Reply To: Lipedema

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Lilia Graue, MDLilia Graue, MD

Below is a background summary from an ongoing clinical trial. Full paper here, also downloadable for free in pdf:

Lipedema was first described in 1940 by Allen and Hines [1] and has a registered diagnosis in the International Classification of Diseases [2] (ICD-10_GM Version 2020) coded stage-specifically (ICD10-E88.20, E88.21, E88.22, and E88.28). This disease, almost exclusively affecting females, is characterised by a symmetrical bilateral increase of the adipose tissue in the upper and/or lower extremities. It is unresponsive to physical activity or dietary intervention [3, 4]. A large proportion of the patients also shows the involvement of the arms [5]. Lipedema is associated with pain of the adipose tissue, increased touch sensitivity, feeling of tension in the affected regions and an increased tendency to haematoma after minor traumas [6]. Data on the prevalence of lipedema is limited. The prevalence of lipedema may be between 10 and 18% among women [7,8,9]. Child et al. estimated the prevalence within the female population as 1 in 72,000 [3]. However, lipedema is often misdiagnosed as obesity or lymphedema and therefore is incorrectly treated [3, 5]. Shivat et al. have established lipedema diagnostic algorithm to guide clinicians in making the correct diagnosis [10].

The aetiology of lipedema is unknown, but a genetic disposition is likely since lipedema is often seen in patients with a family history of this disease. X-linked dominant or gender-limiting autosomal dominant inheritance is assumed [3]. An important factor in the pathophysiology of lipedema is the increased capillary permeability, which leads to orthostatic edema and seems to be responsible for the increased tissue sensitivity in response to touch and pressure. Increased capillary fragility could explain the tendency towards haematoma formation [11].

The course of the disease is individual: some patients develop lipedema at a lower degree of severity and with stabilisation. Other patients show progression after stressful situations like pregnancy or surgery [12]. The onset of lipedema is common in teenage years, but is also observed after pregnancy or even menopause [3, 13].

Lipedema is divided into three stages according to skin morphology and palpation: stage I is characterised by smooth skin surface, thickened subcutis and small-nosed fat structure. In stage II, nodules appear in the fatty tissue and the skin surface is uneven, while in stage III, the tissue is hardened and rough with the presence of large deforming fat flaps [4].

The comprehensive decongestive therapy (CDT) is part of the standard conservative treatment [6]. Originally developed for the treatment of lymphedema, improvement has also been seen in patients with lipedema (reduction in leg circumference, tension, and pressure pain). As the treatment success is short-term, therapy is permanently necessary [4, 12].

CDT consists of a combination of lymphatic drainage, compression therapy (usually flat-knitted compression stockings), skin care and physical activity. It is unclear whether CDT influences the progression of pain in addition to the abovementioned benefits. However, the specific fat accumulation and distribution with the accompanying pain and tendency to form haematomas remain [5]. Physical activity, if necessary in combination with a dietary intervention, aims to reduce or prevent obesity, which has a negative prognostic influence on the development of lipedema [6, 10].

Starting in the 1990s, liposuction has emerged as the most important surgical intervention for lipedema. The pathologically altered subcutaneous fatty tissue is surgically removed without damaging the lymphatic system. Liposuction methods are tumescent anaesthesia (TA) and water-assisted liposuction (WAL). In TA, several litres of an anaesthetic solution are infused into the subcutaneous area (‘wet technique’) so that the fat cells swell and vessels constrict. Then, the subcutaneous fat is suctioned off by a very fine, blunt, vibrating cannula (power/vibration-assisted liposuction, PAL). In WAL, a jet of water releases the fat cells from the connective tissue and a mixture of fat cells, water and tumescent solution is aspirated [14,15,16]. This method is a tissue-friendly surgical method and minimises the risk of injuring vascular, lymphatic and nerve structures [4, 14, 17,18,19]. TA is standard for surgical treatment nowadays and recommended as the method of choice in the guidelines for lipedema [6].

Liposuction leads to reduction of lipedema-specific fat accumulation and distribution, pain sensitivity, edema, tendency to haematoma and restriction of movement [4, 19]. An improvement in the quality of life and a decrease in the need for conservative therapy has been shown [4, 11, 20,21,22].

In summary, clinical experiences as well as small trials demonstrate a positive effect of liposuction in lipedema with respect to pain, movement improvement and quality of life. However, controlled and randomised clinical trials comparing conservative and surgical treatments with valid statistical methods are missing. There is no evidence that surgical treatment is equivalent or even superior to conservative treatment with respect to pain in lipedema stages I, II and III. Therefore, a large-scale randomised controlled investigator-blinded trial will be performed with the aim to provide a definitive answer.

Liposuction is therefore discussed as a useful addition to or even alternative therapy for CDT, especially in the early stages of lipedema in order to prevent possible long-term effects such as the development of secondary lymphedema and disease progression, to improve the quality of life and to reduce the need for CDT [4, 11, 20,21,22].