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Lipoedema is an infrequently recognized disorder in women. Lipoedema is characterized by bilateral enlargement of the legs due to abnormal depositions of subcutaneous fat associated with often mild oedema. There is substantial variability in disease severity. The diagnosis should be made as early as possible to prevent complications of the disorder, which is associated with increasing functional and cosmetic morbidity. This review describes clinical manifestations, pathogenesis, technical investigations, management and therapies of lipoedema, with the aim of optimizing management and care of patients with lipoedema.

Lipoedema is an infrequently recognized disorder in women. Lipoedema is characterized by bilateral enlargement of the legs due to abnormal depositions of subcutaneous fat associated with often mild oedema. There is substantial variability in disease severity. The diagnosis should be made as early as possible to prevent complications of the disorder, which is associated with increasing functional and cosmetic morbidity. This review describes clinical manifestations, pathogenesis, technical investigations, management and therapies of lipoedema, with the aim of optimizing management and care of patients with lipoedema.

Lymphedema results from impaired lymphatic transport with increased limb volume. Primary and secondary forms can be distinguished. Secondary lymphedema of the upper limb is the most frequent in France. A 2-cm difference on any segment of the limb confirms the diagnosis of lymphedema. Calculated lymphedema volume using the formula for a truncated cone is required to assess the efficacy of treatment and to monitor follow-up. Primary lymphedema is sporadic but rarely familial. Lymphoscintigraphy is useful in the primary form to evaluate precisely lymphatic function of the two limbs. Erysipelas is the main complication,but psychological or functional discomfort may occur throughout the course of lymphedema. Lipedema is the main differential diagnosis, defined as an abnormal accumulation of fat from hip to ankle and occurs almost exclusively in obese women.

Overweight and obesity is a public health problem in Hungary and in the Western world. It is important to underline that obesity is an illness and an important risk factor for several skin and other diseases. An overview of skin diseases caused or aggravated by obesity (acanthosis nigricans, acrochordons, keratosis pilaris, hyperandrogenism, stria, adiposis dolorosa, lymphoedema, chronic venous insufficiency, plantar hyperkeratosis, lipoedema, skin infections, acne inversa, psoriasis, tophi) helps us to look and see as well. Look for the possibility of skin infections as it helps the early diagnosis and to avoid complications. Draw patients' attention to the preventive importance of skin care. In case of an obese patient the usual dosage of most local and systemic drugs should be modified. It must be kept in mind that obesity directly or indirectly starts unfavorable processes in almost all organ systems. Therefore, only a multidisciplinary care may secure treatment and rehabilitation of obese patients. Dermatological and lymphological care is often part of the rehabilitation.

Lipofibromatosis is a slow-growing, childhood soft-tissue neoplasm that is often confused with other conditions. We report a patient with lipofibromatosis causing extremity enlargement at birth. The lesion initially was thought to be a vascular anomaly or lipedema on clinical and MRI examination. When involving the lower extremity, diffuse lipofibromatosis must be differentiated from more common causes of lower limb enlargement in children: lymphatic malformation, lymphedema, or lipedema. Compared with these more frequent conditions, lipofibromatosis usually causes less morbidity. Management of the tumor includes observation or excision. Because complete extirpation of the lesion is difficult, the recurrence rate is high.

The challenges of providing effective treatment for patients with lymphoedema-related disorders have never been more prominent. Conservative management of lymphoedema remains of central importance, however, there is increasing evidence that other therapies, such as surgical approaches, may have an important part to play in the arena of treatment. The findings from these proceedings highlight the potential role that surgical approaches, including liposuction, can have in transforming the lives of patients. Conservative approaches, while effective in many patients, do not provide a solution for some of the complex issues patients face, nor do they always provide sustained results even after periods of expensive, intensive treatment. Relatively little attention has been placed on the impact that lymphatic conditions have on the lives of patients and their families. However, research is beginning to show that patients’ health-related quality of life is significantly affected and that previously dismissed symptoms, such as pain, are a feature of the condition. Lymphoedema and lipoedema are both likely to have a deleterious effect on the body image of patients who live day-to-day with distorted limbs. Treatments such as liposuction have the potential to reverse some of the crippling effects of these disorders on the psychosocial health of patients. The indications from these proceedings are that this is an important and encouraging area of practice that must be considered as part of the armoury of treatment. Liposuction has often been viewed negatively by the lymphoedema community, who have been sceptical about its value and concerned for its safety. This document shows that when it is used appropriately, by well trained surgeons, it can be very effective. In fact, the area of liposuction now has a growing evidence base that rivals many of the other conservative areas of lymphoedema management. Management of lymphatic conditions demands that the professional groups begin to work together in a more collaborative way in order to develop treatments that really address the growing number of patients with these types of conditions.

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, hypertrophy 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.

Tissue proliferation can appear with or without gigantism and can be generalized or localized. It is not unusual for tissue proliferation to be mistaken for lymphedema.

Lipoedema 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.

Lipoedema is a disease of women, characterised by a circumscribed accumulation of subcutaneous fat, mainly in the legs, with oedema, hematomas, and pain from tension and pressure. Up to now, only conservative treatment with manual lymphatic drainage and compression was possible. Nowadays surgical therapy with liposuction under tumescent local anaesthesia can not only improve morphology but can also reduce complaints. When performed with modern techniques by experienced surgeons, liposuction is very safe and highly effective. The combination of conservative and surgical methods can considerably improve quality of life.

Lymphoedema is a debilitating, progressive and incurable pathological condition caused by a chronic imbalance between the production and transport of lymph within the lymphatic system. There are many reasons for this damage or disruption to the lymphatic system. Lymphoedema results in significant physical and psychological morbidity. Approximately 140–250 million people worldwide are affected by this condition, mostly in developing countries as a result of lymphatic filariasis, a parasitic infection transmitted by mosquitoes. In the majority of cases, treatment is non-curative, complex and requires a multidisciplinary clinical approach. Surgery takes place as a last resort when conservative treatment has proven ineffective to restore function, reduce comorbidity or the frequency of erysipelas attacks.

Lipedema is a common but rarely diagnosed disease or frequently confused with obesity. Patients are almost exclusively women. It is characterised by symmetrical, circumscribed, in advanced form deforming fat tissue accumulation on the legs that is associated with lymphedema. Spontaneous pain, pain to pressure and tendency to hematoma are characteristic. One of the possible causes of a fat leg, that is a very common complaint, is lipedema. Main differential diagnoses are obesity, lipohypertrophy and primary and secondary lymphedema. It is often associated with chronic venous and lymphatic insufficiency, early degenerative articular disease and obesity. The disease is rarely recognized and the treatment modalities are not widely known. Therefore patients feel very frustrated that leads to psychologic disorders. Until recently only conservative treatment was possible (combination of manual or intermittent pneumatic drainage, compression bandages and garments and physiotherapy). More recently surgical intervention (liposuction) is also included in the treatment options. The significance of lipedema is due not only to the disease itself, but also to the combination of lipedema and the group of associated and secondary diseases (articular and venous diseases, lymphedema, obesity, psychologic disorders). The more diseases coexist, the worse is the prognosis of lipedema itself. To prevent and delay this disease, it is indispensable to recognise it as early as possible and to treat it expertly and follow up patients suffering from lipedema.

Lymphoedema is a clinical manifestation of an impaired lymphatic drainage with accumulation of lymphatic fluid. Lipoedema is characterized by bilateral enlargement of the legs and/or arms due to abnormal deposition of fatty tissue, which accumulates fluid. Conservative treatment including compressions garments and lymphatic drainage is suitable to prevent ongoing clinical deterioration although both diseases cannot be cured. The ability to properly diagnose lymphoedema and lipoedema is crucial to prevent the significant morbidity and loss of quality of life that is associated with this condition. It is imperative that patients with lymphoedema are referred to specially trained healthcare professionals to ensure optimal treatment. Continuous therapy with strict compliance of the patients is essential, and premature interruption is the most frequent mistake. Lipoedema is a different entity but patients are still fighting for acceptance. The mutual relation of lipoedema and obesity and the poor knowledge of the underlying mechanisms limit the acceptance of lipoedema as a relevant disease.

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.