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At the 3rd International Lymphoedema Framework (ILF) conference in Toronto, an attempt was made to begin to try to address this issue. Over thirty people attended a lipoedema workshop run by the author. In this workshop, I had the opportunity to talk to this international group (Canada, USA, Netherlands, Australia, UK, Germany) who shared their experiences from across the world. One of the goals of the workshop was to create an initial broad consensus about the worldwide diagnosis and management of lipoedema.The outcomes of that workshop are presented in the following report.

It is estimated that 3.5 million patients, both male and female, suffer from a congenital lipid distribution disorder of the arms and legs. Because these lipid distribution disorders are painful, owing to a lymphological high volume insufficiency and a structural drainage incompetence, the term lipohyperplasia dolorosa has become established as the name for the clinical picture of lipoedema. This lymphological clinical picture has also undergone a paradigm change with regard to the therapeutic options over the last 15 years: lipohyperplasia dolorosa is curable. This four part series reports on the principles and successes in operative lymphology. Zusammenfassung Geschätzt werden 3,5 Millionen PatientInnen, die an einer angeborenen Fettverteilungsstö-rung der Beine und Arme leiden. Da diese Fettverteilungsstörungen aufgrund einer lym -phologischen Hochvolumeninsuffizienz und einer strukturellen Drainageschwäche schmerzhaft ist, hat sich der Begriff Lipo-hyperplasia dolorosa zur Bezeichnung des Krankheitsbildes Lipödem durchgesetzt. Auch in Bezug auf die therapeutischen Optionen erlebte dieses lymphologische Krankheitsbild in den letzten 15 Jahren einen Paradigmenwechsel: Lipohyperplasia dolorosa ist heilbar. In einer vierteiligen Serie wird über Grundlagen und Erfolge der operativen Lymphologie berichtet werden.

Lipedema is an infrequently recognized and often neglected clinical entity that nearly always affects women. It poses a diagnostic challenge as one of the common disorders that is easily confused with lymphedema.

Lipoedema is a distinct clinical condition characterized by bilateral, symmetrical enlargement of the buttocks and lower limbs owing to excess deposition of subcutaneous fat. It is found almost exclusively in women. The common features associated with this condition are 'column- shaped' legs with sparing of the feet, bruising, sensitivity to pressure, and orthostatic oedema. The progression to lipo-lymphoedema or morbid obesity is possible. Conservative measures used in the management of lymphoedema can prevent progression/limit the orthostatic oedema. Surgical procedures may also play a part in the management of lipoedema.

PURPOSE: To enhance the learner's competence in caring for patients with lipedema through understanding the differential diagnoses, pathophysiology, and treatment/management options. TARGET AUDIENCE: This continuing education activity is intended for physicians and nurses with an interest in skin and wound care. OBJECTIVES: After participating in this educational activity, the participant should be better able to: 1. Differentiate lipedema from other similar diagnoses. 2. Tell patients with lipedema and their caregivers about treatment of this condition. 3. Construct assessments, treatment plans, and management options for patients with lipedema.

Lipohyperplasia dolorosa and lymphedema are completely different disease entities, which are both, however, classified under lymphology. While in lipohyperplasia dolorosa a congenital lipid distribution disorder leads to a high volume insufficiency and the corresponding clinical symptoms, lymphedema is characterized by a congenital transport incompetence of the vessels or acquired disorders of transport capacity. Both lymphedemas of different genesis are familial volume alterations of the affected regions and the increase in volume is irreversible if not exclusively still in stage I or II. According to current knowledge the solid increase in volume by lymphedema is due to a malfunctioning biomechanism by which the release of additional proteoglycans in the homeostasis system of the fluid in the interstital space plays an important role. Removal of this tissue and the sponge-like substance of proteoglycans is the aim of therapeutic approaches. Manual lymph drainage and compression can evacuate the sponge but not remove it. Lymphological liposculpture is a successful dermatosurgical measure even for secondary lymphedema. Reduction of the necessity of complex hemostasis therapy to 20% of the initial value and an adjustment of the affected extremity on the healthy side, represent a clear improvement in quality of life of patients. The same dermatosurgical method, lymphological liposculpture, has been known for many years to fulfil the successfully proven purpose for the treatment of lipohyperplasia dolorosa by the removal of subcutaneous fatty tissue, present as hyperplasia and not hypertrophy. Tenderness and the necessity for complex hemostasis therapy are no longer present or no longer necessary after lymphological liposculpture for lipohyperplasia dolorosa. This condition is permanent because the congenital fatty masses do not reoccur following surgical removal. Lipohyperplasia dolorosa is therefore curable by lymphological liposculpture. For secondary lymphedema a drastic improvement in quality of life of the patient can be achieved by this method which is demonstrated by the adjustment of symmetry of the extremities and reduction or even avoidance of complex hemostasis therapy.

In the guidelines for lipedema treatment, in addition to decongestive therapy, surgical therapy (liposuction) is advised as a standard treatment. However, in most cases the German social health insurance system refuses to pay for the costs of treatment. While primarily medical arguments are cited, it is clear that this refusal is chiefly due to the socioeconomic restrictions in the German social health insurance system. At present objective criteria in the form of prioritization are being tested for evaluation and to create a ranking list for the validity of treatment. For liposuction it is not yet clear whether in addition to an improvement in the quality of life, a reduction in lifetime treatment costs can be achieved.

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.