Lipedema, Lipodema, Lipoedema, Lipo-Lipedema Facts

Lipedema, Lipodema, Lipoedema, Lipo-Lipedema Facts

Related Terms: Lipedema, lipedema, lipodystrophy, lipadema, lipo-lymphedema

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What is:

Lipodema is a medical condition that is often confused with lymphedema. The individual with this condition may appear to be simply obese and/or to have extremely swollen legs and swollen abdomen. The condition is an uneven distribution of fat cells in the sub-cutaneous regions generally in the legs or abdomen. One major frustration of people with lipodema is that they are accused of being simply "fat," which is absolutely not the case.

Etiology/ Cause:

Unknown, hereditary, genetic

Complications:

Perhaps one of the most critical complication is the acquisition of secondary lymphedema. The increased weight can crush the lymphatics causing blockages and hindrances to lymphatic flow. Another complication is deterioration of the joints and vertebrae from the excessive weight. Other complications may include varicose veins and/or the deep venous system. Many lipodema patients also experience a tremendous amount of pain due to the condition and the affects on the body's systems. Other complications may include "pins and needles" discomfort, decreased vascular flow in the affected limbs and a decreased skin temperature in the affected limbs.

Treatments:

There is no known "cure" for lipodema. Because it is not a medical condition caused by over-eating and improper nutrition habits, diets will not a much of an effect. Neither can the condition be treated with medicines or diuretics.

Treatments that have helped include massage therapy in conjunction with compression bandages, benzopyrones, which includes the use of coumarin. Liposuction has also been used, but the long term success is still open to debate.

Lipodema or Lymphedema:

The signs of lipodema are distinctly differant from lymphedema. First, swelling does not extend to the feet, but extends from the abdomen to the ankle. Second, the limb texture is rubbery not hard. Third, pitting edema is not present. Fourth, Stemmer's sign is negative and finally infections that plague lymphedema patients are generally not a problem with lipodema. The reason for this is that lipodema is not caused by a malformed or damaged lymphatic system.

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Lipedema – what do we know?

by Professor Horst Weissleder

Allen and Hines described in 1940 (1) “a clinical syndrome, lipedema of the legs, which is frequently very distressing. In our experience it affects solely women. The chief complaint is of swelling of the legs and feet. On questioning, the physician may elicit that enlargement of the limbs has always been generalized and symmetrical. The swelling below the knees is accentuated when patients are on their feet much and in warm weather. Aching distress in the legs is common.”Since then most of the articles dealing with lipedema have been published in Europe.

Definition

Lipedema (painful fat syndrome) can be defined as a chronic disease that occurs in female patients and is characterized by bilateral, symmetrical fatty tissue augmentation mainly in the hip region and upper and lower leg combined with a tendency to orthostatic edema. In male patients lipedema is extremely rare (2).

Morphology

Fat cells have a spherical shape and like any other cells contain fat deposits covered by a micro filament-reinforced cell membrane. Groups of fat cells form lobes which are surrounded by fibrous tissue. The septa between the lobes contain blood and lymph vessels and nerves. Fatty tissue has an excellent blood supply but fat cell-associated lymph capillaries are not present. Lipedema is likely triggered by a microangiopathy with an increased fragility of the blood capillaries. The increased capillary permeability then leads to a protein-rich intercellular edema. In subsequent stages, inflammation sets in leading to perivascular fibrosis, afew areas of fatty tissue necrosis, oil cysts and an increase in the number of the macrophages. Prominent mast cell recruitment and other inflammatory effects eventually lead to interstitial fibrosis (4).

Pathogenesis

Right now, the pathogenesis of lipedema and other types of fatty tissue enlargement (lipohypertrophy and obesity) is still unclear. According to the literature, the regulation of the fat metabolism seems to be a very complex process. There might be interaction with the nervous system, different hormones and the microcirculation (3). Genetic factors may play a role too. Details regarding the connections of the different systems and the possible influence of drugs have yet to be clarified. In addition, it is not known exactly if the increase of fatty tissue is caused by hypertrophy or hyperplasia of the fat cells (adipocytes). An increased blood flow and an increased angiomotoricity support fat reduction. In contrast a reduced blood flow seems to increase the storage of fat. The same may be true in cases of a reduced lymph flow.

Diagnosis

Basic diagnostic procedures including medical history, inspection and palpation and additional laboratory tests provide the basis for further examinations that might be necessary to finally assess the disease. In most cases, lipedema (symmetrical, pillar like volume augmentation) develops during puberty or some years later. Heavy hips and thighs are obvious signs. Women with lipedema frequently complain about pain by compression independent of the extent of fatty tissue proliferation. Small injuries that cause no macroscopic lesions in healthy people can result in hematoma due to an increased fragility of the blood vessel wall. When the lymphatic system is not damaged the feet are normal and the Stemmer sign negative. Lipedema is frequently seen in female relatives (e.g. mother, grandmother, sisters).Based on inspection and palpation lipedema can be graded into three stages:Stage I: has a normal skin surface. The subcutaneous fatty tissue has a soft consistency but multiple small nodules can be palpated. This stage can last for several years.Stage II: the skin surface becomes uneven and harder due to the increasing nodular structure (big nodules) of the subcutaneous fatty tissue (liposclerosis).Stage III: is characterized by lobular deformation due to increased fatty tissue. The palpable nodules varying in size from a walnut up to a fist and can be distinguished from the surrounding tissue by palpation. When the skin is pushed together, the so called peau d’orange or mattress phenomenon can be demonstrated.

Differential diagnosis

The most common mistake in the assessment of lipedema is diagnosis as lymphedema. The false diagnosis is usually based on the fact that the entire set of lipedema symptoms are often not known and cannot be found in many textbooks. Therefore, massive lipedematous volume increases in the legs are commonly diagnosed as obesity or lymphostatic elephantiasis (7, 8). On the other hand lipedema can be seen quite often combined with other diseases e.g. obesity, chronic venous insufficiency and lymphedema. With lipo-lymphedema, the tissue is indurated and pretibial indentations can be made. Contrary to simple lipedema, the regions of the ankle and the dorsal foot are edematous. Natural skin creases are deepened and a positive Stemmer sign confirms damage of the lymphatic system (7, 8).

Treatment

Up till now lipedema could only be treated using conservative methods with complex physical therapy and exercises. By this, a reduction of edema and a decrease or elimination of tension and pain could be reached. Nowadays the increased fat volume can be removed by surgical therapy with liposuction in tumescent local anesthesia. However, an existing lymphangiopathy must be considered as a contraindication. Liposuction is the most frequently used cosmetic procedure not only in the United States but also in Germany. Cosmetic surgery is lucrative and is increasingly being performed by non-plastic surgeons and non-physicians, some of whom have little more experience than a weekend seminar. The public perception of this cosmetic procedure as minor cosmetic surgery fails to consider the possibility that major complications may result from this which could cause fatal outcome according to recent publications. Therefore it is strongly recommended that liposuction in lipedema be performed only in specialized centers using modern techniques (5, 6). Finally, it should be mentioned that lipedema is not caused by excessive consumption of calories; hence calorie reduction will not be beneficial. Dietary measures are advised only with obesity.

References

Allen E V, Hines E A, Lipoedema of the legs. A syndrome characterized by fat legs and edema. Proc Staff. Meat. Mayo Clinic 1940; 15:1842) Chen, S. G., Hsu, S. D., Chen, T. M., Wang, H. J. Painful fat syndrome in a male patient. Br J Plast Surg 2004;57(3):282-2863) Fasshauer,M., Klein J., Blueher M., Paschke R., Adipokine: Moegliches Bindeglied zwischen Insulinresistenz und Adipositas.Dtsch Arztebl 2004;101:A3491-34954) Kaiserling E. Morphologische Alterationenen, in M.Foeldi, S.Kubik, Lehrbuch der Lymphologie, 5.Auflage, Urban & Fischer 20025) Lehnhardt, M. Homann,H.H., Druecke, . D., Palka, P., Steinau, H.U. Liposuktion–kein Problem? Majorkomplikationen und Todesfälle im deutschsprachigen Raum zwischen 1998 und 2002. LymphForsch 2004;8(2):74-786) Schmeller W., Meier-Vollrath I. Moderne Therapie des Lipödems: Kombination von konservativen und operativen Maßnahmen. LymphForsch 2004;8(1):22-267) Stroessenreuther R.H.K. Lipoedem und Cellulitis. Koeln, Viavital 20018) Weissleder H. , Schuchhardt C., Lipedema in H.Weissleder, C.Schuchhardt, ed. Lymphedema – Diagnosis and Therapy. Köln, Viavital 2001:247-265

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Lymphatic and venous function in lipoedema.

Harwood CA, Bull RH, Evans J, Mortimer PS.Department of Dermatology, St George's Hospital, London, U.K.Lipoedema is a common but infrequently recognized condition causing bilateral enlargement of the legs in women. Although generally considered to be the result of an abnormal deposition of subcutaneous fat with associated oedema, the precise mechanisms responsible for oedema formation have yet to be fully established. In order to evaluate the possible role of lymphatic or venous dysfunction in the pathogenesis of lipoedema, 10 patients were investigated by photoplethysmography (venous function) and quantitative lymphoscintigraphy (lymphatic function). The results were compared with those from patients with primary lymphoedema and those from healthy volunteers. The results demonstrated minor abnormalities of venous function in only two patients. One patient had moderately impaired lymphatic function in both legs and seven patients had a marginal degree of impairment in one or both legs. However, in none of these cases did the impairment attain the low levels seen in true lymphoedema. Lipoedema appears to be a distinct clinical entity best classified as a lipodystrophy rather than a direct consequence of any primary venous or lymphatic insufficiency.

Publication Types:
Clinical Trial
Controlled Clinical Trial
PMID: 8745878 [PubMed - indexed for MEDLINE]

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