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Lipedema is a clinical entity frequently misdiagnosed or confound with primary lymphedema. Lipedema is a disorder of adipose tissue that occurs almost exclusively in obese women. It is characterized by bilateral enlargement from hip to ankle due to abnormal depositions of subcutaneous fat associated with often mild edema, usually sparing the feet. Disease onset is usually at or soon after puberty. Lipedema results in considerable frustration and distress resulting from the cosmetic appearance. Patients may complain of pain, tenderness, easy bruising of the affected areas with moderate to severe sensitivity to digital pressure or pinching. Imaging studies using computed tomography, magnetic resonance imaging, ultrasound, lymphoscintigraphy are not indicated, except if the diagnosis is atypic or doubtful. Long-term evolution may alter lymphatic system and lead to a lipo-lymphedema with specific complications such as cellulitis. Lipedema management is not codified and included weight loss (poorly improving leg appearance or discomfort), psychological counselling and compression therapy. Liposuction, especially using tumescent local anaesthesia, may reduce edema, spontaneous pain, sensitivity to pressure, bruising and improve appearance resulting in a important increase in quality of life.

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.

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.

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.

BACKGROUND: Lipoedema is a painful disease in women with circumscribed increased subcutaneous fatty tissue, oedema, pain and bruising. Whereas conservative methods with combined decongestive therapy (manual lymphatic drainage, compression garments) have been well established over the past 50years, surgical therapy with tumescent liposuction has only been used for about 10years and long-term results are unknown. OBJECTIVES: To determine the efficacy of liposuction concerning appearance (body shape) and associated complaints after a long-term period. METHODS: A total of 164 patients who had undergone conservative therapy over a period of years, were treated by liposuction under tumescent local anaesthesia with vibrating microcannulas. In a monocentric study, 112 could be re-evaluated with a standardized questionnaire after a mean of 3years and 8months (range 1year and 1month to 7years and 4months) following the initial surgery and a mean of 2years and 11months (8months to 6years and 10months) following the last surgery. RESULTS: All patients showed a distinct reduction of subcutaneous fatty tissue (average 9846mL per person) with improvement of shape and normalization of body proportions. Additionally, they reported either a marked improvement or a complete disappearance of spontaneous pain, sensitivity to pressure, oedema, bruising, restriction of movement and cosmetic impairment, resulting in a tremendous increase in quality of life; all these complaints were reduced significantly (P<0·001). Patients with lipoedema stage II and III showed better improvement compared with patients with stage I. Physical decongestive therapy could be either omitted (22·4% of cases) or continued to a much lower degree. No serious complications (wound infection rate 1·4%, bleeding rate 0·3%) were observed following surgery. CONCLUSIONS: Tumescent liposuction is a highly effective treatment for lipoedema with good morphological and functional long-term results.

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.

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.

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.

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.

Lymphedema and lipedema are chronic progressive disorders for which no causal therapy exists so far. Many general practitioners will rarely see these disorders with the consequence that diagnosis is often delayed. The pathophysiological basis is edematization of the tissues. Lymphedema involves an impairment of lymph drainage with resultant fluid build-up. Lipedema arises from an orthostatic predisposition to edema in pathologically increased subcutaneous tissue. Treatment includes complex physical decongestion by manual lymph drainage and absolutely uncompromising compression therapy whether it is by bandage in the intensive phase to reduce edema or with a flat knit compression stocking to maintain volume.

BACKGROUND: There are many causes for a large lower limb in the pediatric age group. These children are often mislabeled as having lymphedema, and incorrect diagnosis can lead to improper treatment. The purpose of this study was to determine the differential diagnosis in pediatric patients referred for lower extremity "lymphedema" and to clarify management. METHODS: The authors' Vascular Anomalies Center database was reviewed between 1999 and 2010 for patients referred with a diagnosis of lymphedema of the lower extremity. Records were studied to determine the correct cause for the enlarged extremity. Alternative diagnoses, sex, age of onset, and imaging studies were also analyzed. RESULTS: A referral diagnosis of lower extremity lymphedema was given to 170 children; however, the condition was confirmed in only 72.9 percent of patients. Forty-six children (27.1 percent) had another disorder: microcystic/macrocystic lymphatic malformation (19.6 percent), noneponymous combined vascular malformation (13.0 percent), capillary malformation (10.9 percent), Klippel-Trenaunay syndrome (10.9 percent), hemihypertrophy (8.7 percent), posttraumatic swelling (8.7 percent), Parkes Weber syndrome (6.5 percent), lipedema (6.5 percent), venous malformation (4.3 percent), rheumatologic disorder (4.3 percent), infantile hemangioma (2.2 percent), kaposiform hemangioendothelioma (2.2 percent), or lipofibromatosis (2.2 percent). Age of onset in children with lymphedema was older than in patients with another diagnosis (p = 0.027). CONCLUSIONS: "Lymphedema" is not a generic term. Approximately one-fourth of pediatric patients with a large lower extremity are misdiagnosed as having lymphedema; the most commonly confused causes are other types of vascular anomalies. History, physical examination, and often radiographic studies are required to differentiate lymphedema from other conditions to ensure the child is managed appropriately.

BACKGROUND: Lipedema is a painful, genetically induced abnormal deposition of subcutaneous fat in the extremities of almost exclusively women. The pathogenesis is unknown and no curative treatment is available. Conservative therapy consisting of lymphatic drainage and compression stockings is often recommended, but is only effective against the edema. Some patients show a short-term improvement when treated in this way. The removal of the increased fat tissue of lipedema has become possible by employing advanced liposuction techniques which utilize vibrating microcannulas under tumescent local anesthesia. The effectiveness of this approach to lipedema is the subject of this study. PATIENTS AND METHODS: 25 patients were examined before liposuction and six months thereafter. The survey included the measurement of the volume of the legs and several parameters of typical pain and discomfort. The parameters were measured using visual analogue scales (VAS, scale 0-10). RESULTS: The volume of the leg was reduced by 6.99 %. Pain, as the predominant symptom in lipedema, was significantly reduced from 7.2 ± 2.2 to 2.1 ± 2.1 (p < 0.001). Quality of life as a measure of the psychological strain caused by lipedema improved from 8.7 ± 1.7 to 3.6 ± 2.5 (p < 0.001). Other parameters also showed a significant improvement and the over-all severity score improved in all patients. CONCLUSION: Liposuction reduces the symptoms of lipedema significantly.

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.

OBJECTIVE. The objective of our study was to examine the frequency and significance of visualization of popliteal nodes during lymphoscintigraphy for the investigation of lower extremity swelling. MATERIALS AND METHODS. Technetium-99m–labeled nanocolloid was injected subcutaneously in the first web spaces of both feet of 204 consecutive patients (69 males, 135 females; age range, 11–79 years) undergoing routine, clinically indicated lymphoscintigraphy; imaging was performed 5, 45, and 150 minutes after injection. The patients were asked not to undertake any vigorous exercise between the injection and completion of imaging. RESULTS. No popliteal nodes were visualized in 29 patients in whom there was no evidence of lymphedema on clinical or lymphoscintigraphic examination (group 1). Unilateral or bilateral popliteal nodes were visualized in 10 of 39 patients (25.6%) with clinical evidence of lymphedema but normal lymphoscintigraphy findings (group 2) (p < 0.005 vs group 1). In 136 patients with clinical evidence of lymphedema and abnormal lymphoscintigraphy findings (group 3), unilateral or bilateral popliteal nodes were visualized in 59 (43.4%) (p < 0.0001 vs group 1). Popliteal nodes were visualized in 40 of 73 limbs with “dermal backflow” (54.8%) and 42 of 335 limbs without dermal backflow (12.5%) (p < 0.0001). CONCLUSION. Popliteal node visualization after subcutaneous foot web space injection is an important sign of abnormal lymphatic function in patients with clinical lymphedema of the lower extremities.

It is estimated that 3.5 million patients, both male and female, suffer from a congenital lipid distribution disorder of the arms and legs. Because these lipid distribution disorders are painful, owing to a lymphological high volume insufficiency and a structural drainage incompetence, the term lipohyperplasia dolorosa has become established as the name for the clinical picture of lipoedema. This lymphological clinical picture has also undergone a paradigm change with regard to the therapeutic options over the last 15 years: lipohyperplasia dolorosa is curable. This four part series reports on the principles and successes in operative lymphology. Zusammenfassung Geschätzt werden 3,5 Millionen PatientInnen, die an einer angeborenen Fettverteilungsstö-rung der Beine und Arme leiden. Da diese Fettverteilungsstörungen aufgrund einer lym -phologischen Hochvolumeninsuffizienz und einer strukturellen Drainageschwäche schmerzhaft ist, hat sich der Begriff Lipo-hyperplasia dolorosa zur Bezeichnung des Krankheitsbildes Lipödem durchgesetzt. Auch in Bezug auf die therapeutischen Optionen erlebte dieses lymphologische Krankheitsbild in den letzten 15 Jahren einen Paradigmenwechsel: Lipohyperplasia dolorosa ist heilbar. In einer vierteiligen Serie wird über Grundlagen und Erfolge der operativen Lymphologie berichtet werden.

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.

Background: As we did not find any reliable datas concerning the prevalance of lipoedema (in Middle Europe), we started an anamnestic, clinical and (duplex-)sonographic investigation in 62 professional women. We were endavoured to avoid a bias of assertion and to get a socially correct composition of the collective. Results: Including all stages of lipoedema, also the mild ones (lipohyperplasia), 39 % of women were concerned. Regarding only the moderate to distict findings of markedly filled in ankles, 9.7 % were concerned. Only the breeches-type was found in 4.8 %. There were no relevant differences in age, length and age of menarche in women without and with lipoedema. Women with lipoedema were heavier than those without and they had accordingly a higher body-mass index. The thickness of subcutis + cutis 8 cm above the medial malleolus was 11 ± 2.8 mm in women without lipoedema and about 16 mm in those with lipoedema (beyond the double standard deviation). The thickness of the cutis lied on average distictly under 2 mm in both groups. Sonography seems to be a safe tool in the judgment of the subcutis – also in epidemiological studies.

Lipedema is an infrequently recognized and often neglected clinical entity that nearly always affects women. It poses a diagnostic challenge as one of the common disorders that is easily confused with lymphedema.