Lipedema

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Lipedema
Other namesLipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome
Specialty Vascular medicine
Symptoms Increased fat deposits under the skin in the legs, increased extracellular fluid, inflammation, [1] easy bruising, pain [2]
CausesUnknown [2]
Risk factors Family member with the condition [3]
Differential diagnosis Lipohypertrophy, chronic venous insufficiency, lymphedema [2]
Treatment Physiotherapy, exercise [2] compression stockings, emollients, liposuction [3]
FrequencyUp to 11% of women [2]

Lipedema is a medical 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 develop lipedema [2] [3] and the fat associated with lipedema is resistant to traditional weight-loss methods. [4] There is no cure and typically it gets worse over time, pain may be present, and patients bruise easily. [2] Over time mobility may be reduced, and due to reduced quality of life, patients often experience depression. [3] In severe cases the trunk and upper body may be involved. [2] Lipedema is commonly misdiagnosed. [5]

Contents

The cause is unknown but is believed to involve genetics and hormonal factors that regulate the lymphatic system, thus blocking the return of fats to the bloodstream. [2] It often runs in families; having a family member with the condition is a risk factor for developing it. [2] [3] Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema. [2] It is estimated to affect up to 11% of women. [2] Onset is typically during puberty, pregnancy, or menopause. [2]

The fat associated with lipedema is shown to be resistant to weight loss methods; however, unlike other fat, lipedema is not associated with increased risks of diabetes or cardiovascular disease. [4] Physiotherapy may help to preserve mobility for a little longer than would otherwise be the case. Exercise, only as much as the patient is able to do without causing damage to the joints, may help with overall fitness but will not prevent 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 to remove the fat 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]

Presentation

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 treatment for the 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] Obesity is not the cause of Lipedema. [11]

Diagnosis

Differential diagnosis

[12] [13] [14] [15] [16] [5]

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 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:Family history likelyFamily history of lipedema likelyFamily history not likely unless primary lymphedemaFamily history likelyVery likely family history

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

A number of 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 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 regard the procedure as reconstructive and medically necessary and do 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 blood vessels. [29]

Prognosis

There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor quality of life (QOL), 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] In spite of 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]

See also

Related Research Articles

<span class="mw-page-title-main">Lymphedema</span> Medical condition

Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fluid to the bloodstream.

<span class="mw-page-title-main">Edema</span> Accumulation of excess fluid in body tissue

Edema, also spelled oedema, and also known as fluid retention, dropsy, hydropsy and swelling, is the build-up of fluid in the body's tissue. Most commonly, the legs or arms are affected. Symptoms may include skin which feels tight, the area may feel heavy, and joint stiffness. Other symptoms depend on the underlying cause.

<span class="mw-page-title-main">Lipoma</span> Benign tumor made of fat tissue

A lipoma is a benign tumor made of fat tissue. They are generally soft to the touch, movable, and painless. They usually occur just under the skin, but occasionally may be deeper. Most are less than 5 cm (2.0 in) in size. Common locations include upper back, shoulders, and abdomen. It is possible to have several lipomas.

<span class="mw-page-title-main">Breast reduction</span> Plastic surgery procedure

Reduction mammoplasty is the plastic surgery procedure for reducing the size of large breasts. In a breast reduction surgery for re-establishing a functional bust that is proportionate to the patient's body, the critical corrective consideration is the tissue viability of the nipple–areola complex (NAC), to ensure the functional sensitivity and lactational capability of the breasts. The indications for breast reduction surgery are three-fold – physical, aesthetic, and psychological – the restoration of the bust, of the patient's self-image, and of the patient's mental health.

<span class="mw-page-title-main">Cellulitis</span> Bacterial infection of the inner layers of the skin called the dermis

Cellulitis is usually a bacterial infection involving the inner layers of the skin. It specifically affects the dermis and subcutaneous fat. Signs and symptoms include an area of redness which increases in size over a few days. The borders of the area of redness are generally not sharp and the skin may be swollen. While the redness often turns white when pressure is applied, this is not always the case. The area of infection is usually painful. Lymphatic vessels may occasionally be involved, and the person may have a fever and feel tired.

<span class="mw-page-title-main">Angiosarcoma</span> Cancer of the lining of the blood or lymphatic vessels

Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are more commonly affected, and the skin is the most affected area, with approximately 60% of cases being cutaneous(skin). Specifically, the scalp makes up ~50% of angiosarcoma cases, but this is still <0.1% of all head and neck tumors. Since angiosarcoma is an umbrella term for many types of tumor that vary greatly in origin and location, many symptoms may occur, from completely asymptomatic to non-specific symptoms like skin lesions, ulceration, shortness of breath and abdominal pain. Multiple-organ involvement at time of diagnosis is common and makes it difficult to ascertain origin and how to treat it.

<span class="mw-page-title-main">Yellow nail syndrome</span> Medical condition

Yellow nail syndrome, also known as "primary lymphedema associated with yellow nails and pleural effusion", is a very rare medical syndrome that includes pleural effusions, lymphedema and yellow dystrophic nails. Approximately 40% will also have bronchiectasis. It is also associated with chronic sinusitis and persistent coughing. It usually affects adults.

Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare disorder marked by the presence of an angiosarcoma in a person with chronic (long-term) lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema. Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment. The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.

<span class="mw-page-title-main">Podoconiosis</span> Human disease

Podoconiosis, also known as nonfilarial elephantiasis, is a disease of the lymphatic vessels of the lower extremities that is caused by chronic exposure to irritant soils. It is the second most common cause of tropical lymphedema after lymphatic filariasis, and it is characterized by prominent swelling of the lower extremities, which leads to disfigurement and disability. Methods of prevention include wearing shoes and using floor coverings. Mainstays of treatment include daily foot hygiene, compression bandaging, and when warranted, surgery of overlying nodules.

Dercum's disease is a rare condition characterized by multiple painful fatty tumors, called lipomas, that can grow anywhere in subcutaneous fat from scalp to plantar surface of the foot. Sometimes referred as adiposis dolorosa in medical literature, Dercum’s disease is more of a syndrome than a disease. While the term adiposis dolorosa may be correct, the term Dercum's disease is more often used, along with the acronym DD.

<span class="mw-page-title-main">FOXC2</span> Protein-coding gene in the species Homo sapiens

Forkhead box protein C2 (FOXC2) also known as forkhead-related protein FKHL14 (FKHL14), transcription factor FKH-14, or mesenchyme fork head protein 1 (MFH1) is a protein that in humans is encoded by the FOXC2 gene. FOXC2 is a member of the fork head box (FOX) family of transcription factors.

<span class="mw-page-title-main">Milroy's disease</span> Lymphedema commonly located in legs, caused by congenital abnormalities in the lymphatic system

Milroy's disease (MD) is a familial disease characterized by lymphedema, commonly in the legs, caused by congenital abnormalities in the lymphatic system. Disruption of the normal drainage of lymph leads to fluid accumulation and hypertrophy of soft tissues.

Persistent edema of rosacea is a hard, nonpitting edema found on the areas involved, those mainly being the forehead, glabella, upper eyelids, nose, and/or cheeks.

Liposuction, or simply lipo, is a type of fat-removal procedure used in plastic surgery. Evidence does not support an effect on weight beyond a couple of months and does not appear to affect obesity-related problems. In the United States, liposuction is the most common cosmetic surgery.

<span class="mw-page-title-main">Cellulite</span> Medical condition

Cellulite or gynoid lipodystrophy (GLD) is the herniation of subcutaneous fat within fibrous connective tissue that manifests as skin dimpling and nodularity, often on the pelvic region, lower limbs, and abdomen. Cellulite occurs in most postpubescent females. A review gives a prevalence of 85–98% of women of European descent, but it is considerably less common in women of East Asian descent. It is believed to be physiological rather than pathological. It can result from a complex combination of factors, including diet, sedentary lifestyle, hormonal imbalance or heredity, among others.

Tumescent liposuction is a technique that provides local anesthesia to large volumes of subcutaneous fat and thus permits liposuction.

Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucosis and elephantosis verrucosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.

<span class="mw-page-title-main">HAIR-AN syndrome</span> Medical condition

The HAIR-AN syndrome is a rare subtype of polycystic ovary syndrome (PCOS) characterized by hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN). The symptoms of the HAIR-AN syndrome are largely due to severe insulin resistance, which can be secondary to blocking antibodies against the insulin receptor or genetically absent/reduced insulin receptor number/function. Insulin resistance leads to hyperinsulinemia which, in turn, leads to an excess production of androgen hormones by the ovaries. High levels of androgen hormones (hyperandrogenism) in females causes excessive hair growth, acne and irregular menstruation. Patients with both underlying mechanisms of insulin resistance may have more severe hyperandrogenism. Insulin resistance is also associated with diabetes, heart disease and excessive darkening of the skin

Fat removal procedures are used mostly in cosmetic surgery with the intention of removing unwanted adipose tissue. The procedure may be invasive, as with liposuction, or noninvasive using laser therapy, radiofrequency, ultrasound or cold to reduce fat, sometimes in combination with injections.

<span class="mw-page-title-main">Familial multiple lipomatosis</span> Hereditary adipose tissue disorder

Familial multiple lipomatosis is a hereditary adipose tissue disorder that is characterized by the formation of multiple lipomas that occur in a particular distribution. The lipomas are well-encapsulated, slow-growing, benign fatty tumors. The distribution is defined as being focused in the trunk of the body and extremities. Familial Multiple Lipomatosis can be identified when multiple lipomas occur in multiple family members that span different generations. Some people may have hundreds of lipomas present.

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