Lipedema, a Rare Disease

Abstract

Lipedema is a chronic disease of lipid metabolism that results in the symmetrical impairment of fatty tissue distribution and storage combined with the hyperplasia of individual fat cells. Lipedema occurs almost exclusively in women and is usually associated with a family history and characteristic features. It can be diagnosed based on clinical history and physical examination. Lipedema is usually symmetrical, but spares the feet, is often painful to palpation, and is negative for Stemmer's sign. Additionally, lipedema patients can present with microangiopathies and lipomas. The well-known therapies for lipedema include complex decongestive therapy, pneumatic compression, and diet modifications. However, whether these treatments help reduce swelling is debatable. We encountered a case of lipedema that was initially misdiagnosed as lymphedema. The patient's clinical features and history were different from those typical of lymphedema, prompting a diagnosis of lipedema and she was treated with a complex decongestive therapy program.

Pathophysiological dilemmas of lipedema

“…the inflammation of sensory nerves derived from sympathetic nerve fibers distributing among adipocytes. In case if microangiopathy also exists, disturbance of protopathic sensibility may be aggravated due to the inappropriate blood supply of the peripheral nerves [70]. Taking into consideration that accumulation of subcutaneous adipose tissue is also involved in the pathogenesis of lipohypertrophy, which lacks both edema and pain [64], mechanical stimulus by itself fails to explain the neurological background.…”

“…As a part of the diagnostic criteria (Table 1), the affected subcutaneous regions are often tender and painful, which may cause difficulties in the application of bandaging, however, this pain is improved after mild manual lymphatic drainage [70] and liposuction [64].…”

The Lymphatic Vasculature: Its Role in Adipose Metabolism and Obesity

“…They described that the deposition of fat developed frequently after puberty, progressed gradually, and was aggravated by activity and warm temperatures (Lohrmann et al, 2009). Lipedema is found nearly exclusively in females and probably caused by a genetic condition (Bilancini et al, 1995; Okhovat and Alavi, 2015; Shin et al, 2011; Wagner, 2011). Lipedema is often misdiagnosed as obesity or lymphedema (Bilancini et al, 1995; Lohrmann et al, 2009).…”

Restoration of lymphatic function rescues obesity in Prox1-haploinsufficient mice

“…Additionally, an abnormal lymphoscintigraphic pattern, with a slowing of lymphatic flow, has been reported in patients suffering from lipedema, a chronic disease of lipid metabolism characterized by bilateral and symmetric lower-extremity fat deposition (19). Moreover, in sustained cases of lipedema, the lymph vessels are unable to function, and lymph fluid accumulates (lympholipedema) (20). …”