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Knowledge of both the morphology/ pathomorphology and physiology/pathophysiology of adipose tissue is limited and the behaviour of fat tissue has not been widely researched. Changes in adipose tissue (hyperplasia, hyper trophy and atrophy) are defined differently and, as a consequence, diagnostic failures frequently occur. Figure 1 shows the complicated regulatory mechanisms which are responsible for lipolysis and lipogenesis. Even if body weight is normal, appearance depends on the distribution of subcutaneous adipose tissue (Figure 2). The amount of fat we carry and how we look are, in part, subject to fashion. What was still sexy several decades ago, a bigger waist and more on the hips, no longer corresponds to how we see perfection today. Figure 2 shows the different ideals of beauty — ‘Wonderwoman’, for example, where you can detect as little fat as possible. Recently, there has been a mania for slimness. In order to comply with the current trend in fashion, young girls starve or undergo surgical procedures to have the typical female fatty tissue removed.

Lymphatic disease is quite prevalent, and often not well clinically characterized. Beyond lymphedema, there is a broad array of human disease that directly or indirectly alters lymphatic structure and function. The symptomatic and objective presentation of these patients can be quite diverse. In this review, we have attempted to provide a systematic overview of the subjective and objective spectrum of lymphatic disease, with consideration of all of the categories of disease that primarily or secondarily impair the functional integrity of the lymphatic system. Lymphedema is discussed, along with chromosomal disorders, lymphangioma, infectious diseases, lymphangioleiomyomatosis, lipedema, heritable genetic disorders, complex vascular malformations, protein-losing enteropathy, and intestinal lymphangiectasia.

ipoedema is a disease characterised by an abnormal, circumscribed accumulation of subcutaneous fat, mainly in the lower extremities, in combination with oedema. This results in an obvious disproportion between the upper and lower half of the body, as well as pain (Figure 1). Although lipoedema was first described in 1940 by Allen and Hines in the United States of America, the discussion still continues today as to whether this disease really is an entity, with some clinicians even doubting that it exists. In the 1940s it was described as a symmetrical subcutaneous deposition of fat in the buttocks and lower legs, together with an accumulation of fluid that begins almost imperceptibly, progressing gradually. Lipoedema was often associated with weight gain and accentuated by orthostatic activity. There are still aspects of lipoedema that are poorly understood. However, what we do know is that lipocytes, capillaries and venules are involved. An increased number and/or size of lipocytes results in increased fat volume, increased permeability of the capillaries causes oedema, and increased fragility of venules leads to haematoma (Table 1). Oedema is the main and most obvious cause of pain in people with lipoedema, so conservative therapy which reduces oedema can lessen the pain suffered. There are probably other factors causing pain, but oedema seems to be the most obvious. Lipoedema only develops in adult females. Figures 2a and 2b show the same patient at different ages. In most cases, lipoedema starts after puberty, a time when women want to look slim and beautiful. Figures 3a and 3b show the legs of a girl at the age of 17 and then 10 years later. The increase in volume can be seen mainly in the thighs. For some people lipoedema continues to progress until the end of life and is accentuated by pregnancy, birth and other hormonal changes, such as the menopause. In others, it remains at a steady level without progressing. The disease can also develop later in life. Figures 4a and 4b show a patient’s arm at the age of 51 and 10 years later. The forearms have stayed the same, while the upper arms have dramatically increased. It is not known why only the upper part of the arm is affected and the lower part remains unchanged.

Lipedemawas first described by Allen and Hines in 1940 (1).Today, however, a large number of physicians remain unaware of the disease and its symptoms. Many lipedema patients are not correctly diagnosed or effectively treated until after they have endured decades of suffering (2). Epidemiological statistics on the incidence of lipedema are not available. An investigation of patients treated at one lymphedema clinic from 1995 to 1996 revealed lipedema in approximately 15% of the patients treated on an inpatient basis (3). A similar number (8% to 17%) was reported in surveys carried out in 2003 in four lymphedema clinics in Germany (4). In contrast to lymphedema, literature on lipedema is extremely sparse. Lipedema findings in the Anglo-American literature in particular often report only isolated case studies (5-8). Many more publications, including a monograph, are available in German literature (9). Table 7-1 lists synonyms for lipedema.

Introduction: Complaints of lipoedema are mainly referred to as pressure pain or tension in the scientific literature. Objective: The objective was to enable a further differentiation of the quality of the perceived discomfort and provide a preliminary quantification of pain severity. Methods: 50 patients with Stage II lipoedema were asked to complete a questionnaire containing 30 items corresponding to sensory and affective pain sensations. For these items, they could choose between “completely applicable”, “largely applicable”, “somewhat applicable”, or “not applicable”. The patients were also asked to describe their complaints in their own words. Results: The items pressing and dull were specified most frequently. The following were indicated to a lesser extent: severe, tearing, nagging, unnerving, fierce, unbearable, exhausting, and sharp. Compared to patients with chronic pain, the mean values of all the patients’ sensory and affective scores yielded an average pain severity. However, when the patients used their own formulations, the complaints were often described as being very pronounced. Conclusion: Only a small portion of the adjectives offered was suitable for characterising the sensations of lipoedema. There were considerable inter-individual variations in how the pain was perceived. The severe symptoms, which are partially subjective, should be considered when characterising lipoedema.

Introduction: In the scientific literature complaints in patients with lipoedema are described as pain due to pressure or as tenderness. Objective: To better classify the quality of pain in lipoedema and to find out about the extent of complaints. Methods: 50 patients with lipoedema stage II received a questionnaire with 30 items containing adjectives of sensory and affective pain qualities. For these items they could choose between »fits exactly«, »fits fairly well«, »fits little« or »does not fit«. In addition, patients were asked to describe their complaints with their own words. Results: In most cases the items pressing and lumpish were chosen. Many patients used the characterizations heavy, tearing, distressing, enervating, fierce, unbearable, exhausting, and pinching. The median values of all the patients´ sensory and affective scores showed – compared to people with chronic pain – »average« values. However, by using their own words, the complaints were often described as extremely burdensome. Conclusion: For describing pain in lipoedema only few items were used by the patients. There were great interindividual variations. Aspects of pain can be quite burdensome; they should be used in the characterization and the grading of lipoedema. Key words: lipedema, pain assessment, pain experience scale

Lipedemawas first described by Allen and Hines in 1940 (1).Today, however, a large number of physicians remain unaware of the disease and its symptoms. Many lipedema patients are not correctly diagnosed or effectively treated until after they have endured decades of suffering (2). Epidemiological statistics on the incidence of lipedema are not available. An investigation of patients treated at one lymphedema clinic from 1995 to 1996 revealed lipedema in approximately 15% of the patients treated on an inpatient basis (3). A similar number (8% to 17%) was reported in surveys carried out in 2003 in four lymphedema clinics in Germany (4). In contrast to lymphedema, literature on lipedema is extremely sparse. Lipedema findings in the Anglo-American literature in particular often report only isolated case studies (5-8). Many more publications, including a monograph, are available in German literature (9). Table 7-1 lists synonyms for lipedema.

Lipedema is characterized by symmetric lower extremity enlargement secondary to the deposition of fat. Lipedema is not rare, but it is commonly misdiagnosed as lymphedema. We describe a 20-year-old woman with massive lower extremity enlargement that did not respond to compression therapy. Magnetic resonance imaging of the lower extremities helped to confirm the diagnosis.

Fluorescence microlymphography (FML) is an almost atraumatic technique used to visualize the superficial skin network of initial lymphatics through the intact skin of man. Visualization was performed with an incident light fluorescence microscope following subepidermal injection of minute amounts of FITC-dextran 150,000 using microneedles. Emanating from the bright dye depot, the surrounding network of microvessels is filled, documentation performed by photography or video film. In congenital Milroy lymphedema, a lack of microlymphatics (aplasia) is typical while in other primary lymphedemas and in secondary lymphedema after mastectomy or irradiation of proximal lymph nodes, the network remains intact but the depicted area is enlarged. Lymphatic microangiopathy characterized by obliterations of capillary meshes or mesh segments develops in phleboedema with trophic skin changes, progressive systemic sclerosis and Fabry's disease. In lipedema, lymphatic microaneurysms are stained. Microlymphatic pressure may also be measured using FML. For this purpose, glass micropipettes are inserted into the capillaries by means of a micromanipulator and pressure is determined by the servo-nulling technique. Normal subjects produced significantly lower pressure (7.9 +/- 3.4 mmHg) compared to patients with primary lymphedema (15.0 +/- 5.1 mmHg, p<0.001). This characteristic lymphatic hypertension may be improved by complex physiotherapy or local application of prostaglandins. Additionally, a modification of the FML procedure can be used to measure lymphatic capillary flow velocity in controls and patients. FML is suited to confirm the clinical diagnosis of lymphedema, contributes to distinguish among various forms of edema, and is useful in clinical research. In addition, FML has also become a tool for experimental animal studies including the depiction of gastric microlymphatics, the measurement of flow velocity in the naked mouse tail, and in evaluation of lymphangiogenesis in a model of Milroy disease.

Lipoedema: a new therapy regimen b Lipoedema, a disease in women, is characterised by circumscribed accumulation of subcutaneous fat, mainly in the legs, with oedema, tension pain, tenderness and haematomas.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Discuss the initial evaluation of a patient presenting with lower extremity enlargement. 2. Distinguish underlying medical conditions causing lower extremity enlargement, including lymphedema and lipedema. 3. Discuss appropriate management and treatment for patients presenting with these conditions. BACKGROUND: Given the epidemic of obesity in the United States, many patients will consult the plastic surgeon with complaints of lower extremity enlargement secondary to "fat legs." In addition to cosmetic disfigurement, some patients may suffer from underlying medical conditions that are responsible for their symptoms. Knowledge of these other causes, including lymphedema and a disorder of abnormal fat deposition known as lipedema, ensures appropriate management and/or surgical treatment for affected patients. METHODS: Initial evaluation for lower extremity enlargement should include a discussion of pertinent medical history and a focused physical examination for findings that might indicate a pathologic underlying cause. When indicated, patients should undergo additional testing, including radiologic studies, to confirm their diagnoses. RESULTS: For those patients found to have lymphatic dysfunction, conservative management, such as massage therapy, use of compression garments, and limb elevation, should be initially recommended. Excisional or suction-assisted lipectomy may be considered in patients who fail conservative therapy. More extensive consultation with the plastic surgeon is recommended for patients seeking aesthetic improvement in contour and shape of large legs without a specified underlying abnormality. CONCLUSIONS: Patients with lower extremity enlargement may present to the plastic surgeon unsure of the specific cause of their deformity. A broad differential diagnosis exists for their presentation, which can be narrowed by using the common features and unique manifestations of the conditions.

In 10 patients, lymphatic drainage was studied before and after liposuction of the legs, employing dynamic lymph scintigraphy. The data (comparison of right/left, stress/rest, before/after liposuction) were well reproducible. Lymphatic drainage was not altered significantly after liposuction as compared to the pretherapeutic status. Particularly, no relevant disturbance of lymphatic drainage became evident. Our limited data indicate that liposuction does not cause relevant damage to lymph vessels.

Surgical approaches are usually not part of the strand rad approach in lymphologic therapy. Classic therapy is conservative and controls symptoms rather than seeking cures. Plastic surgical tissue reduction results in impaired lymph flow in many cases. Improving the lymphologic disease while reducing the need for complex compression therapy are major therapeutic goals. Lymphologic liposculpture offers a successful way to treat lipohyperplasia dolorosa and offers a new concept in the treatment of secondary lymphedema.

he term “lipedema“ was first used in 1974 by ALLEN and HINES. Their publication is regarded as “the classical description“ of the syndrome5; p. 184: “We wish to describe 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... Occasionally, a patient feels, that her large legs have ‘ruined her life.’ Many are ‘ashamed’ of their legs.” This describes the patient’s problems very well, but it is difficult to define lipedema precisely because the definition depends mainly on subjective findings. There are no medical or laboratory tests to distinguish local lipohypertrophy (local fat tissue increase) of the legs or hips from lipedema or general obesity. Consequently, lipedema is not generally accepted as a real disease. We will attempt to describe the current knowledge on the pathophysiology and treatment of lipedema. Lipedema is a metabolic disorder of the adipose (fat) tissue with unknown etiology, affecting almost exclusively females. The following clinical and pathophysiological findings are of importance for differential diagnosis.