Lipedema: an overview of its clinical manifestations, diagnosis and treatment of the disproportional fatty deposition syndrome - systematic review.
TL;DR: Early diagnosis and treatment are mandatory for this disorder otherwise gradual enlargement of fatty deposition causes impaired mobility and further comorbidities like arthrosis and lymphatic insufficiency.
Abstract: Lipedema is a disproportionate, symmetrical fatty swelling characterized by pain and bruising existing almost exclusively among women. We undertook a systematic review of the available literature about lipedema, given the lack of knowledge and little evidence about this disorder especially among obesity experts. Diagnosis of lipedema is usually based on clinical features. Symmetrical edema in the lower limbs with fatty deposits located to hips and thighs usually appears at puberty and often affects several members of the same family. Main disorders considered for differential diagnosis are lymphedema, obesity, lipohypertrophy and phlebedema. Treatment protocols comprise conservative (decongestive lymphatic therapy) and surgical (liposuction) approaches. Early diagnosis and treatment are mandatory for this disorder otherwise gradual enlargement of fatty deposition causes impaired mobility and further comorbidities like arthrosis and lymphatic insufficiency.
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01 Aug 2011
TL;DR: The use of “red flags” to guide decisions about the need for further investigation of suspected vertebral fracture in patients presenting with low-back pain is unfounded, and an updated review provides mixed relief for people with diabetes suffering from foot ulcers.
Abstract: The use of “red flags” (specific information from the clinical history and examination used as sentinels of disease) to guide decisions about the need for further investigation of suspected vertebral fracture in patients presenting with low-back pain is unfounded. That’s the discouraging conclusion of the latest diagnostic test accuracy review. The authors looked at eight studies and found that many red flags have high falsepositive rates. The limited evidence makes recommending specific red flags a challenge, but three (older age, significant trauma and corticosteroid use) were more promising, and combinations of red flags were more informative than individual tests (doi: 10.1002/14651858.CD008643.pub2). The primary prevention of cardiovascular disease comes under the spotlight in one new and one updated review. Selenium supplementation is aggressively marketed in some quarters, but the evidence from a new review of 12 studies involving nearly 20 000 participants does not support its use, especially in populations with adequate selenium status. Furthermore, the authors are cautious not to rule out the possibility of an increased risk of type 2 diabetes from taking selenium-only supplements (doi: 10.1002/14651858.CD009671. pub2). More definitive is the evidence to support the use of statins in people at low risk of cardiovascular disease. This comes from an updated review that now includes data on 57 000 participants from 18 randomised trials. Statins reduced major vascular events as well as all-cause mortality. Added bonuses were the absence of serious harms and the likelihood that statins are cost-effective (doi: 10.1002/14651858.CD004816.pub5). An updated review provides mixed relief for people with diabetes suffering from foot ulcers. Non-removable, pressure-relieving casts heal ulcers more effectively than removable casts or dressings alone, but this comes at the price of restricted movement and a possible decrease in quality of life (doi: 10.1002/14651858.CD002302.pub2). Hiccups are a passing nuisance for most of us, but spare a thought for the few who suffer from persistent or intractable hiccups (defined as lasting for more than 48 hours and more than 1 month, respectively). Unfortunately, there’s little relief in sight, since the four poor-quality trials reviewed all investigated treatment with acupuncture and showed that it may be no better than placebo (doi: 10.1002/14651858.CD008768.pub2). Find these and other new and updated reviews this month in The Cochrane Library at www.thecochranelibrary.com. From The Cochrane Library
123 citations
Journal Article•
Georgetown University1, University of São Paulo2, University of California, San Diego3, Katholieke Universiteit Leuven4, Mayo Clinic5, George Washington University6, Flinders University7, St George's, University of London8, University of Bonn9, Stanford University10, The Catholic University of America11, University of Szeged12, Uniformed Services University of the Health Sciences13
TL;DR: Treatment in the maintenance phase should include compression garments, self-management, including self-massage, meticulous personal hygiene and skin care, in addition to lymphtransport-promoting excercises and activities, and, if desired, pneumatic compression therapy applied in the home.
Abstract: Primary lymphedema can be managed effectively as a form of chronic lymphedema by a sequenced and targeted treatment and management program based around a combination of Decongestive Lymphatic Therapy (DLT) with compression therapy, when the latter is desired as an adjunct to DLT. Treatment in the maintenance phase should include compression garments, self-management, including self-massage, meticulous personal hygiene and skin care, in addition to lymphtransport-promoting excercises and activities, and, if desired, pneumatic compression therapy applied in the home. When conservative treatment fails, or gives sub-optimal outcomes, the management of primary lymphedema can be improved, where appropriate, with the proper addition of surgical interventions, either reconstructive or ablative. These two surgical therapies can be more effective when fully integrated with manual lymphatic drainage (MLD)-based DLT postoperatively. Compliance with a long-term commitment to MLD/DLT and particularly compression postoperatively is a critical factor in determining the success of any new treatment strategy involving either reconstructive or palliative surgery. The future of management of primary lymphedema has never been brighter with the new prospect of gene-and perhaps stem-cell oriented management.
103 citations
TL;DR: According to the hypothesis, lipedema is an estrogen-regulated polygenetic disease, which manifests in parallel with feminine hormonal changes and leads to vasculo- and lymphangiopathy and is likely to be a secondary phenomenon maintaining a vicious cycle.
76 citations
Cites background from "Lipedema: an overview of its clinic..."
...Decongestive lymphatic therapy including manual lymphatic drainage, physical exercise, multilayered and multicomponent compression bandaging and meticulous skin care is the standard conservative approach for lipedema treatment [5]....
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...8% of the patients in their unit with enlargement of the lower limbs between 2005 and 2012 [5]....
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TL;DR: Ledema is a painful psychologically distressing fat disorder, more foe than friend especially due to associated obesity and lymphedema, and more controlled studies are needed to study the mechanisms and treatments for lipedema.
Abstract: Background Lipedema is a chronic disorder presenting in women during puberty or other times of hormonal change such as childbirth or menopause, characterized by symmetric enlargement of nodular, painful subcutaneous adipose tissue (fat) in the limbs, sparing the hands, feet and trunk. Healthcare providers underdiagnose or misdiagnose lipedema as obesity or lymphedema. Materials and methods The benefits (friend) and negative aspects (foe) of lipedema were collected from published literature, discussions with women with lipedema, and institutional review board approved evaluation of medical charts of 46 women with lipedema. Results Lipedema is a foe because lifestyle change does not reduce lipedema fat, the fat is painful, can become obese, causes gait and joint abnormalities, fatigue, lymphedema and psychosocial distress. Hypermobility associated with lipedema can exacerbate joint disease and aortic disease. In contrast, lipedema fat can be a friend as it is associated with relative reductions in obesity-related metabolic dysfunction. In new data collected, lipedema was associated with a low risk of diabetes (2%), dyslipidemia (11.7%) and hypertension (13%) despite an obese average body mass index (BMI) of 35.3 ± 1.7 kg/m2. Conclusion Lipedema is a painful psychologically distressing fat disorder, more foe than friend especially due to associated obesity and lymphedema. More controlled studies are needed to study the mechanisms and treatments for lipedema.
66 citations
Additional excerpts
...8% in their patients [1], [11], [12], [13], [14], [15], [16], [17]....
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TL;DR: The present, revised guidelines on lipedema were developed under the auspices of and funded by the German Society of Phlebology (DGP) and contain recommendations with respect to diagnosis and management oflipedema.
Abstract: Summary
The present, revised guidelines on lipedema were developed under the auspices of and funded by the German Society of Phlebology (DGP). The recommendations are based on a systematic literature search and the consensus of eight medical societies and working groups. The guidelines contain recommendations with respect to diagnosis and management of lipedema.
The diagnosis is established on the basis of medical history and clinical findings. Characteristically, there is a localized, symmetrical increase in subcutaneous adipose tissue in arms and legs that is in marked disproportion to the trunk. Other findings include edema, easy bruising, and increased tenderness. Further diagnostic tests are usually reserved for special cases that require additional workup. Lipedema is a chronic, progressive disorder marked by the individual variability and unpredictability of its clinical course.
Treatment consists of four therapeutic mainstays that should be combined as necessary and address current clinical symptoms: complex physical therapy (manual lymphatic drainage, compression therapy, exercise therapy, and skin care), liposuction and plastic surgery, diet, and physical activity, as well as psychotherapy if necessary. Surgical procedures are indicated if – despite thorough conservative treatment – symptoms persist, or if there is progression of clinical findings and/or symptoms. If present, morbid obesity should be therapeutically addressed prior to liposuction.
66 citations
References
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TL;DR: The survey results suggest AD is a painful syndrome involving growths in all subcutaneous fat, which can be inherited or sporadic, beginning in the third decade of life, but can occur in children and is accompanied by obesity, fatigue, joint and muscle aches, and other signs and symptoms affecting multiple organ systems.
Abstract: mass index of 34.7 8.7 kg/m 2 and had AD for 14.5 11.9 years. Onset of growths began primarily in the arm or leg at a mean age of 34.5 12.8 years (range, 7– 67 years). The majority reported pain, fatigue, weight gain (25.7 15 kg), multiple growths, and weakness at onset. The median number of palpable growths was 77.3 84.5 in subcutaneous fat from scalp to dorsal foot. Growths ranged from firm and pea-sized to large, fixed, and deep. Pathology descriptions of growths included lipoma, fibrolipoma, or angiolipomas. Autoimmune disease and fibromyalgia were prominent (38.2%), as was diabetes (16.4%). Almost all respondents reported exercise-associated pain in growths, and 100% saw no decrease in growths with weight loss. Over 75% had liposuction and over half reported regrowth. Almost half reported family members with growths. Conclusion: Our survey results suggest AD is a painful syndrome involving growths in all subcutaneous fat. It can be inherited or sporadic, beginning in the third decade of life, but can occur in children and is accompanied by obesity, fatigue, joint and muscle aches, and other signs and symptoms affecting multiple organ systems. Exercise induces pain and has no effect on fatty growth size. The etiology of this syndrome remains unknown.
36 citations
"Lipedema: an overview of its clinic..." refers background in this paper
...The most common locations of painful fatty depositions are the limbs, trunk, the pelvic area and the buttocks (27,28)....
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TL;DR: Post-pubertal women with idiopathic edema are most evident in the feet or abdomen after prolonged standing or sitting and in the fingers and eyelids after recumbency overnight.
36 citations
"Lipedema: an overview of its clinic..." refers background in this paper
...Based on the result of Streeten test together with clinical features, the diagnosis of lipedema can be suspected (34,35)....
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TL;DR: Die lymphatische Insuffizienz konnte bei dem fast ausschlieslich bei Frauen auftretenden Lipodem patho-physiologisch eine bedeutende Rolle spielen einen normalen Befund hinsichtlich der visuellen Bewertung and des quantitativen Wertes.
Abstract: Ziel: Die lymphatische Insuffizienz konnte bei dem fast ausschlieslich bei Frauen auftretenden Lipodem patho-physiologisch eine bedeutende Rolle spielen. Uber die Pathophysiologie dieser abnorm lokalisierten Korperfett-ablagerungen ist wenig bekannt. Untersucht wurde die Beteiligung des Lymphsystems bei Lipodem vom Typ Allen-Hines sowie bei Typus Rusticanus-Moncorps. Patienten, Methoden: Bei 28 Patienten mit Lipodem wurden die Standard-Lymphszintigraphie (epifaszial) und eine modifizierte Lymphszintigraphie (subkutan) durchgefuhrt. Die prozentualen Uptakes in Relation zur injizierten Dosis dienten als funktionale quantitative Parameter. Beide Studien wurden visuell bewertet. Patienten mit Odemen der Beine infolge Veneninsuffizi-enz (Stadium II nach Widmer) dienten als Kontrollgrup-pe. Ergebnisse: Die Standard-Lymphszintigraphie zeigte bei allen Patienten der Kontrollgruppe sowie allen Pati-enten mit Lipodem vom Typ Rusticanus-Moncorps einen normalen Befund hinsichtlich der visuellen Bewertung und des quantitativen Wertes. Der Lymphtransport aus dem subkutanen Fettgewebe war im Patientenkollektiv mit Lipodem vom Typ Allen-Hines signifikant hoher (p
31 citations
"Lipedema: an overview of its clinic..." refers background in this paper
...The enlargement of the lower limbs is disproportionate in relation to the upper part of the body (18)....
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TL;DR: The case shows a novel association of two rare conditions Pit-1 mutation and lipoedema in a family that has not been described before and allows formulation of hypothesis on the interaction of growth hormone and sex steroids resulting in abnormal fat distribution in predisposed subjects at the time of puberty.
Abstract: Background A 23-year-old male was referred to our clinic with diagnosis of idiopathic isolated growth hormone deficiency. A detailed family history revealed short stature and swelling of legs which only affected females in four generations of his family. Methods Combined pituitary function tests revealed growth hormone deficiency, secondary hypothyroidism and hypoprolactinemia in the proband. His mother had hypoprolactinemia and growth hormone deficiency. A diagnosis of inherited combined pituitary deficiency due to a PIT-1 mutation was suspected in view of the short stature with associated multiple pituitary hormone deficiencies. Results A mutation was identified in PIT-1 (POU1F1), 196C>T, which produces the amino acid change P24L in exon 1. The mutation was also found in the mother of the proband but not in his phenotypically normal half-sister. Conclusion The case shows a novel association of two rare conditions Pit-1 mutation and lipoedema in a family that has not been described before. It also allows formulation of hypothesis on the interaction of growth hormone and sex steroids resulting in abnormal fat distribution in predisposed subjects at the time of puberty.
26 citations
"Lipedema: an overview of its clinic..." refers background in this paper
...have recently found a Pit-1 mutation in members of a family with lipedema (47) and screening of lipedema family members strongly suggests inheritance (22)....
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23 citations