Lipedema is a frequently unrecognized and misdiagnosed disorder of the fatty tissue of extremities and hips, which affects almost purely women. The beginning of the disease usually occurs with hormonal changes, such as puberty, pregnancy, or menopause. Women suffer from pain, easy bruising, and disfigurement, which may lead to early immobility and social stress. Accurate diagnosis and treatment are essential. The differentiation between obesity and lipedema is difficult, as these two different entities often occur together. Other differential diagnoses are lymphedema, benign lipohypertrophy, and Dercum’s disease. A therapy targeting the underlying cause of lipedema is not available because the exact etiology of the disorder is not clarified yet. Decongestive physical therapy is the basic conservative treatment, which is usually necessary lifelong. However, liposuction has led to a paradigm shift in the treatment of lipedema. The purposes of this article are to describe the symptoms and treatment options of the still fairly unknown disease Lipedema and to show the distinctions to its differential diagnoses.
Lipedema is a painful disease of the subcutaneous tissue, which was first named in 1940 by Allen and Hines. They described a syndrome characterized by “large legs due to the subcutaneous deposition of fat in the buttocks and lower extremities and the accumulation of fluid in the legs”[
Lipedema is possibly a common but underdiagnosed disorder, which is almost exclusively found in females, but, as there is no standardized diagnostic test, the exact prevalence is unknown. According to German studies, 8%-18% of patients referred to a lymphedema clinic suffer from lipedema[
The pathogenesis is not fully understood yet. Lipedema often affects several female members of the same family, suggesting a genetic disorder[
Histologically, the fat deposition is a result of hyperplasia and hypertrophy of fat cells in the subcutaneous adipose tissue[
Due to lack of knowledge, lipedema used to be a frequently unrecognized and misdiagnosed disorder. For a long time, the disease was equated with obesity, although lipedema fat is more resistant to reduction by diet and exercise than non-lipedema fat. The condition should be clearly distinguished from other dysfunctions of fat distribution, mixed forms of obesity and lymphedema. In addition to the progressive physical symptoms and consequences (lymphatic, dermatologic, and orthopedic problems), psychosocial distress with comfort eating and depression frequently arise in lipedema patients.
The diagnosis of lipedema is usually based on medical history and clinical features[
Disproportionate enlargement of the lower limbs in relation to the upper part of the body
Typical cuff sign. The disproportionate fat accumulation in Stage 1-3 Lipedema patients stops proximal of the malleoli
The severity of lipedema can be classified into four clinical stages according to skin conditions and the sizes of the palpable and visible fat nodules[
Stage 1: Flat skin with thickened subcutaneous tissue;
Stage 2: Increasing subcutaneous fat and walnut to apple-like indurations in the skin akin to a mattress;
Stage 3: Larger indurations and deforming skin-fat lobes, especially in the thighs and knee areas;
Stage 4: Development of additional lymphedema (lipolymphedema).
Different stages of lipedema according to the size of the fat nodules in the subcutaneous tissue: (left) Stage 1 shows a thickening of the subcutaneous tissue with small palpable nodules; (middle) Stage 2 already shows bigger fatty nodules up to walnut-size; and (right) Stage 3 is defined by overhanging skin-fat lobules, especially in the medial knee area or the development of severe column legs with cuff sign
The development of lymphedema with lipedema (often known as lipolymphedema) can occur with any stage[
In advanced stages, joint malformations are commonly seen due to the mass of soft tissue.
According to the pattern of fat distribution, one classification distinguishes five types of lipedema[
Type I: Pelvis, buttocks, and hips (saddle bag phenomenon);
Type II: Buttocks to knees, with formation of folds of fat around the inner side of the knee;
Type III: Buttocks to ankles;
Type IV: Arms;
Type V: Lower leg.
There may be a mixture of lipedema types in one person. Only the arms may be affected in 3% of lipedema cases (Type IV)[
Lipedema is often misdiagnosed and differential diagnosis is sometimes challenging. The disease has to be clearly distinguished from other entities.
One such is benign lipohypertrophy. Morphologically, lipohypertrophy may resemble lipedema. Women suffer from a constitutional disproportion of body shape with symmetrical hip- and thigh-obesity. The most common form of lipohypertrophy is the “riding breeches” obesity. The upper extremities are rarely affected. In contrast to lipedema, lipohypertrophy presents without pain, edema, or bruising[
Another differential diagnosis of lipedema is primary lymphedema, which may also affect women around puberty. Concerning differences between lipedema and primary lymphedema, patients with lipedema present symmetrical swellings, whereas primary lymphedema is usually asymmetrical
Asymmetrical limb swelling in a lymphedema patient with her left lower limb affected
Stemmer sign. It is negative in Lipedema patients. Here you can lift the skin of the dorsum of the second toe, which is not possible in Lymphedema patients (positive Stemmer sign)
Phlebolymphedema is the result of chronic venous insufficiency. It may occur in men and women, either uni- or bilaterally. Discolorations of the skin, varicose veins, or ulcer formations are typical symptoms. In contrast to lipedema, ultrasound examination in phlebolymphedema shows pathological findings. However, women with lipedema may also have varicose veins and may develop phlebolymphedema as a result of these[
Dercum’s disease, also known as adipositas dolorosa, describes a condition characterized by generalized obesity and painful, fatty tumors (lipomata) in the adipose tissue, occurring almost exclusively in women. The tumors are found on the extremities, without involvement of the feet. It is said that it commonly develops around menopause. In contrast to lipedema, edema is not present. Muscular weakness and fatigue, emotional instability, depression, and alcohol abuse are potential features of the disease[
Madelung’s Disease, also known as benign symmetrical lipomatosis and Launois-Bensaude disease, is a disorder of fat metabolism that results in an unusual accumulation of fat deposits around the neck (Type I), shoulder areas and upper arms (Type II), or pelvic areas (Type III). The condition is most common in men and almost always associated with alcohol abuse and liver damage[
Madelungs disease Type II with fat accumulations in the neck, shoulders, and arms
Lipedema is frequently misdiagnosed as obesity. While obesity affects the whole body, lipedema usually affects the upper and/or lower limbs and does not involve the feet and hands. Patients with lipedema hardly respond to restricted diet[
Patient after bariatric surgery and massive weight loss. She did not lose the lipedema fat in the affected lower legs
A recent review highlights the utility of developing a genetic diagnostic test containing candidate genes for lipedema and causative genes of diseases that can be confused with lipedema, to help differentiate lipedema from other diagnoses[
List of differential diagnoses criteria
Sex | Onset | Localisation | Symmetry | Fat increase | Pressure sensitivity | Edema | Involvement | Diets successflu | Disproportion | Pain | Other | |
---|---|---|---|---|---|---|---|---|---|---|---|---|
Lipedema | Women | Usually puberty | Legs, arms | + | + | + | + | - | - | + | + | Easy bruising |
Lipohypertrophy | Women | Usually puberty | Legs, arms | + | + | - | - | - | - | + | - | Common condition |
Primary Lymphedema | Women, men | Usually puberty | Legs | - | - | - | + | + | - | - | - | Positive Stemmer sign, pathologic lymphscintigraphy |
Phlebedema | Women, men | Adolescence | Legs | - | - | - | + | + | - | - | - | Pathologic vein function test |
Dercum’s disease | Usually women | Usually menopause | Legs | - | + | + | - | + | - | + | + | Muscle weakness, alcohol abuse, depressions |
Madelung’s disease | Usually men | Adolescence | Neck, shoulder, pelvis | + | + | + | - | + | - | + | - | Alcohol abuse, liver damage |
Obesity | Women, men | Each age | Whole body | + | + | - | - | + | + | - | - | BMI >25 |
Differential diagnoses criteria of lipedema listed regarding sex, onset of disease, affected localization, symmetry, pain, edema, and other symptoms
A targeted therapy aimed at the cause of lipedema is not known because the exact etiology is still unexplained. Therapy has essentially two objectives: (1) resolve or improve symptoms (edema, pain, and disproportion); and (2) prevent disease progression and the development of complications (lymphatic, dermatologic, and orthopedic problems). A distinction is made between conservative therapy to decongest the subcutaneous tissue and surgical therapy such as liposuction to reduce adipose tissue. As discussed above, weight management is a key component of both objectives.
Conservative approaches include compression garment therapy or wrapping to reduce edema and manual lymphatic drainage (MLD) as part of an outpatient context or as part of a complex decongestive physiotherapy (CDP). CDP is a very time-consuming therapy, being performed twice a day for 45-60 min over a period of 3-4 weeks, mainly in lymphedema clinics in an inpatient context. A component of CDP is manual lymphatic drainage. MLD is a type of gentle skin massage which stimulates contraction of the lymph collectors and enhances protein resorption. In addition to improving lymphatic circulation, MLD increases blood flow in deep and superficial veins[
CDP can also be combined with intermittent pneumatic compression (IPC). During this therapy, chambers in the sleeves, gloves, or boots of the device are inflated rhythmically from distal to proximal by an air pump to improve venous and lymphatic drainage of the limbs. Pressures between 30 and 60 mmHg are used. One cycle takes approximately 30 seconds and the treatment usually 30 min. IPC can also be used at home. Other components of conservative treatments are physical activity, healthy food plans to reduce any obesity component of lipedema, and psychosocial counseling. There is, however, a current debate about the value of the use of MLD in the routine management of lipedema[
According to studies, decongestive treatments significantly reduce capillary fragility and the number of petechiae, as well as the mean limb volume in patients with lipedema[
Currently, there is growing interest in the use of liposuction as a surgical treatment for lipedema. Decades ago, surgical therapy of lipedema consisted of extensive lipectomies and conventional liposuctions with sharp needles without use of tumescent solution. This was associated with life-threatening complications as well as persistent postoperative edema due to damage to the lymphatic vessels. As a result, conventional dry liposuction under general anesthesia was therefore contraindicated in lipedema patients[
Nowadays, the “wet”, “super-wet”, and “tumescent” techniques are used, which are less likely to damage the lymphatic system compared to the conventional “dry” technique. The use of microcannulas as well as new liposuction techniques such as power-assisted liposuction with vibrating cannulas or waterjet-assisted liposuction have been shown to further minimize tissue trauma and complication rates.
When performing liposuction in lipedema patients, the crisscross technique, which is commonly used in aesthetic surgery, is contraindicated because of the higher risk of harming lymph vessels with consecutive development of lymphedema. Therefore, it is of great importance to take the lymph vessel anatomy into account and move the cannula parallel to the lymph vessels in order to save them. This is why more incisions are usually needed to remove the fat from the affected area.
Since 2005, guidelines of the German Society of Phlebology recommend liposuction as an integrated part of therapy[
Most patients undergoing liposuction for lipedema require several treatments over several months. According to the guidelines, more than 4-6 liters of fat per session should not be removed because the risk of cardiopulmonary complications increases with increasing blood and fluid loss. Electrolyte imbalances are particularly dangerous. Usually, a minimal interval of at least three months between the sessions is recommended.
Lipedema Stage 3 patient before and after five sessions of liposuction in the lower legs
In conclusion, Lipedema is a frequently unrecognized and misdiagnosed disorder. Clinicians should be aware of clinical signs and clearly distinguish the condition from other entities. Accurate diagnosis and treatment are essential because they determine the patient’s prognosis. A targeted therapy for lipedema is not known because the exact etiology of the disorder is not clarified yet. Decongestive physical therapy is the basic conservative treatment, which is usually necessary lifelong. However, liposuction has led to a paradigm shift in the treatment of lipedema. While conservative therapy may reduce symptoms temporarily, liposuction is able to remove the pathologic adipose tissue, which may result in a sustainable symptom relief. Liposuction is an effective surgical method and should be individually considered for patients suffering from lipedema. Weight management is a key component of successful treatment whether conservative or surgical.
Made substantial contributions to conception and design of the article: Wiedner M, Aghajanzadeh D, Richter DF
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None.
All authors declared that there are no conflicts of interest.
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Written informed consent for publication of images was obtained from the patients shown on the images.
© The Author(s) 2020.