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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.

Edema of the limbs is a common reason for medical consultation, for which the lymphoscintigraphy is considered to be a reliable method for its differential diagnosis. OBJECTIVE: To evaluate the usefulness of radionuclide studies in the differential diagnosis of edema, and the diagnostic yield of different scintigraphic patterns. MATERIAL AND METHODS: A total of 61 patients, mean age 43 years, referred to our Department in the last three years for suspected lymphoedema, were considered. One patient was discarded due to lack of diagnosis, 56 had lower limb edema and 4 upper limb edema. After intradermal injection of two doses of (99m)Tc-nanocolloid, scintigraphic scans were made at 30 and 120minutes. The final diagnosis was based on imaging tests, clinical course, and response to treatment. We calculated the parameters of the diagnostic yield of four different scintigraphic patterns (presence of dermal backflow, asymmetry-alteration in inguinal/axillary nodes, presence of collateral pathways, and visualization of intermediate lymph nodes), considering them individually and jointly. RESULTS: The best diagnostic yield was achieved by considering dermal backflow and asymmetry in inguinal/axillary nodes (accuracy 88.9%, specificity 96.4%, PPV 95.5%). Evaluation of intermediate lymph nodes and presence of collateral pathways contributed little to the diagnostic yield, showing poor sensitivity and high false positive rates. CONCLUSION: The lymphoscintigraphy had high diagnostic yield, allowing early treatment of lymphœdema. The dermal backflow and asymmetry in inguinal/axillary nodes had the greatest diagnostic accuracy. Evaluation of intermediate lymph nodes and visualization of collateral pathways contributed little to improving the diagnosis.

The pathophysiology of skin diseases associated with monoclonal gammopathies is generally unknown. Our aim was to investigate whether a monoclonal gammopathy could be a causal factor in progressive lymphedema. We describe a 75 year old patient with a rapidly progressive lipo-lymphedema and a monoclonal gammopathy of unknown significance (MGUS) suspected as a key etiological factor. Dermal fibroblasts were cultured from lesional lower leg skin and non-lesional abdominal skin and compared to healthy control fibroblasts. We found 10-fold elevated basic fibroblast growth factor 2 (FGF-2) in the patient's serum and significantly increased basal FGF-2 production of lesional and non-lesional fibroblasts compared to healthy controls. Upon restimulation with patient or healthy control serum, lesional fibroblasts showed significantly increased proliferation rates and FGF-2 production in vitro. Non-lesional abdominal fibroblasts showed an intermediate phenotype between lesional and control fibroblasts. Our findings provide the first evidence that lesional dermal fibroblasts from lipo-lymphedema with plasma cell infiltration show increased proliferation and FGF-2 production and that both local tissue factors and altered FGF-2 serum levels associated with monoclonal gammopathies might contribute to this phenotype. Thus we propose a possible pathophysiologic link between the gammopathy-associated factors and the generation of lymphedema with initial fibrogenesis aggravating pre-existing lipedema.

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.

Lipedema is a disproportional obesity due to unknown pathomechanism. Its major hallmark is frequent hematoma formation related to increased capillary fragility and reduced venoarterial reflex. Beyond microangiopathy, both venous and lymphatic dysfunction have also been documented. However, arterial circulation in lipedema has not been examined, and therefore we explored aortic elastic properties by echocardiography. Fourteen women with and 14 without lipedema were included in the study. Each subject consented to blood pressure measurement, physical examination, and transthoracic echocardiography. Aortic stiffness index (beta), distensibility, and strain were evaluated from aortic diameter and blood pressure data. Mean systolic (30.0 +/- 3.2 vs. 25.5 +/- 3.6, P < 0.05) and diastolic (27.8 +/- 3.3 vs. 22.3 +/- 3.1) aortic diameters (in mm) and aortic stiffness index (9.05 +/- 7.45 vs. 3.76 +/- 1.22, P < 0.05) were significantly higher, while aortic strain (0.082 +/- 0.04 vs. 0.143 +/- 0.038, P < 0.05) and distensibility (2.24 +/- 1.07 vs. 4.38 +/- 1.61, P < 0.05) were significantly lower in lipedematous patients compared to controls. Thus, lipedema is characterized with increased aortic stiffness.

Lipedema is a condition characterized by abnormal deposition of adipose tissue in the lower extremities leading to circumferential bilateral lower extremity enlargement typically seen extending from the hips to the ankles. Diagnosis of the condition is often challenging, and patients frequently undergo a variety of unsuccessful therapies before receiving the proper diagnosis and appropriate management. Patients may experience pain and aching in the lower extremity in addition to distress from the cosmetic appearance of their legs and the resistance of the fatty changes to diet and exercise. We report a case of a patient with lipedema who was treated with suction-assisted lipectomy and use of compression garments, with successful treatment of the lipodystrophy and maintenance of improved aesthetic results at 4-year postoperative follow-up.

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.