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BACKGROUND: Lipedema is a rare female disorder with a characteristic distribution of adipose tissue hypertrophy on the extremities, with pain and bruising. In advanced stages, reduction of adipose tissue is the only available effective treatment. In elderly patients with advanced lipedema, correction of increased skin laxity has to be considered for an optimal outcome. METHODS: We report on a tailored combined approach to improve advanced lipedema in elderly females with multiple comorbidities. Microcannular laser-assisted liposuction of the upper legs and knees is performed under tumescent anesthesia. Medial thigh lift and partial lower abdominoplasty with minimal undermining are used to correct skin laxity and prevent intertrigo. Postsurgical care with nonelastic flat knitted compression garments and manual lymph drainage are used. RESULTS: We report on three women aged 55-77 years with advanced lipedema of the legs and multiple comorbidities. Using this step-by-step approach, a short operation time and early mobilization were possible. Minor adverse effects were temporary methemoglobinemia after tumescent anesthesia and postsurgical pain. No severe adverse effects were seen. Patient satisfaction was high. CONCLUSION: A tailored approach may be useful in advanced lipedema and is applicable even in elderly patients with multiple comorbidities.
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OBJECTIVE: Elephantiasis nostras is a rare complication in advanced lipo-lymphedema. While lipedema can be treated by liposuction and lymphedema by decongestive lymphatic therapy, elephantiasis nostras may need debulking surgery. METHODS: We present 2 cases of advanced lipo-lymphedema complicated by elephantiasis nostras. After tumescent microcannular laser-assisted liposuction both patients underwent a debulking surgery with a modification of Auchincloss-Kim's technique. Histologic examination of the tissue specimen was performed. RESULTS: The surgical treatment was well tolerated and primary healing was uneventful. After primary wound healing and ambulation of the patients, a delayed ulceration with lymphorrhea developed. It was treated by surgical necrectomy and vacuum-assisted closure leading to complete healing. Mobility of the leg was much improved. Histologic examination revealed massive ectatic lymphatic vessels nonreactive for podoplanin. CONCLUSIONS: Debulking surgery can be an adjuvant technique for elephantiasis nostras in advanced lipo-lymphedema. Although delayed postoperative wound healing problems were observed, necrectomy and vacuum-assisted closure achieved a complete healing. Histologic data suggest that the ectatic lymphatic vessels in these patients resemble finding in podoplanin knockout mice. The findings would explain the limitations of decongestive lymphatic therapy and tumescent liposuction in such patients and their predisposition to relapsing erysipelas.
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The International Compression Club (ICC) is a partnership between academics, clinicians and industry focused upon understanding the role of compression in the management of different clinical conditions. The ICC meet regularly and from these meetings have produced a series of eight consensus publications upon topics ranging from evidence-based compression to compression trials for arm lymphoedema. All of the current consensus documents can be accessed on the ICC website (http://www.icc-compressionclub.com/index.php). In May 2011, the ICC met in Brussels during the European Wound Management Association (EWMA) annual conference. With almost 50 members in attendance, the day-long ICC meeting challenged a series of dogmas and myths that exist when considering compression therapies. In preparation for a discussion on beliefs surrounding compression, a forum was established on the ICC website where presenters were able to display a summary of their thoughts upon each dogma to be discussed during the meeting. Members of the ICC could then provide comments on each topic thereby widening the discussion to the entire membership of the ICC rather than simply those who were attending the EWMA conference. This article presents an extended report of the issues that were discussed, with each dogma covered in a separate section. The ICC discussed 12 'dogmas' with areas 1 through 7 dedicated to materials and application techniques used to apply compression with the remaining topics (8 through 12) related to the indications for using compression.
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A grossly obese woman was wrongly diagnosed throughout her adult life of having lymphoedema. Her condition was subsequently confirmed as lipoedema, an entirely different condition, which is noted in medical text books but is seldom taught to medical students or to general practitioners. The condition is caused by abnormal deposition of adipose tissue in the extremities (usually the lower limbs) and almost exclusively affects women. It often starts at puberty or may occur after pregnancy. The exact aetiology is not yet understood but genetic and hormonal factors may be implicated. The problem is that misdiagnosis leads to inappropriate tests and improper treatment to the patient. When recognised it is often too late to do anything for the patient and they become highly dependent on social care. This case describes how the diagnosis can be confirmed through an ultrasound image and illustrates the need for early recognition to facilitate specialist care.
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BACKGROUND: Functional pelvic disorders in patients undergoing conservative surgical approach for rectal cancer are considered a major public health issue and represent one third of cost of colorectal cancer. We investigated the hypothesis that lymphadenectomy, involves the pelvic floor results in a localized hides or silent pelvic lymphedema characterized by symptoms without signs. PATIENTS AND METHODS: We examined 13 colo-rectal cancer patients: five intra-peritoneal adenocarcinoma: 1 sigmoid and 4 upper third rectal cancer (1 male and 3 female) and 9 extra-peritoneal adenocarcinoma: 3 middle and 5 lower third rectal cancer (4 male and 5 female) using 1.5-T magnetic resonance, one week before and twelve months after discharged from hospital. RESULTS: Lymphedema was discovered on post-operative magnetic resonance imaging of all 9 patients with extra-pertitoneal cancer, whereas preoperative magnetic resonance imaging as well as a post-operative examination of 4 intra-peritoneal adenocarcinoma, revealed no evidence of lymphedema. Unlike the common clinical skin signs that typify all other sites of lymphedema, pelvic lymphedema is hides or silent, with no skin changes or any single symptom manifested. Magnetic resonance imaging showed that pelvic illness alone is accompanied by lymphedema related exclusively to venous congestion, and accumulation of liquid in adipose tissue or lipedema. CONCLUSIONS: Alteration of the pelvic lymphatic network during pelvic surgery can lead to lymphedema and, pelvic floor disease. Patients should be routinely examined for the possibility of developing this post-surgical syndrome and further studies are needed to establish diagnosis and to evaluate treatment preferences.
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Due to its increased presence in the press and on television, the diagnosis of lipedema is on the way to becoming a trendy diagnosis for those with thick legs. Despite this, one must recognize that lipedema is a very rare disease. It is characterized by disproportional obesity of the extremities, especially in the region of the hip and the legs, hematoma development after minimal trauma, and increased pressure-induced or spontaneous pain. Aids for making the correct diagnosis are (duplex) sonography, the waist-hip index or the waist-height index and lymphoscintigraphy. Important differential diagnoses are constitutional variability of the legs, lipohypertrophy in obesity, edema in immobility, edema in chronic venous insufficiency and rheumatic diseases. The symptom-based therapy of lipedema consists of conservative (compression, manual lymphatic drainage, exercise) and surgical treatments (liposuction). Until now there is no curative therapy. Obesity is an important risk factor for the severity and prognosis of lipedema. Further studies for a better understanding of the pathogenesis of lipedema and in the end possible curative treatments are urgently needed.
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Classification, lipoedema, obesity, oedema.
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Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.
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Background: Butcher’s broom plant extract has been reported to improve lymphatic flow and the trace mineral, selenium, has been shown to improve lymphedema. This retrospective case study examines the effectiveness of Butcher’s broom in conjunction with selenium to decrease limb volume of a patient with lipedema, a common fat distribution disorder with excess adipose tissue fluid. Methods: Selenium (400 mcg) was initiated 6 days prior to limb volume evaluation utilizing perometry. The patient underwent physical therapy that consisted of manual lymph drainage (MLD) with Histological Variable Manual Technique (HIVAMAT), and compression bandaging. Butcher’s broom (one gram daily) was added on day 95 of treatment in addition to selenium and both were continued through day 293 (end of study). Results: Total volume reduction over the study period for the left and right upper extremities and left and right lower extremities was 525 ml and 225 ml (p<0.05), and 1769 ml and 1614 ml (p<0.0001), respectively. The total percent volume reduction during the time period when MLD with HIVAMAT and compression bandaging were performed for the left and right legs was 70.6 and 79.0%, respectively. In the absence of compression bandaging, the left and right arms lost 21.2 and 10% of initial volumes, respectively at the 6 month follow-up visit. During the latter part of the study when the patient was performing a home maintenance program, at which time selenium and Butcher’s broom were continued, the left and right lower extremities decreased an additional 29.4 and 20.9% of initial volumes, respectively, despite a lack of exercise due to a foot injury during the last 46 days. Conclusion: Butcher’s broom and selenium may offer new tools in conjunction with physical therapy to improve swelling and pain associated with lipedema.
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A 54-year-old female patient reported that a characteristic of her family was 'fat legs' with postural edema since adolescence. Over the years the patient had been gaining weight with an increase in fatty tissue in the legs and arms. At the age of 24 years she started taking oral contraceptives and noted worse swelling and pain in the lower limbs. She was advised to suspend the use of the contraceptives and to start using a transdermal lymphatic system drug and physical exercise which partially improved the symptoms. Three years ago she noted that the swelling was increasing without improvement and sought a physician who raised the hypothesis of lymphedema and referred her to a specialized center. Lipedema and lymphedema was diagnosed in the physical examination. A 3-day intensive treatment program (8 h daily) was started for lymphedema which included manual and mechanical lymph drainage associated with short-strech (<50 mm Hg) compression stockings custom made using a cotton-polyester fabric. Volumetry and perimetry were performed before starting and after the treatment and the legs were photographed. Volumetric and perimetric reductions were obtained suggesting the involvement of regional cutaneous lymphostasis in this disease.
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DEFINITION AND CLINICAL PICTURE: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum's disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches. CLASSIFICATION: We suggest that Dercum's disease is classified into: I. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form - a form with pain in and around multiple lipomas IV. Juxtaarticular form - a form with solitary deposits of excess fat for example at the medial aspect of the knee. EPIDEMIOLOGY: Dercum's disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum's disease has not yet been exactly established. AETIOLOGY: Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma. DIAGNOSIS AND DIAGNOSTIC METHODS: Diagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded. DIFFERENTIAL DIAGNOSIS: Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours. GENETIC COUNSELLING: The majority of the cases of Dercum's disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum's disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition. MANAGEMENT AND TREATMENT: The following treatments have lead to some pain reduction in patients with Dercum's disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum's disease should be treated in multidisciplinary teams specialised in chronic pain. PROGNOSIS: The pain in Dercum's disease seems to be relatively constant over time.
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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.
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Liposuction is an integral part of the wide range of surgical procedures in dermatology. Dermatologists established tumescent local anesthesia in combination with the use of micro-cannulas; especially dermatologists from Germany and Austria actively designed and developed these new techniques. In this position paper, we discuss the history, various interdisciplinary aspects, the significance, and the treatment indications for this procedure as well as its role within dermatologic training programs and research. For quality reasons, members of the Germany Society of Dermatologic Surgery and the Austrian Society of Dermatologic Surgery discuss several fundamental professional aspects as well as the historical development of liposuction.
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Background: Elevated stearoyl-CoA desaturase activity has been described in obese states, with an increased desaturation index (DI) suggesting enhanced lipogenesis. Differences in the DI among various phenotypes of abnormal adiposity have not been studied. Abnormal accumulation of subcutaneous adipose tissue occurs in rare adipose disorders (RADs) including Dercum’s disease (DD), multiple symmetric lipomatosis (MSL), and familial multiple lipomatosis (FML). Examining the DI in subcutaneous fat of people with DD, MSL and FML may provide information on adipose tissue fatty acid metabolism in these disorders. The aims of this pilot study were: 1) to determine if differences in adipose tissue DIs are present among RADs, and 2) to determine if the DIs correlate to clinical or biochemical parameters. Methods: Subcutaneous adipose tissue was obtained from human participants with DD (n = 6), MSL (n = 5), FML (n = 8) and obese Controls (n = 6). Fatty acid composition was determined by gas chromatography/mass spectrometry. The DIs (palmitoleic/palmitic, oleic/stearic, vaccenic/stearic ratios) were calculated from the gas chromatogram peak intensities. SCD1 gene expression was determined. Spearman’s correlations between the DIs and available clinical or biochemical data were performed. Results: In DD subjects, the vaccenic/stearic index was lower (p < 0.05) in comparison to Controls. Percent of total of the saturated fatty acid myristic acid was higher in DD compared with Controls and FML. Percent of monounsaturated vaccenic acid in DD trended lower when compared with Controls, and was decreased in comparison to FML. In MSL, total percent of the polyunsaturated fatty acids was significantly lower than in the Control group (p < 0.05). In the total cohort of subjects, the palmitoleic/palmitic and oleic/stearic DIs positively correlated with age, BMI, and percent body fat. Conclusions: The positive associations between the DIs and measures of adiposity (BMI and percent body fat) support increased desaturase activity in obesity. The lower vaccenic/stearic DI in DD SAT compared with Controls suggests presence of other factors involved in fat accumulation in addition to lifestyle. Other mechanisms driving fat accumulation in DD such as inflammation or lymphatic dysfunction should be investigated.
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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.
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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.
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At the 3rd International Lymphoedema Framework (ILF) conference in Toronto, an attempt was made to begin to try to address this issue. Over thirty people attended a lipoedema workshop run by the author. In this workshop, I had the opportunity to talk to this international group (Canada, USA, Netherlands, Australia, UK, Germany) who shared their experiences from across the world. One of the goals of the workshop was to create an initial broad consensus about the worldwide diagnosis and management of lipoedema.The outcomes of that workshop are presented in the following report.
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The aim of this study was to evaluate the prevalence of concomitant idiopathic cyclic edema with Grade II and III cellulite. All patients treated for Grade II and III cellulite were evaluated for idiopathic cyclic edema in a retrospective, quantitative and cross-sectional study. The study was carried out at the Godoy Clinic in the period from 2006 to 2010. All patients with body mass indexes > 25, Grade I cellulite and other causes of edema were excluded. The diagnosis of idiopathic cyclic edema was based on a clinical history and fluid retention throughout the day, in particular difficulty in removing rings on waking in the morning which improves later in the day. All patients with cyclic edema were treated with 75 mg aminaphtone three times daily. Statistical analysis considered the frequency of edema. Of the 82 women evaluated with ages between 18 and 58 years old (mean of 34.9 years) 41 (50.0%) were diagnosed with idiopathic cyclic edema. Idiopathic cyclic edema is an aggravating factor for cellulite and is frequently associated with the more advanced stages of the disease. Its control is essential in the treatment of cellulite.
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