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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.