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Expert representatives from 11 professional societies, as part of an autonomous work group, researched and developed appropriate use criteria (AUC) for lymphoscintigraphy in sentinel lymph node mapping and lymphedema. The complete findings and discussions of the work group, including example clinical scenarios, were published on October 8, 2022, and are available at https://www.snmmi.org/ClinicalPractice/ content.aspx?ItemNumber=42021. The complete AUC document includes clinical scenarios for scintigraphy in patients with breast, cutaneous, and other cancers, as well as for mapping lymphatic flow in lymphedema. Pediatric considerations are addressed. These AUC are intended to assist health care practitioners considering lymphoscintigraphy. Presented here is a brief overview of the AUC, including the rationale and methodology behind development of the document. For detailed findings of the work group, the reader should refer to the complete AUC document online.

Since 1940, several commonly cited lipedema diagnostic criteria have been published, but no one criteria has been universally adopted by research or clinical communities. A consequence of this is that interpretation of the lipedema research is dependent on knowledge of the lipedema parameters used to recruit lipedema patients into research studies, as they may vary between studies. This table summarizes key areas of agreement and descrepancy between published works and encourages all authors to rigorously document the inclusion and exclusion criteria that serve as the foundation of their clinical studies.

2 Abstract 2.1 Participating professional associations and organisations These guidelines for the diagnosis and treatment of varicose veins were prepared under the guidance of the Deutsche Gesellschaft für Phlebologie e. V. (DGP) in cooperation with the Deutsche Gesellschaft für Gefäßchirurgie und Gefäßmedizin—Gesellschaft für operative, endovaskuläre und präventive Gefäßmedizin e. V. (DGG), the Deutschen Gesellschaft für Angiologie, Gesellschaft für Gefäßmedizin e. V. (DGA), the Deutsche Dermatologischen Gesellschaft (DDG), the Deutsche Gesellschaft für Dermatochirurgie e. V. (DGDC), the Berufsverband der Phlebologen e. V. (BVP), and the Arbeitsgemeinschaft der niedergelassenen Gefäßchirurgen Deutschlands e. V. (ANG). This updated 2018/2019 version is based on the guidelines agreed and drafted by the same associations in 2004 and 2009, and it was adopted by the Boards of the participating professional associations on 30 April 2019. 2.2 Development stage of the guidelines These guidelines are based on a structured consensus process, drawing on published data to create consensus-based guidelines at development stage S2k. 2.3 Delegates of the professional associations See: https://www.awmf.org/uploads/tx_szleitlinien/037-018l_S2k_Varikose_Diagnostik-Therapie_2019-07.pdf. 2.4 Selected literature The recommendations are based on the same publications used in previous versions and a systematic literature review carried out on 21 July 2016 in the German Institute for Vascular Public Health Research (DIGG). The review included randomised studies, meta-analyses, and controlled studies. The literature search was carried out in the Medline and PubMed databases with the following search fields in German and/or English: sclerotherapy, endovenous thermal ablation, mechanochemical ablation, cyanoacrylate glue, surgical procedures (stripping), and diagnosis, prognosis, and postoperative care of varicose veins. A manual search was carried out for later publications up to December 2018.

BackgroundLipedema is a loose connective tissue disease predominantly in women identified by increased nodular and fibrotic adipose tissue on the buttocks, hips and limbs that develops at times of hormone, weight and shape change including puberty, pregnancy, and menopause. Lipedema tissue may be very painful and can severely impair mobility. Non-lipedema obesity, lymphedema, venous disease, and hypermobile joints are comorbidities. Lipedema tissue is difficult to reduce by diet, exercise, or bariatric surgery.MethodsThis paper is a consensus guideline on lipedema written by a US committee following the Delphi Method. Consensus statements are rated for strength using the GRADE system.ResultsEighty-five consensus statements outline lipedema pathophysiology, and medical, surgical, vascular, and other therapeutic recommendations. Future research topics are suggested.ConclusionThese guidelines improve the understanding of the loose connective tissue disease, lipedema, to advance our understanding towards early diagnosis, treatments, and ultimately a cure for affected individuals.

Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 (https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer’s sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient’s quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.

The COVID-19 pandemic poses a challenge to the management of non-COVID pathologies such as lymphatic diseases and lipoedema. The use of telemedicine can prevent the spread of the disease. A system is needed to help determine the clinical priority and selection of face-to-face or telemedicine options for each patient and how to carry them out during the pandemic. The Spanish Lymphology Group has drafted a consensus document with recommendations based on the literature and clinical experience, as clinical practice guidelines for the management of lymphatic abnormalities and lipoedema during the COVID-19 pandemic. These recommendations must be adapted to the characteristics of each patient, the local conditions of the centres, and the decisions of health care professionals. The document contains minimum criteria, subject to modifications according to the evolution of the pandemic, scientific knowledge and instructions from health authorities.

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 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.

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.

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.

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.

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.