirstein F, et al. Patient-Reported Outcomes of Liposuction for Lipedema Treatment. Healthcare (Basel) 2023; 11.
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