Lipoedema is not lymphoedema: A review of current literature
Eran Shavit, Uwe Wollina, Afsaneh Alavi, Int Wound J. 2018;1–8
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Lipoedema is not lymphoedema: A review of current literature
Eran Shavit, Uwe Wollina, Afsaneh Alavi, Int Wound J. 2018;1–8
Lipoedema is a rare painful disorder of the adipose tissue. It essentially affects females and is often misdiagnosed as lymphoedema or obesity. It is globally misdiagnosed or underdiagnosed, and the literature is lacking appropriate guidance to assist clinicians towards this diagnosis. However, the need to recognise this disorder as a unique entity has important implications to establish proper treatment and, therefore, its tremendous effect on patients. Early diagnosis and treatment can turn these patients’ lives upside down. The aim of this review is to focus on the clinical guidance, differential diagnosis, and management strategies. In addition, other aspects of lipoedema, including epidemiology and pathogenesis, are also being discussed here. Lipoedema is distinct from obesity and distinct from lymphoedema, although it might progress to involve the venous and lymphatic system (venolipedema or lympholipedema or both). Late diagnosis can leave the patient debilitated. Management of lipoedema includes weight loss, control of oedema, complex decongestive physiotherapy, liposuction, and laser-assisted lipolysis. However; there are increasing reports on tumescent liposuction as the preferred surgical option with long-lasting results. The role of more randomised controlled studies to further explore the management of this clinical entity remains promising
Main findings
- The paper suggests a number of key messages:
- lipoedema is a rare painful disorder of the fatty tissue that affects women after puberty and is often misdiagnosed or underdiagnosed
- lipoedema is a distinct entity that must be differentiated from obesity and lymphedema, although it may progress to involve the venous and lymphatic systems, which increases the difficulty of its diagnosis
- the diagnosis of lipoedema is clinical, and it is essential to recognise this unique entity in order to establish proper treatment
- the literature is lacking an appropriate guidance towards this diagnosis; therefore, we have provided in this article an algorithm to guide clinicians to diagnose lipoedema
- the management of lipoedema includes conservative and surgical approaches; in case the former fails and the latter is required, tumescent liposuction is marked as the preferred option with long-lasting results
- The exact genes involved in lipoedema have not been identified.
- Lipoedema is encountered exclusively in female patients, during or following puberty, during the second to the third decades of life. However, there have been reports of onset after pregnancy or even menopause.
- Most patients with lipoedema have a high BMI, which can be either because they are overweight or obese; however, many of these patients have a normal appearance above their waist, accounting for a disfigurement between their lower and upper extremities, and this might falsely elevate their BMI levels.
- A positive family history suggesting lipoedema is linked to a genetic component has been described in up to 60% of cases.
- The possible mechanism leading to the development of lipoedema may involve increased adipogenesis, leading to hypoxia, further adipocyte necrosis, and macrophage recruitment. However, in a recent study, proteins from adipose tissue of lipoedema patients were harvested and did not indicate tyrosine-phosphorylated proteins in lipoedema tissue and controls. These results suggest the absence of activated growth factor receptors in the pathways of adipogenesis of the lipoedema patient. Another interesting finding was that stromal vascular fraction cells (CD90+, CD146+) were significantly enhanced in lipoedematous adipose tissue compared with normal adipose tissue. On the other hand, the adipogenic differentiation potential of these cells was significantly reduced when compared with healthy controls.
- The diagnosis of lipoedema is clinical and mainly relies only on history and physical examination. Even though lipoedema is commonly mistaken to be obesity and lymphoedema, striking clinical features, sparing feet and hands, filling of retromalleolar sulci with fat, and pressure sensitivity of the below-knee medial fat pad is helpful for diagnosis when clinicians are familiar with the disease.
- Lipoedema is always bilateral and non-pitting, and other diagnoses that might cause bilateral lower limb with pitting oedema include early lipodermatosclerosis, dependent oedema, limb swelling because of internal (such as thyroid, heart, kidney) diseases, and oedema because of certain medications.
- Lipoedema typically presents in women bilaterally and symmetrically with a sharp demarcation at the ankle, referred to as the “cuff sign” or “reverse shouldering”.
- The goal of therapy is to improve symptoms and prevent secondary complications, particularly to reduce pain and decrease the bulk of fat deposition.
- Conservative treatment should be employed to control associated oedema, and options include: combined decongestive therapy, manual lymphatic drainage, compression garments or bandages, and mobilisation.
- Compression therapy is a challenge in these patients because of lower leg tenderness. The better outcome is associated with the introduction of compression therapy to these patients at the early stage of the disease.
- A good support measure can include manual lymphatic drainage and intermittent pneumatic compression if the pneumatic compression has at least 6 to 12 chambers.
- The role of diet is much more relevant in the case of combined obesity.
- The largest study to date has demonstrated tumescent liposuction to be an effective option.This procedure is associated with a pronounced improvement in pain, tenderness to pressure, oedema, and easy bruising.
