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Lipedema, or adiposis dolorosa, is a common adipose tissue disorder that is believed to affect nearly 11% of adult women worldwide. It is characterized most commonly by disproportionate adipocyte hypertrophy of the lower extremities, significant tenderness to palpation, and a failure to respond to extreme weight loss modalities. Women with lipedema report a rapid growth of the lipedema subcutaneous adipose tissue in the setting of stress, surgery, and/or hormonal changes. Women with later stages of lipedema have a classic "column leg" appearance, with masses of nodular fat, easy bruising, and pain. Despite this relatively common disease, there are few physicians who are aware of it. As a result, patients are often misdiagnosed with lifestyle-induced obesity, and/or lymphedema, and subjected to unnecessary medical interventions and fat-shaming. Diagnosis is largely clinical and based on criteria initially established in 1951. Treatment of lipedema is effective and includes lymphatic support, such as complete decongestive therapy, and specialized suction lipectomy to spare injury to lymphatic channels and remove the diseased lipedema fat. With an incidence that may affect nearly 1 in 9 adult women, it is important to generate appropriate awareness, conduct additional research, and identify better diagnostic and treatment modalities for lipedema so these women can obtain the care that they need and deserve.
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Lipedema is a chronic, incurable, often progressive affliction that occasionally causes significant morbidity. Initially, patients develop a disproportionate increase of body fat in the legs, buttocks and/or arms. Dieting and physical exercise have only limited effect on this disproportionate body fat distribution. The legs may be sensitive and are prone to bruising after only mild trauma. This can deteriorate into severe pain and reduced mobility, ultimately leading to a limitation of activity and social participation. As a result, lipedema patients may often be diagnosed with obesity. Dietary measures generally affect the obesity component but have little effect on the disproportionate body fat distribution. Because lipedema contributes to an increased BMI, even in non-‐obese patients, a connection between lipedema and excessive calorie-‐intake or obesity is often incorrectly assumed. In addition to physical problems, lipedema can also lead to psychoso-‐ cial problems. These are often caused by the failure of consulting professionals to recognize or acknowledge the condition, or because (incorrect) recommendations for weight loss and physical exercise do not contribute to improvements in the complaints. Therefore, it is important to recognize lipedema early so that its accompanying symptoms can be acknowl-‐ edged at an early stage and be incorporated into an integrated treatment. Little consistent information regarding the diagnosis or treatment of lipedema is found in the literature. Therefore, the goal of this directive is to attempt to establish a consensus for the diagnosis of lipedema and to discuss its symptoms and influencing factors, as well as its effects on activity and social limitations, as these negatively influence the life of the patient.
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The International Compression Club (ICC) is a partnership between academics, clinicians and industry focused upon understanding the role of compression in the management of different clinical conditions. The ICC meet regularly and from these meetings have produced a series of eight consensus publications upon topics ranging from evidence-based compression to compression trials for arm lymphoedema. All of the current consensus documents can be accessed on the ICC website (http://www.icc-compressionclub.com/index.php). In May 2011, the ICC met in Brussels during the European Wound Management Association (EWMA) annual conference. With almost 50 members in attendance, the day-long ICC meeting challenged a series of dogmas and myths that exist when considering compression therapies. In preparation for a discussion on beliefs surrounding compression, a forum was established on the ICC website where presenters were able to display a summary of their thoughts upon each dogma to be discussed during the meeting. Members of the ICC could then provide comments on each topic thereby widening the discussion to the entire membership of the ICC rather than simply those who were attending the EWMA conference. This article presents an extended report of the issues that were discussed, with each dogma covered in a separate section. The ICC discussed 12 'dogmas' with areas 1 through 7 dedicated to materials and application techniques used to apply compression with the remaining topics (8 through 12) related to the indications for using compression.
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Chronic edema is a multifactorial condition affecting patients with various diseases. Although the pathophysiology of edema varies, compression therapy is a basic tenant of treatment, vital to reducing swelling. Clinical trials are disparate or lacking regarding specific protocols and application recommendations for compression materials and methodology to enable optimal efficacy. Compression therapy is a basic treatment modality for chronic leg edema; however, the evidence base for the optimal application, duration and intensity of compression therapy is lacking. The aim of this document was to present the proceedings of a day-long international expert consensus group meeting that examined the current state of the science for the use of compression therapy in chronic edema. An expert consensus group met in Brighton, UK, in March 2010 to examine the current state of the science for compression therapy in chronic edema of the lower extremities. Panel discussions and open space discussions examined the current literature, clinical practice patterns, common materials and emerging technologies for the management of chronic edema. This document outlines a proposed clinical research agenda focusing on compression therapy in chronic edema. Future trials comparing different compression devices, materials, pressures and parameters for application are needed to enhance the evidence base for optimal chronic oedema management. Important outcomes measures and methods of pressure and oedema quantification are outlined. Future trials are encouraged to optimize compression therapy in chronic edema of the lower extremities.
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DEFINITION AND CLINICAL PICTURE: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum's disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches. CLASSIFICATION: We suggest that Dercum's disease is classified into: I. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form - a form with pain in and around multiple lipomas IV. Juxtaarticular form - a form with solitary deposits of excess fat for example at the medial aspect of the knee. EPIDEMIOLOGY: Dercum's disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum's disease has not yet been exactly established. AETIOLOGY: Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma. DIAGNOSIS AND DIAGNOSTIC METHODS: Diagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded. DIFFERENTIAL DIAGNOSIS: Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours. GENETIC COUNSELLING: The majority of the cases of Dercum's disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum's disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition. MANAGEMENT AND TREATMENT: The following treatments have lead to some pain reduction in patients with Dercum's disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum's disease should be treated in multidisciplinary teams specialised in chronic pain. PROGNOSIS: The pain in Dercum's disease seems to be relatively constant over time.
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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.
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The primary lymphedema is a pathological interstitial accumulation of lymphatic fluid. Lower limbs are mainly affected by primary lymphedema. It is caused by a hereditary lack or complete absence of lymphatic vessel systeme. Final complications of chronic limb lymphedema include »elephantiasis«, lymphatic ulceration and malignant degeneration. Secondary lymphedema, obesity, phlebedema and lipedema are most important differential diagnoses.Treatment of primary lymphedema includes early manual lymph drainages, decrease in weight, exercise, prevention or therapy of skin infections. Up to the present primary lymhedema cannot be cured in causality.
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Lipoedema: Improved Quality of Life by Combination of Therapies Lipoedema is a chronic progressive disease with increased fat volumes mainly of the legs, causing body disproportion together with orthostatic oedema, pain and bruising. Early diagnosis can be achieved by clinical symptoms. Complex physical therapy with manual lymphatic drainage, compression and physiotherapy reduces oedema and complaints. Surgical therapy with liposuction reduces circumscribed fat volumes, improves body proportions and diminishes oedema and bruising. Following surgery physical therapy can be diminished in intensity and number. Nowadays the combination of physiotherapy and liposuction is considered the optimal treatment regime which can tremendously improve the patient's quality of life.
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Because of the lifelong and often progressive course and the mental trauma to the patients, lipoedema is an important dermatologic disorder. Complex physical therapy programs were introduced as a standard therapy years ago and can achieve an impressive oedema reduction. Liposuction in tumescent local anesthesia with vibrating microcannulas has proved to be a new effective treatment. A targeted and permanent reduction of the fat tissue leads to an increased quality of life due to an improved appearance, reduced tendency to swelling and less pain.
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Up until recently, complex physical therapy has been the mainstay in treatment of lipedema. This generally improved edema and reduced pain and tension in affected patients. More recently, surgical approaches such as liposuction have been used to reduce the fat volume under tumescent local anesthesia. Combining both methods, dramatic improvements can be achieved in treating the disease and in improving the quality of life. However liposuction in lipedema should only be performed in specialized medical centers.
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HYPOTHESIS: The causes and management of lower limb lymphedema in the Western population are different from those in the developing world. OBJECTIVE: To look at the differential diagnosis, methods of investigation, and available treatments for lower limb lymphedema in the West. DATA SOURCE: A PubMed search was conducted for the years 1980-2002 with the keyword "lymphedema." English language and human subject abstracts only were analyzed, and only those articles dealing with lower limb lymphedema were further reviewed. Other articles were extracted from cross-referencing. RESULTS: Four hundred twenty-five review articles pertaining to lymphedema were initially examined. This review summarizes the findings of relevant articles along with our own practice regarding the management of lymphedema. CONCLUSIONS: The common differential diagnosis in Western patients with lower limb swelling is secondary lymphedema, venous disease, lipedema, and adverse reaction to ipsilateral limb surgery. Lymphedema can be confirmed by a lymphoscintigram, computed tomography, magnetic resonance imaging, or ultrasound. The lymphatic anatomy is demonstrated with lymphoscintigraphy, which is particularly indicated if surgical intervention is being considered. The treatment of choice for lymphedema is multidisciplinary. In the first instance, combined physical therapy should be commenced (complete decongestive therapy), with surgery reserved for a small number of cases.
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Lipodystrophy, almost exclusively seen in female patients, causes psychological problems at an early age. In later life, additional complaints are heavy painful legs, edema, and varicose veins. Nearly all patients suffer from alimentary adiposity. Dermolipectomies in general are not advisable because of the resulting large scars and the risk of damaging the lymphatic system. Subcutaneous lipectomy according to the Illouz method can provide good results. Weight loss is a good alternative to liposuction of the upper legs. After sufficient weight loss, only additional liposuction of the trochanteric area and the medial side of the knee is needed. No unpleasant scarring of the leg results. Unfortunately, older patients often require a skin reduction. The results of surgery in the lower legs were, except in 2 patients, good to excellent. The results in the upper legs were disappointing because 9 of 11 patients gained weight again after surgery. Considering this, the preferable treatment now is liposuction of the lower legs, medial side of the knee, and the txochanteric area. Only in cases of ptotic skin on the medial side of the upper legs is skin reduction without lipectomy indicated. Lipodystrophy suggests a disappearance of the subcutaneous fat. When this occurs in the upper part of the body, it is called progressive lipodystrophy. Lipodystrophy is known as an abnormality of the lower half of the female body, swollen by deposition of subcutaneous fat and determined by heredity. It occurs more frequently in the lower social classes and is often accompanied by an alimentary obesity, which is a psychological reaction to the disturbed body image. Nearly all patients have severe feelings of inferiority because people mock and laugh at them; thus, the alimentary component of the obesity is a form of compensation for lack of love. By dieting, the adiposity disappears only partly because the typical deformity of lipodystrophy remains. I believe that in the lipodystrophy patient a lipedema may develop resulting from lymphatic and venous disease, which may arise later in life. Younger patients' complaints are mainly psychological and result from a disturbed body image, whereas older patients are troubled by pain and leg weariness. Treatment consists of dieting, subcutaneous lipectomy by liposuction, and prescription of elastic stockings.
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Lipedema is a common disease in the usual clinical practice. None organic description about the clinical symptoms and signs associated to this condition has been published. Fifty women with lipedema have been examined by the authors, and incidence rates of symptoms and signs have been emphasized. The following signs and symptoms were constantly reported: "Egyptian column", elastic edema, negative Stemmer's sign, alterated plantar support, cutaneous hypothermia. Some others were frequently found: ecchymosis, spontaneous pain, liposclerosis on the thigh, hypodermic hyperalgesia and pain on the internal face of the knee. Moreover, the two most relevant differential diagnosis as well as their two variant's clinical features (mixed lipedema and "thin women" lipedema) have been described.
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Leg swelling is often of edematous nature. The most important differential diagnosis lies in the distinction between venous or lymphatic forms of edema. An increased vascular permeability and alterations in blood composition have also to be taken into account. A particular entity is the lipedema. Next to an accurate history, specific inspection and palpatory criteria permit to distinguish the various forms. Tests for venous function, laboratory and technologically investigative techniques increase diagnostic accuracy. Lymphedema can only be diagnosed by an exact clinical diagnosis.
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