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In the differential diagnosis of lymphedema, lipedema is often mentioned; however, in lipedema, there is initially no primary lymphatic impairment due to dysfunction. In the later stages of lipedema, obesity is often involved and will influence the patient negatively. In daily practice, contrary to the treatment point of view, a therapeutic approach of manual lymph drainage and compression therapy is often used for both lipedema and lymphedema, although these are two distinguishable diagnoses. Therefore, differentiating these two conditions is crucial for an optimal, dedicated treatment program. Because there is no consensus on the criteria for the diagnosis lipedema, a new method should be used to categorize and stratify patients to offer a dedicated treatment program and psycho-social support. The WHO method of International Classification of functioning, disability and health (ICF) is designed for a new approach of chronic diseases and can be of help in patients with lipedema. Lipedema is a debilitating, incurable chronic pathological condition that is often misdiagnosed or unrecognized. The most common form of symmetrical fat distribution is obesity. However, many diseases and syndromes, such as Cushing’s disease21 and polycystic ovary syndrome22, can lead to unusual fat deposition patterns. Lipedema almost exclusively affects women and is characterized by a bilateral, progressive accumulation and misdistribution of subcutaneous fat, usually below the waist. Only two cases of lipedema in male patients have been reported. The onset of lipedema is often soon after puberty; however, lipedema can develop later in life, such as during pregnancy or menopause. During the course of lip edema, very often a obesity component will be present. As a clinical syndrome, lipedema was first described as the presence of excessive fat deposits on the buttocks, thighs and legs associated with mild edema. Once accumulated, the excessive fat deposits respond poorly to vigorous dietary measures. Dietary measures predominately reduce fat in the areas of the body without lipedema. This process leads to an asymmetrical distribution of body fat between the upper body and lower extremities, a disproportional fat distribution. Because of the diet-resistant nature of lipedema part, many patients are reluctant to pursue stringent dietary measures, as this emphasizes the disproportionate body fat. Approximately 50% of lipedema patients have an elevated body mass index (BMI), which complicates the differentiation between lipedema and obesity (from the Latin obesus, grown fat by eating). Nevertheless, very often volume reduction of a lipedema leg is achieved after a conservative treatment program and weight reduction. In addition, large accumulations of subcutaneous fat deposits can mechanically compress existing and previously functional lymphatic structures, leading to a mechanical insufficiency and secondary lymphedema over time. In practice, diagnosing lipedema is often complicated, but lipedema must be differentiated from lipohypertrophy, lymphedema and obesity to provide the patient with adequate treatment options. The exact etiology of lipedema is still unknown, although recent research has suggested the involvement of several genetic factors. Because lipedema is often not recognized or misdiagnosed, the scarcely available prevalence figures are likely a significant underestimation. All of the current treatment options are non-curative, complex and require a multidisciplinary approach. Surgical treatment of the affected limbs occurs when conservative treatment options are no longer effective and daily functioning becomes severely compromised.

Lipedema is a chronic fat disorder with disproportional fat distribution, especially around the legs, hips, and sometimes arms, and it affects women almost exclusively. The major symptoms and complaints include pain in the soft tissue and the feeling of “heavy legs.” The perception of pain depends to a high degree on the psychological condition of the patient. Obesity is the major comorbidity and can worsen lipedema. In combination with impaired levels of physical activity, there is an increase in interstitial filtration, and noninfectious inflammation with capillary leakage can occur. Eventually, chronic edema develops due to the dynamic insufficiency of the lymphatic system. However, edema is not a pathognomonic aspect of lipedema. There are many controversies and myths about the condition lipedema, and no objective diagnostic tool is available to confirm the diagnosis. Therapeutic approaches in lipedema are multimodal and focus on the biomedical, psychological, and functional aspects involved with the disorder. A lifestyle change is often part of the therapeutic program, along with other therapeutic interventions, such as exercise, compression, weight management, and nutritional and psychological support. Clinimetrics with validated techniques in all fields of human functioning of a lipedema patient are mandatory to objectively evaluate the improvements that are due to the treatment program. Liposuction is a technique to remove fat and is therefore introduced to treat lipedema. Some doctors who offer the procedure state that “liposuction leads to comprehensive and long-term improvements in lipedema and cures the disease.” These statements are often too optimistic and lead to high costs for the patient and disappointment in the end. However, liposuction can lead to improvements in lipedema when offered within a therapeutic program that includes other nonoperative modalities for the correct selection of patients according to their medical criteria. It cannot be emphasized enough that liposuction is not a method that should be used to treat obesity.

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.

Lipoedema is a disorder of adipose tissue that occurs almost exclusively in women; the pathophysiology and aetiology are yet not well understood (Wold et al, 1951; Child et al, 2010; Fife et al, 2010). The condition was originally described in 1943 by Allen and Hines (Wold et al, 1951). The exact prevalence of lipoedema in women is unknown; its presence in the general female population has been estimated at 11% (Földi and Földi, 2012). It is a chronic, progressive condition that is associated with considerable morbidity, including discomfort, easy bruising and tenderness of the disproportionately enlarged legs, which may progress to highintensity pain and limited mobility, along planus, and complaints about general fatigue and physical impairment are often observed. In later stages, body mass index (BMI) ≥30 kg/m2 (obesity) may also develop. Clinical characteristics of lipoedema include swelling and symmetrical enlargement of the lower limbs due to abnormal deposition of subcutaneous fat, with a sharp transition area of affected to unaffected tissue occasionally accompanied by over-hanging lipoedema tissue (Box 1). This is recognised as the typical ‘cuff-sign’, also called as ‘inverse shouldering’ or the ‘bracelet effect’. Lipoedema often co-exists with obesity, and obesity may be misdiagnosed, although Abstract