Lipedema
Lipedema is a disorder where there is enlargement of both legs due to deposits of fat under the skin. Typically it gets worse over time, pain may be present, and sufferers bruise easily. In severe cases the trunk and upper body may be involved. Lipedema is commonly misdiagnosed.
The cause is unknown but is believed to involve genetics and hormonal factors. It often runs in families, and is hormone related. Other conditions that may present similarly include lipohypertrophy, chronic venous insufficiency, and lymphedema.
A number of treatments may be useful including physiotherapy and exercise. 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. While surgery can remove fat tissue it can also damage lymphatic vessels. Treatment does not typically result in complete resolution. It is estimated to affect up to 11% of women. Onset is typically during puberty, pregnancy, or menopause.
Many clinicians either are unaware of the disease or have a hard time differentiating it from obesity or other types of edema.
Diagnosis
Differential diagnosis
| Lipedema | Lipo-lymphedema | Lymphedema | Obesity | Venous 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/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 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.
Similar conditions
Lipedema is often underdiagnosed due to the difficulty in differentiating it from lymphedema, obesity, or other edemas. Many clinicians are unaware of the disease.
Trayes 2013 published some tools including tables and a flow chart that can be used to diagnose lipedema and other edemas.
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.
Treatment
A number of treatments may be useful including physiotherapy and light exercise which does not put undue stress on the lymphatic system. The two most common conservative treatments are manual lymph drainage (MLD) where a therapist gently opens lymphatic channels and move the lymphatic fluid using hands-on techniques, and compression garments that keep the fluid at bay and assist the sluggish lymphatic flow.
The use of surgical techniques is not universal but research has shown positive results in both short-term and long-term studies. regarding lymph-sparing liposuction and lipectomy.
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. 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. 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.
Prognosis
Complications include a malformed appearance, reduced functionality (mobility and gait), poor Quality of Life (QOL), 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.
History
Lipedema was first identified in the United States, at the Mayo Clinic in 1940. Most attribute the original identification of lipedema to EA Hines and LE Wold (1951). 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.
See also
- Lymphedema
- Steatopygia
- Adiposis dolorosa
- Lipodystrophy