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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.
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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.
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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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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.
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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.
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Lipedema is an underdiagnosed syndrome of unclear etiology, characterized by symmetric painful enlargement of the buttocks and lower extremities, which spares the feet. This enlargement is caused by the deposition of adipose tissue. It was first described by Allen and Hines in 1940, who observed that it had a female predilection; patients commonly had an associated family history. We describe a patient with classic lipedema and multiple lipomas of her arms and trunk.
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BACKGROUND AND PURPOSE: Unclear extremity complaints are common symptoms of inpatients. In a subset of these patients, a clinical suspicion of deep vein thrombosis (DVT) results; this needs to be quickly and definitively clarified by a vascular physician. The question arose of how often a clinical suspicion of DVT was confirmed in an inpatient population and which alternative diagnoses were able to be made by angiologists. PATIENTS AND METHODS: In a retrospective analysis, all inpatients in the Angiologic Vascular Diagnostics Center of the University Hospital Halle, Germany, examined in 2007 for a suspicion of DVT were evaluated with respect to the definitively made diagnosis. RESULTS: In 213 (28.6%) of 745 suspected cases of DVT, a DVT was confirmed. In 532 patients (71.4%), DVT was excluded. In 314 of these patients, 436 alternative diagnoses were recorded in the diagnostic reports of angiologic examinations. In 38.6% (n = 168), other venous causes could be confirmed as the most common alternative diagnosis. There were chronic venous diseases in 28% (n = 122), superficial thrombophlebitis (n = 27), and tumor-related pelvic vein compression (n = 19). 17.4% (n = 76) exhibited lymphedema. In 13.3% (n = 58), a generalized edema was diagnosed. Arthrogenic causes followed with 12.8% (n = 56). Lipedema (5.3%) and hematoma (5%) could be verified as other important differential diagnoses. Rare causes were symptomatic or ruptured Baker's cysts (2.5%), erysipelas (2.5%), abscess, aneurysm, muscle tears, and tumors. CONCLUSION: The variety of alternative diagnoses in patients with clinical suspicion of DVT is high. The knowledge and systematic examination of potential, even rare differential diagnoses after exclusion of DVT are part of the repertoire of the vascular physician. Unnecessary and expensive, as well as onerous, diagnostic procedures on the patient can be avoided. Anticoagulation that was begun as a result of the suspicion of DVT can quickly be stopped.
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Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia. Sequential compression relieves lymphedema pain; we therefore hypothesized that whole body cyclic pneumatic hypobaric compression may relieve pain in AD. To avoid exacerbating hyperalgesia, we utilized a touch-free method, which is delivered via a high-performance altitude simulator, the Cyclic Variations in Altitude Conditioning™ (CVAC™) process. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 20-40 minutes of CVAC process daily for 5 days. Participants lost weight (195.5 ± 17.6-193.8 ± 17.3 lb; P = 0.03), and bioimpedance significantly decreased (510 ± 36-490 ± 38 ohm; P = 0.01). There was a significant decrease in scores on the Pain Catastrophizing Scale (P = 0.039), in average (P = 0.002), highest (P = 0.029), lowest (P = 0.04), and current pain severity (P = 0.02) on the Visual Analogue Scale, but there was no change in pain quality by the McGill Pain Questionnaire. There were no significant changes in total and physical SF-36 scores, but the mental score improved significantly (P = 0.049). There were no changes in the Pain Disability Index or Pittsburgh Sleep Quality Index. These data present a potential, new, noninvasive means of treating pain in AD by whole body pneumatic compression as part of the CVAC process. Although randomized, controlled trials are needed to confirm these data, the CVAC process could potentially help in treating AD pain and other chronic pain disorders.
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BACKGROUND: Lipoedema is an accumulation of fat abnormally distributed in the lower limbs, and lymphoedema is oedema caused by a deficiency of the lymphatic system. High-resolution ultrasound operating at 20 MHz makes it possible to characterize dermal oedema. OBJECTIVES: The purpose of our study was to demonstrate that high-resolution ultrasound imaging of the skin can differentiate lipoedema from lymphoedema. METHODS: Sixteen patients with lymphoedema (22 legs), eight patients with lipoedema (16 legs) and eight controls (16 legs) were included. Patients with lipolymphoedema were excluded. Ultrasound examinations were carried out with a real-time high-resolution ultrasound device on three different sites for each lower limb. The images were then anonymized and examined by an independent dermatologist who was blind to the clinical diagnosis. A new series of images was examined by three dermatologists to check interobserver agreement. RESULTS: A significant difference in dermal thickness was observed between patients with lymphoedema and those with lipoedema and between patients with lymphoedema and controls. No significant difference in dermal thickness was shown between patients with lipoedema and controls at the thigh or ankle. Dermal hypo-echogenicity was found in at least one of the three sites in 100% of patients with lymphoedema, 12.5% of cases with lipoedema and 6.25% of the controls. Hypoechogenicity affected the entire dermis in all cases of lymphoedema except one. In cases of lipoedema and controls, hypoechogenicity was localized at the ankle and prevailed in the upper dermis. The expert correctly diagnosed all lower limbs with lymphoedema. No cases of lipoedema were diagnosed as lymphoedema. Exact interobserver agreement was excellent (0.98). CONCLUSIONS: High-resolution cutaneous ultrasonography makes it possible to differentiate lymphoedema from lipoedema. Obtaining a reliable diagnosis through high-resolution cutaneous ultrasonography might be valuable for improving the treatment of lipoedema and lymphoedema.
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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.
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Lipedema is a condition characterized by swelling and enlargement of the lower limbs due to abnormal deposition of subcutaneous fat. Lipedema is an under-recognized condition, often misdiagnosed as lymphedema or dismissed as simple obesity. We present a series of pedigrees and propose that lipedema is a genetic condition with either X-linked dominant inheritance or more likely, autosomal dominant inheritance with sex limitation. Lipedema appears to be a condition almost exclusively affecting females, presumably estrogen-requiring as it usually manifests at puberty. Lipedema is an entity distinct from obesity, but may be wrongly diagnosed as primary obesity, due to clinical overlap. The phenotype suggests a condition distinct from obesity and associated with pain, tenderness, and easy bruising in affected areas.
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Lipoedema is a distinct clinical condition characterized by bilateral, symmetrical enlargement of the buttocks and lower limbs owing to excess deposition of subcutaneous fat. It is found almost exclusively in women. The common features associated with this condition are 'column- shaped' legs with sparing of the feet, bruising, sensitivity to pressure, and orthostatic oedema. The progression to lipo-lymphoedema or morbid obesity is possible. Conservative measures used in the management of lymphoedema can prevent progression/limit the orthostatic oedema. Surgical procedures may also play a part in the management of lipoedema.
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Lipohyperplasia dolorosa and lymphedema are completely different disease entities, which are both, however, classified under lymphology. While in lipohyperplasia dolorosa a congenital lipid distribution disorder leads to a high volume insufficiency and the corresponding clinical symptoms, lymphedema is characterized by a congenital transport incompetence of the vessels or acquired disorders of transport capacity. Both lymphedemas of different genesis are familial volume alterations of the affected regions and the increase in volume is irreversible if not exclusively still in stage I or II. According to current knowledge the solid increase in volume by lymphedema is due to a malfunctioning biomechanism by which the release of additional proteoglycans in the homeostasis system of the fluid in the interstital space plays an important role. Removal of this tissue and the sponge-like substance of proteoglycans is the aim of therapeutic approaches. Manual lymph drainage and compression can evacuate the sponge but not remove it. Lymphological liposculpture is a successful dermatosurgical measure even for secondary lymphedema. Reduction of the necessity of complex hemostasis therapy to 20% of the initial value and an adjustment of the affected extremity on the healthy side, represent a clear improvement in quality of life of patients. The same dermatosurgical method, lymphological liposculpture, has been known for many years to fulfil the successfully proven purpose for the treatment of lipohyperplasia dolorosa by the removal of subcutaneous fatty tissue, present as hyperplasia and not hypertrophy. Tenderness and the necessity for complex hemostasis therapy are no longer present or no longer necessary after lymphological liposculpture for lipohyperplasia dolorosa. This condition is permanent because the congenital fatty masses do not reoccur following surgical removal. Lipohyperplasia dolorosa is therefore curable by lymphological liposculpture. For secondary lymphedema a drastic improvement in quality of life of the patient can be achieved by this method which is demonstrated by the adjustment of symmetry of the extremities and reduction or even avoidance of complex hemostasis therapy.
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A prospective single-center study with mail questionnaire – in most cases combined with clinical controls – was carried out in 112 patients with lipedema after a period from 8 months to nearly 7 years following liposuction in tumescent local anesthesia.
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Lipedema is a disease with unknown etiology presenting as bilateral and symmetric enlargement of the lower extremities due to subcutaneous deposition of the adipose tissue. Here we describe the histopathological features of the lipedema tissue and nonaffected adipose tissue obtained from a typical patient with severe lipedema. Immunohistochemical analyses indicated degenerative and regenerative changes of the lipedema tissue, characterized by crown-like structures (necrotizing adipocytes surrounded by infiltrating CD68+ macrophages; a feature commonly seen in obese adipose tissue) and proliferation of adipose-derived stem/progenitor/stromal cells (Ki67+CD34+ cells), respectively. These findings suggested increased adipogenesis in the lipedema tissue, which may further lead to hypoxia similar to that seen in obesity, resulting in adipocyte necrosis and macrophage recruitment. The confinement to the lower extremities and the difference from systemic obesity warrants further elucidation in future studies.
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Lipoedema is an infrequently recognized disorder in women. Lipoedema is characterized by bilateral enlargement of the legs due to abnormal depositions of subcutaneous fat associated with often mild oedema. There is substantial variability in disease severity. The diagnosis should be made as early as possible to prevent complications of the disorder, which is associated with increasing functional and cosmetic morbidity. This review describes clinical manifestations, pathogenesis, technical investigations, management and therapies of lipoedema, with the aim of optimizing management and care of patients with lipoedema.
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Lipoedema is an infrequently recognized disorder in women. Lipoedema is characterized by bilateral enlargement of the legs due to abnormal depositions of subcutaneous fat associated with often mild oedema. There is substantial variability in disease severity. The diagnosis should be made as early as possible to prevent complications of the disorder, which is associated with increasing functional and cosmetic morbidity. This review describes clinical manifestations, pathogenesis, technical investigations, management and therapies of lipoedema, with the aim of optimizing management and care of patients with lipoedema.
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We report a familial Sotos syndrome in two children, boy and girl, aged 17 and 8 years, and in their 44 year old mother, who displayed normal intelligence at adult age, but suffered from insulin dependent diabetes mellitus, bronchial asthma, and severe lipedema. The underlying missense mutation, C2175S, occurred in a conserved segment of the NSD1 gene. Our findings confirm that familial cases of SS are more likely to carry missense mutations. This case report may prove useful to avoid underestimation of the recurrence rate of SS, and to demonstrate that the developmental delay may normalize, enabling an independent life and having an own family.
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Overweight and obesity is a public health problem in Hungary and in the Western world. It is important to underline that obesity is an illness and an important risk factor for several skin and other diseases. An overview of skin diseases caused or aggravated by obesity (acanthosis nigricans, acrochordons, keratosis pilaris, hyperandrogenism, stria, adiposis dolorosa, lymphoedema, chronic venous insufficiency, plantar hyperkeratosis, lipoedema, skin infections, acne inversa, psoriasis, tophi) helps us to look and see as well. Look for the possibility of skin infections as it helps the early diagnosis and to avoid complications. Draw patients' attention to the preventive importance of skin care. In case of an obese patient the usual dosage of most local and systemic drugs should be modified. It must be kept in mind that obesity directly or indirectly starts unfavorable processes in almost all organ systems. Therefore, only a multidisciplinary care may secure treatment and rehabilitation of obese patients. Dermatological and lymphological care is often part of the rehabilitation.
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