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Figure 4. Asymmetrical limb swelling in a lymphedema patient with her left lower limb affected

Figure 5. Stemmer sign. It is negative in Lipedema patients. Here you can lift the skin of the dorsum of the second toe, which is not
possible in Lymphedema patients (positive Stemmer sign)

Madelung’s Disease, also known as benign symmetrical lipomatosis and Launois-Bensaude disease, is a
disorder of fat metabolism that results in an unusual accumulation of fat deposits around the neck (Type I),
shoulder areas and upper arms (Type II), or pelvic areas (Type III). The condition is most common in men
[24]
and almost always associated with alcohol abuse and liver damage [Figure 6].
Lipedema is frequently misdiagnosed as obesity. While obesity affects the whole body, lipedema usually
affects the upper and/or lower limbs and does not involve the feet and hands. Patients with lipedema
[25]
hardly respond to restricted diet . Even after extreme weight loss, for example after bariatric surgery
or in cancer cachexia, patients typically lose less fat in the areas affected by lipedema than in the non-
[26]
affected areas [Figure 7]. Lipedema and obesity share the hallmark of symmetrical fat increase . However,
differential diagnostic criteria include the different distribution of fat in obesity (which is typically more
in the “central” pattern) and that the fat is not usually tender/painful. Weight loss by dieting and exercise
in chronic lipedema patients can often be frustrating, because there is less fat reduction in the affected
limbs than on the trunk. Furthermore, weight gain can result in excess fat deposition in the legs. Thus, in
women with lipedema who cannot sustain weight loss, there is a risk of progression of the lipedema. This
seems to be more likely in women who experience periods of weight loss followed by periods of weight
gain. Management of any associated obesity is therefore crucial to the successful management of lipedema.
[27]
Bariatric surgery may be a successful way of reducing weight and maintaining the achieved loss .

A recent review highlights the utility of developing a genetic diagnostic test containing candidate genes for
lipedema and causative genes of diseases that can be confused with lipedema, to help differentiate lipedema