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Dr. Allen and Dr. Hines pioneered and first described lipedema in the 1940s, a common subcutaneous adipose tissue disorder characterized as enlargement of both lower extremities. Lipedema is not edema; it is a genetically determined disturbance in adipose tissue mass and adipose tissue distribution.[1][2][3][4] In 1951 a second seminal paper provided more description of lipedema. Fat distribution involves lower extremities, upper arms, hips, buttocks, thighs sparing trunks, and feet.[2] Lower extremities are characterized by pain, easy bruisability, firm subcutaneous nodules of adipose tissue, and resistance of fat to traditional diet and exercise.[5][6]
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Lipomas are defined as a common subcutaneous tumor composed of adipose (fat) cells, often encapsulated by a thin layer of fibrous tissue.[1] In fact, these are the frequently encountered neoplasms by the clinicians. [2] Clinically, they often present in the body's cephalic part, specifically in the head, neck, shoulders, and backs of patients. However, they can less commonly be seen elsewhere, for example, the thighs. The tumors typically lie in the subcutaneous tissues of patients. The masses are often benign, and while the age of onset can vary. There is usually no reason for treatment. They pose no threat to the patient unless they are uncomfortable due to being located on joints or rapidly growing, which is uncommon, as the typical lipoma growth is slow. Lipomas can sometimes, though rare, be associated with certain disorders such as multiple hereditary lipomatosis, Gardner syndrome, adiposis dolorosa, and Madelung disease.[3] [4] Some unconventional forms of lipomas include the following: angiolipoma, chondroid lipoma, lipoblastoma, myolipoma, pleomorphic lipoma/spindle cell lipoma, intramuscular and intermuscular lipoma, lipomatosis of nerve, lipoma of the tendon sheath and joint, lipoma arborescens, multiple symmetric lipomatosis, diffuse lipomatosis, adiposis dolorosa, and hibernoma.
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Lipedema is a painful fat disease of loose connective tissue usually misdiagnosed as lifestyle-induced obesity that affects ~10% of women of European descent as well as other populations. Lipedema is characterized by symmetric enlargement of the buttocks, hips, and legs due to increased loose connective tissue; arms are also affected in 80% of patients. Lipedema loose connective tissue is characterized by hypertrophic adipocytes, inflammatory cells, and dilated leaky blood and lymphatic vessels. Altered fluid flux through the tissue causes accumulation of fluid, protein, and other constituents in the interstitium resulting in recruitment of inflammatory cells, which in turn stimulates fibrosis and results in difficulty in weight loss. Inflammation and excess interstitial substance may also activate nerve fibers instigating the painful lipedema fat tissue. More research is needed to characterize lipedema loose connective tissue structure in depth, as well as the form and function of blood and lymphatic vessels. Understanding the pathophysiology of the disease will allow healthcare providers to diagnose the disease and develop treatments.
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Subcutaneous adipose tissue diseases involving adipose tissue and its fascia, also known as adipofascial disorders, represent variations in the spectrum of obesity. The adipofascia diseases discussed in this chapter can be localized or generalized and include a common disorder primarily affecting women, lipedema, and four rare diseases, familial multiple lipomatosis, angiolipomatosis, Dercum disease, and multiple symmetric lipomatosis. The fat in adipofascial disorders is difficult to lose by standard weight loss approaches, including lifestyle (diet, exercise), pharmacologic therapy, and even bariatric surgery, due in part to tissue fibrosis. In the management of obesity, healthcare providers should be aware of this difficulty and be able to provide appropriate counseling and care of these conditions. Endocrinologists and primary care providers alike will encounter these conditions and should consider their occurrence during workup for bariatric surgery or hypothyroidism (lipedema) and in those that manifest, or are referred for, dyslipidemia or diabetes (Dercum disease). People with angiolipomas should be worked up for Cowden’s disease where a mutation in the gene PTEN increases their risk for thyroid and breast cancer. This chapter provides details on the pathophysiology, prevalence, genetics and treatments for these adipofascial disorders along with recommendations for the care of people with these diseases. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.
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« La parenté de l'ædeme chronique avec la lipomatose a depuis long lemps frappé les auteurs » ( 1 ) . Le cas actuel que nous publions aujourd'hui, après l'avoir présenté en quelques mots à la Société de Neurologie ( 2) , vient à l'appui de celle rollesion Ichard et L. Lévi. OBSERVATION ( PI. LXV ). Il s'agit d'une brodeuse de 39 ans, Mlle Eugénie B ... 1, venue à notre consul tation de l'hôpital Laënnec pour grosseur anormale des membres inférieurs . Antécédenis héréditaires -- La mère est morte à 25 ans, de tuberculose aiguë (alcoolisme possible). Son père est mort à 56 ans, très probablement d'un cancer du pylore. Eugénie B ... a une scur âgée de 41 ans, bien portante. Une de ses tantes aurait eu , pendant longtemps, de la difficulté à marcher . Antécédents personnels. -- Mile B ... n'a jamais été malade. Elle se souvient que, vers l'âge de 8 ans, elle avait déjà de gros mollets qui faisaient l'admira tion de ses camarades ; néanmoins, en promenade, elle se fatiguait plus vite que ses compagnes. Elle n'a jamais ressenti de douleurs vives dans les mem bres inférieurs. A 14 ans ses règles apparaissent régulières, avec un peu de dysmenorrhée. A 22 ans,, elle s'aperçoit que, sans aucun doute, ses jambes grossissent et non le reste du corps . L'ordème aurait envahi, par étapes, les jambes, les cuisses et les fesses . Elle est examinée et traitée à Lille , à Amiens et à Paris. A Lille elle prend de la tyroïdine ( 2 pilules par jour) , et de l'iode , pendant 15 jours . Elle maigrit sous l'influence de ce traitement.
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