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Lipedema is a disorder characterized by large amount of subcutaneous fat in the upper and lower legs due to both hyperplasia and hypertrophy. It occurs almost exclusively in females, although a few cases in men have been reported.(,) The condition is relatively rare and often seen in patients with a family history of the disease.(,) Lipedema does not yet have a registered diagnosis in the International Classification of Diseases (ICD-10) of the World Health Organization (WHO), making it difficult to establish its prevalence. However, lipedema is believed to affect nearly 11% of adult women, with noted significant differences in prevalence worldwide.(,)(,) The literature search for this report did not find epidemiological data for lipedema in Canada. The cause of lipedema is unknown, and it is likely that the condition is frequently misdiagnosed or wrongly diagnosed as lifestyle-induced obesity or lymphedema (i.e., localized fluid retention and tissue swelling).(,) However, although lipedema and obesity can co-occur, unlike obesity, lipedema usually targets the legs and thighs, without affecting the feet or hands, and the adipose tissue in lipedema is painful.(,)(,)(–) The lymphatic system remains unimpaired in the initial stages and can keep up with the increased amount of interstitial fluid.(,) However, patients with lipedema may develop secondary lymphedema (lipolymphoedema) if the fatty deposits compromise the lymphatic system. Lipedema targets both legs (and sometimes, also both hands) to the same extent and has a bilateral, nearly symmetrical presentation.(–) The excessive fat deposits are typically unresponsive to traditional weight loss interventions such as physical activity or dietary measures.(,)(,) Symptoms of the condition include pain in the lower extremities, particularly with pressure, loss of strength, easy bruising, and deterioration in daily activity levels that can greatly impact the health and quality of life of the individual with lipedema.(,)(,) Untreated lipedema may result in secondary problems including osteoarthritis, reduced mobility, psychological impairment, and lowered self-esteem. Over time, the weight of the excessive fat build-up can cause the knees to knock inward or droop to the side of the leg, and impair the inability to walk. As mentioned, in the later stages, secondary lymphedema can occur due to imbalance in the amount of fluid produced and drained by the lymphatic system.(–)(,)(,)(,) Lipedema poses a significant psychosocial burden for most patients, and associated effects often limit capacity for exercise. In severe cases, lipedema may lead to absence from work or occupational disability. There is no known curative therapy for lipedema. The primarily focus of treatment is to reduce its related lower extremity symptoms, disability, and functional limitations to improve patients’ quality of life, as well as preventing disease progression.(–)(,)(,) Treatment is divided into conservative therapy and surgical interventions. The conservative therapy includes promotion of individually adjusted healthy lifestyle, combined decongestive therapy (CDT), and other supportive measures, such as psychosocial therapy and orthopedic counseling. Conservative therapy can alleviate some lipedema symptoms such as heaviness, pain, and secondary swelling. However, these benefits are short-lived, usually requiring repeat treatment within days. Liposuction is the main surgical interventions for lipedema. Commonly used liposuction methods for lipedema are tumescent anesthesia (TA) liposuction, and water assisted liposuction (WAL). In TA liposuction, tumescent is infused in the subcutaneous tissues to cause the fat cells to swell and vessels to constrict; then blunt micro-cannulas are used to suction the fat.(,)(,) Water assisted liposuction uses a pressure spray of tumescent fluid to dislodge the fat from the connective tissue, rather than utilizing a cannula. Unlike traditional liposuction, both TA and WAL rely on the local anesthetics in the tumescent fluid and do not require general anesthesia. The objective of this report is to summarize the evidence regarding the clinical effectiveness of liposuction for the treatment of lipedema and the recommendations of evidence-based clinical guidelines regarding its use for this condition.

Lipedema is a symmetrical disorder of the subcutaneous adipose tissue that affects almost exclusively women in postpubescent age. The trinity of disfiguring hyperplastic adipose tissue of the proximal extremities, increasing pain over time and bruising is characteristic. Lymphedema can occur in both sexes and is differentiated into primary and secondary subtypes. Symmetry is not a prerequisite for diagnosis. Characteristic for lymphedema is the disproportion between lymphatic fluid and the capacity of lymphatic vessels. The most distal body parts are always more severely affected than proximal parts. In the initial phases lymphedema is painless but in advanced stages tension pain can occur. The combination of both disorders has been described; however, lipedema is not responsible for subsequent lymphedema in contrast to central obesity, which significantly increases the risk of lymphedema. The differential diagnosis is of utmost importance for a meaningful management.

Under the direction of the German Society of Phlebology (Deutsche Gesellschaft für Phlebologie) and in cooperation with other specialist associations, the S1 guideline on intermittent pneumatic compression (IPC) was adopted in January 2018. It replaces the previous guideline from March 2005. The aim of the guideline is to optimize the indication and therapeutic use of IPC in vascular diseases and edema. An extensive literature search of MEDLINE, existing guidelines, and work relevant to the topic was performed. In view of the often methodologically weak study quality with often small numbers of cases and heterogeneous treatment protocols, recommendations can often only be derived from the available data using good clinical practice/expert consensus. Intermittent pneumatic compression is used for thromboembolism prophylaxis, decongestive therapy for edema, and to positively influence arterial and venous circulation to improve clinical symptoms and accelerate ulcer healing in both the outpatient and inpatient care setting. The therapy regimens and devices used depend on the indication and target location. They can be used as outpatient and inpatient devices as well as at home for long-term indications. A target indication is thrombosis prophylaxis. IPC should be used in severe chronic venous insufficiency (stages C4b to C6), in extremity lymphedema as an add-on therapy and in peripheral arterial occlusive disease (PAOD) with stable intermittent claudication or critical ischemia. IPC can be used in post-traumatic edema, therapy-resistant venous edema, lipedema and hemiplegia with sensory deficits and edema. Absolute and relative contraindications to IPC must be taken into account and risks considered and avoided as far as possible. Adverse events are extremely rare if IPC is used correctly. If the indication and application are correct-also as an add-on therapy-it is a safe and effective treatment method, especially for the treatment of the described vascular diseases and edema as well as thrombosis prophylaxis.

Lipedema is a little-known alteration or disorder in the distribution of body fat, which affects almost exclusively women and primarily involves the lower extremities.   Epidemiological data are currently scarce and not helpful to determine the exact incidence of lipedema in the general population; its etiology and physiopathology are not clear enough; its diagnosis is basically clinical, since there are no specific diagnostic tests or enough scientific evidence to support it. However, its clinical manifestations imply deterioration in patients’ quality of life due to the physical, psychological and social impact it entails.   Since about 70% of cases are associated with pain in extremities, these are usually referred to phlebology and lymphology specialists. Treatments to control the edema are not usually successful in reducing the volume of the extremities.   This is in addition to determinants of fashion from the 21st century that demand most women to have slender bodies, while the reality is that overweight and obesity are alarmingly increasing. About 80% of obese patients do not admit to have excess weight, complicating its early diagnosis and prevention of its evolution and, consequently, delaying treatment in patients with this disease.   Due to the clinical presentation and symptoms associated with lipedema, patients are often misdiagnosed with lymphedema, obesity, lipodystrophies or chronic venous insufficiency, and therefore they are not correctly and effectively treated; in the best case scenario, they are administered symptomatic treatments.   In the absence of unified criteria for lipedema, and given the national and international controversy surrounding the term, the Spanish Association of Lymphedema and Lipedema (AEL) has put together a multidisciplinary working group of health professionals from different fields involved in the diagnosis and treatment of this disorder to draft this Consensus Document.   The aim was to answer multiple questions using the documentary evidence and clinical experience available to date.   Is the currently described physiopathology enough to explain lipedema?   Is it a progressive alteration? Does it always get worse?   When you have lipedema, is the progressive increase in body fat percentage normal?   If the main symptom is not the edema: Is manual lymphatic drainage an essential tool for the treatment?   Is it correct to prescribe compression garments in all cases?   What are the most effective treatments?   This working group included the participation of: The Spanish Association of Lymphedema and Lipedema (AEL), the Spanish Chapter of Phlebology and Lymphology (CEFyL) from the Spanish Society of Angiology and Vascular Surgery (SEACV), the Spanish Society of Aesthetic Medicine (SEME), the Spanish Society of Plastic, Reconstructive and Aesthetic Surgery (SECPRE), the Spanish Society of Dietetics and Food Science (SEDCA), the Complutense University of Madrid, and doctors specialized in Physical Medicine and Rehabilitation from the Spanish Society of Rehabilitation and Physical Medicine (SERMEF).   This document includes several chapters on the definition and physiopathology of lipedema, its diagnostic methods, differential diagnosis, classification and treatment using physical, pharmacological and surgical means. It is hoped that it can help people with lipedema and health professionals caring for them.   However, there is still a lot to learn about the etiopathogenesis, diagnosis and treatment of lipedema, so research must continue and be completed with epidemiological studies of its incidence and prevalence, always using an interdisciplinary approach.

The present, revised guidelines on lipedema were developed under the auspices of and funded by the German Society of Phlebology (DGP). The recommendations are based on a systematic literature search and the consensus of eight medical societies and working groups. The guidelines contain recommendations with respect to diagnosis and management of lipedema. The diagnosis is established on the basis of medical history and clinical findings. Characteristically, there is a localized, symmetrical increase in subcutaneous adipose tissue in arms and legs that is in marked disproportion to the trunk. Other findings include edema, easy bruising, and increased tenderness. Further diagnostic tests are usually reserved for special cases that require additional workup. Lipedema is a chronic, progressive disorder marked by the individual variability and unpredictability of its clinical course. Treatment consists of four therapeutic mainstays that should be combined as necessary and address current clinical symptoms: complex physical therapy (manual lymphatic drainage, compression therapy, exercise therapy, and skin care), liposuction and plastic surgery, diet, and physical activity, as well as psychotherapy if necessary. Surgical procedures are indicated if - despite thorough conservative treatment - symptoms persist, or if there is progression of clinical findings and/or symptoms. If present, morbid obesity should be therapeutically addressed prior to liposuction.

Introduction Lipedema is a chronic, progressive condition that can result in considerable disability. In 2011, the Dutch Society of Dermatology and Venereology organized a task force to create guidelines on lipedema, using the International Classification of Functioning, Disability and Health of the World Health Organization. Guideline development Clinical questions on significant issues in lipedema care were proposed, involving (1) making the diagnosis of lipedema; (2) clinimetric measurements for early detection and adequate follow-up; and (3) treatment. A systematic review of literature published up to June 2013 was conducted. Based on available evidence and experience of the task force, answers were formed and recommendations were stated. The guidelines define criteria to make a medical diagnosis of lipedema, a minimum data set of (repeated) clinical measurements that should be used to ensure early detection and an individually outlined follow-up plan, pillars on which conservative treatment should be based and recommendations on surgical treatment options. Conclusions Little consistent information concerning either diagnostics or therapy can be found in the literature. It is likely that lipedema is frequently misdiagnosed or wrongly diagnosed as only an aesthetic problem and therefore under- or mis-treated. Treatment is divided into conservative and chirurgic treatment. The only available technique to correct the abnormal adipose tissue is surgery. Recommendations To ensure early detection and an individually outlined follow-up, the committee advises the use of a minimum data set of (repeated) measurements of waist circumference, circumference of involved limbs, body mass index and scoring of the level of daily practice and psychosocial distress. Promotion of a healthy lifestyle with individually adjusted weight control measures, graded activity training programs, edema reduction, and other supportive measures are pillars of conservative therapy. Tumescent liposuction is the treatment of choice for patients with a suitable health profile and/or inadequate response to conservative and supportive measures.

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.

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.

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.

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.

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.

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.