Lipedema | |
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Other names | Lipoedema, lipödem, lipalgia, adiposalgia, adipoalgesia, adiposis dolorosa, lipomatosis dolorosa of the legs, lipohypertrophy dolorosa, painful column leg, painful lipedema syndrome |
Lipedema, type III, stage 1. | |
Specialty | Vascular medicine |
Symptoms | Increased fat deposits under the skin in the legs, increased extracellular fluid, inflammation, [1] easy bruising, pain [2] |
Causes | Unknown [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] |
Frequency | Up 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]
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]
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]
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]
Lipedema | Lipo-lymphedema | Lymphedema | Obesity | Venous insufficiency/venous stasis | |
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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 | 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 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: | Family history likely | Family history of lipedema likely | Family history not likely unless primary lymphedema | Family history likely | Very likely family history |
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]
Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas. [19]
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]
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]
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 as blood vessels. [29]
There is no cure. Complications include a malformed appearance, reduced functionality (mobility and gait), poor quality of life, depression, anxiety, and pain. [5]
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]
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]
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.
Edema, also spelled oedema, and also known as fluid retention, dropsy and hydropsy, is the build-up of fluid in the body's tissue, a type of swelling. Most commonly, the legs or arms are affected. Symptoms may include skin that feels tight, the area feeling heavy, and joint stiffness. Other symptoms depend on the underlying cause.
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.
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.
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.
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.
Lymphangitis is an inflammation or an infection of the lymphatic channels that occurs as a result of infection at a site distal to the channel. It may present as long red streaks spreading away from the site of infection. It is a possible medical emergency as involvement of the lymphatic system allows for an infection to spread rapidly. The most common cause of lymphangitis in humans is bacteria, in which case sepsis and death could result within hours if left untreated. The most commonly involved bacteria include Streptococcus pyogenes and hemolytic streptococci. In some cases, it can be caused by viruses such as mononucleosis or cytomegalovirus, as well as specific conditions such as tuberculosis or syphilis, and the fungus Sporothrix schenckii. Lymphangitis is sometimes mistakenly called "blood poisoning". In reality, "blood poisoning" is synonymous with sepsis.
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.
Adiposis dolorosa is an outdated term for many years used synonymously as Dercum's disease, lipedema or Anders disease. While there are numerous references to adiposis dolorosa, it is recommended that the term no longer be used. Dercum's is now recognized as a separate condition, as is lipedema.
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.
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.
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.
Android fat distribution describes the distribution of human adipose tissue mainly around the trunk and upper body, in areas such as the abdomen, chest, shoulder and nape of the neck. This pattern may lead to an "apple"-shaped body or central obesity, and is more common in males than in females. Thus, the android fat distribution of men is about 48.6%, which is 10.3% higher than that of premenopausal women. In other cases, an ovoid shape forms, which does not differentiate between men and women. Generally, during early adulthood, females tend to have a more peripheral fat distribution such that their fat is evenly distributed over their body. However, it has been found that as females age, bear children and approach menopause, this distribution shifts towards the android pattern of fat distribution, resulting in a 42.1% increase in android body fat distribution in postmenopausal women. This could potentially provide evolutionary advantages such as lowering a woman's center of gravity making her more stable when carrying offspring.
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 (lymphedema). 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.
Webbed penis also known as buried or concealed penis is an acquired or congenital condition in which the scrotal skin extends onto the ventral penile shaft. The penile shaft is buried in the scrotum or tethered to the scrotal midline by a fold or web of skin. The urethra and erectile bodies are usually normal. Webbed penis is usually asymptomatic, but the cosmetic appearance is often unacceptable. This condition may be corrected by surgical techniques.
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.
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