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The primary lymphedema is a pathological interstitial accumulation of lymphatic fluid. Lower limbs are mainly affected by primary lymphedema. It is caused by a hereditary lack or complete absence of lymphatic vessel systeme. Final complications of chronic limb lymphedema include »elephantiasis«, lymphatic ulceration and malignant degeneration. Secondary lymphedema, obesity, phlebedema and lipedema are most important differential diagnoses.Treatment of primary lymphedema includes early manual lymph drainages, decrease in weight, exercise, prevention or therapy of skin infections. Up to the present primary lymhedema cannot be cured in causality.

Lipoedema: Improved Quality of Life by Combination of Therapies Lipoedema is a chronic progressive disease with increased fat volumes mainly of the legs, causing body disproportion together with orthostatic oedema, pain and bruising. Early diagnosis can be achieved by clinical symptoms. Complex physical therapy with manual lymphatic drainage, compression and physiotherapy reduces oedema and complaints. Surgical therapy with liposuction reduces circumscribed fat volumes, improves body proportions and diminishes oedema and bruising. Following surgery physical therapy can be diminished in intensity and number. Nowadays the combination of physiotherapy and liposuction is considered the optimal treatment regime which can tremendously improve the patient's quality of life.

Modern aspects of pathophysiology, epidemiology, symptoms, diagnosis and therapy in lipedema are presented. Within recent years the development of new techniques in local anaesthesia and surgery has revolutionized therapy. By using surgical and conservative methods (tumescent liposuction and combined decongestive therapy) a normalization of body proportions and a reduction of subjective and objective symptoms with a distinct improvement in the quality of life can be achieved.

In vivo measurements in 26 female patients with lipedema and cellulite parameters were carried out before and after therapy by means of complex physical decongestive therapy (CPDT) including manual lymph drainage and compression as main components and/or shock wave therapy (SWT). Oxidative stress parameters of blood serum and biomechanic skin properties/smoothening of dermis and hypodermis surface were evaluated. Oxidative stress in lipedema and cellulite was demonstrated by increased serum concentrations of malondialdehyde (MDA) and plasma protein carbonyls compared with healthy control persons. Both MDA and protein carbonyls in blood plasma decreased after serial shock wave application and CPDT. The SWT itself and CPDT itself lead to MDA release from edematous tissue into the plasma. Obviously both therapy types, SWT and CPDT, mitigate oxidative stress in lipedema and cellulite. In parallel SWT improved significantly the biomechanic skin properties leading to smoothening of dermis and hypodermis surface. Significant correlation between MDA depletion of edematous and lipid enriched dermis and improvement of mechanic skin properties was demonstrated. From these findings it is concluded, that a release of lipid peroxidation (LPO) products from edematous dermis is an important sclerosis-preventing effect of SWT and/or CPDT in lipedema and cellulite. Expression of factors stimulating angiogenesis and lymphangiogenesis such as VEGF was not induced by SWT and/or CPDT and, therefore, not involved in beneficial effects by SWT and/or CPDT.

Lipedema is characterized by bilateral enlargement of the legs due to abnormal deposition of fat tissue from pelvis to ankles. It is seen most frequently in obese women. Lipedema appears to be a distinct clinical entity but may be confounded with lymphedema. AIM OF THE STUDY: To analyze and to compare between lipedema and lymphedema the qualitative and quantitative aspects of lymphoscintigraphy. METHODS: Fifteen women with lipedema were recruited. Mean age of onset of lipedema was 31.5 +/- 15 years. Body mass index was 35.1 +/- 7.9 kg/m2, 13 women were obese. Lipedema was compared to 15 cases of primary lymphedema (women: 13, men: 2) of the lower limbs (unilateral: 13, bilateral: 2), with a mean age at onset of 28.7 +/- 12.6 years. Lymphoscintigraphy of the lower limbs with morphologic (visualization of inguinal lymph nodes) and kinetic (half-life, lymphatic speed of the colloid) studies was performed in all cases. RESULTS: Absence of visualization of inguinal lymph nodes was observed in 14/15 cases of lymphedema and in 1/15 cases of lipedema (p<0.001). In the 13 cases of unilateral lymphedema, colloid half-life was higher in the pathologic limb than in the controlateral limb (230 +/- 92 vs 121 +/- 36 minutes, p<0.01) and lymphatic speed of the colloid was slower (6.91 +/- 0.86 vs 8.16 +/- 1.02 cm/min, p<0.001). The two patients with bilateral lymphedema had an increased half-life and decreased lymphatic speed of the colloid. Colloid half-life was significantly higher in lipedema than in controlateral limbs of lymphedema (154 +/- 23 vs 121 +/- 36 minutes, p<0.01) with no difference in lymphatic speed of the colloid. Colloid half-life was significantly higher in lymphedema than in lipedema (230 +/- 92 vs 154 +/- 23 minutes, p<0.01) and the lymphatic speed of the colloid was slower (6.91 +/- 0.86 vs 8.10 +/- 0.45 cm/min, p<0.001). CONCLUSION: Lower limb lymphoscintigraphy showed lymphatic insufficiency in lipedema without morphologic abnormality as seen in lymphedema. Lymphoscintigraphy is not indispensable but is a useful tool when diagnosis is doubtful. Treatment is difficult and may include weight loss and possible surgery.

Ich habe 2002 in Denver die Operation des Lipodems und den Vorschlag zu ihrer Standardisierung vorgestellt. Heute konnen wir die Follow-up-Untersuchungen der Patienten besprechen. Alle Patienten wurden mittels indirekter Lymphszintigraphie und indirekter Lymphangiographie vor- und nachuntersucht. Die Ergebnisse zeigen eindeutig, dass die Liposuktion beim Lipodem zu einer Normalisierung des Lymphflusses fuhren. Die Schmerzhaftigkeit des Krankheitsbildes Lipohypertrophia dolorosa wird drastisch verbessert. Manuelle Lymphdrainage und Kompression, die bislang bei diesen Patientinnen an Armen und Beinen lebenslang durchgefuhrt werden mussten, werden in der Frequenz mindestens verringert. In den meisten Fallen konnte vollig darauf verzichtet werden. Ich werde eine Variation der Operation in Lagerung und Zugangsweg vorstellen. Wir operieren heute die Arme und Beine in insgesamt zwei Sitzungen. Der Stellenwert der postoperativen manuellen Lymphdrainage und der lang andauernden Kompression durch Garments soll im besonderen noch einmal unterstreichen, dass es sich bei der Operation des Lipodems nicht um eine ausschlieslich asthetische, sondern in erster Linie um eine medizinische Indikation handelt.

Lipedema refers to the abnormal deposition of subcutaneous fat causing a striking enlargement of the lower extremities that is out of proportion to the upper body. Most clinicians are unaware of this disease and thus it is seldom diagnosed correctly. Cutaneous myiasis is the infestation of skin by fly larvae. We describe an unusual case of a woman with lipedema who developed cutaneous myiasis.

Both generalized and localized edema needs to be submitted to a differential diagnostic investigation. In the case of edema affecting the lower extremities, in particular the Stemmer sign which is the inability to tent the skin at the dorsum of the toes is a useful distinguishing aid. If there is acute unilateral swelling of a leg, other processes with diffuse space-consuming processes need to be distinguished from deep venous thrombosis and secondary lymphedema. Chronic bilateral leg edema is usually due to a venous flowoff obstruction (stasis edema). Less commonly, lipedema or a primary lymphedema may be responsible for the swelling.

Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg with tenderness, but excluding the feet. Lipoedema occurs almost exclusively in females, and no male patient has been reported in the literature published in English. We report on an extremely rare presentation of lipoedema in a male patient. A thorough study based on the case history, physical manifestations, and magnetic resonance imaging (MRI) provided valuable clues for a differential diagnosis. Conservative treatment using weight reduction, compression-stocking application, and diuretic therapy was not effective. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment.

Because of the lifelong and often progressive course and the mental trauma to the patients, lipoedema is an important dermatologic disorder. Complex physical therapy programs were introduced as a standard therapy years ago and can achieve an impressive oedema reduction. Liposuction in tumescent local anesthesia with vibrating microcannulas has proved to be a new effective treatment. A targeted and permanent reduction of the fat tissue leads to an increased quality of life due to an improved appearance, reduced tendency to swelling and less pain.

BACKGROUND: Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia. CASE REPORT: We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure. CONCLUSION: This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).

Up until recently, complex physical therapy has been the mainstay in treatment of lipedema. This generally improved edema and reduced pain and tension in affected patients. More recently, surgical approaches such as liposuction have been used to reduce the fat volume under tumescent local anesthesia. Combining both methods, dramatic improvements can be achieved in treating the disease and in improving the quality of life. However liposuction in lipedema should only be performed in specialized medical centers.

As the science of wound healing has evolved over the past two decades, so has awareness of the "hidden epidemic" of lymphedema. Substantial information has been accumulated regarding the pathophysiology and therapy of lymphedema. Until recently, the relationship between wound healing and the negative effects of associated peri-wound lymphedema has received little attention. Identifying wound-related lymph stasis and safe mobilization of the fluid are fundamentals that must be addressed for proper therapy. Experience gained from the successful treatment of primary and secondary lymphedema has proven very useful in the applications to wound-related lymphedema. The mobilization of lymph fluid from the peri-wound area with the use of reasoned compression is essential for proper therapy of the open wound, as are appropriate bandage selection and safeguards for bandage application.

HYPOTHESIS: The causes and management of lower limb lymphedema in the Western population are different from those in the developing world. OBJECTIVE: To look at the differential diagnosis, methods of investigation, and available treatments for lower limb lymphedema in the West. DATA SOURCE: A PubMed search was conducted for the years 1980-2002 with the keyword "lymphedema." English language and human subject abstracts only were analyzed, and only those articles dealing with lower limb lymphedema were further reviewed. Other articles were extracted from cross-referencing. RESULTS: Four hundred twenty-five review articles pertaining to lymphedema were initially examined. This review summarizes the findings of relevant articles along with our own practice regarding the management of lymphedema. CONCLUSIONS: The common differential diagnosis in Western patients with lower limb swelling is secondary lymphedema, venous disease, lipedema, and adverse reaction to ipsilateral limb surgery. Lymphedema can be confirmed by a lymphoscintigram, computed tomography, magnetic resonance imaging, or ultrasound. The lymphatic anatomy is demonstrated with lymphoscintigraphy, which is particularly indicated if surgical intervention is being considered. The treatment of choice for lymphedema is multidisciplinary. In the first instance, combined physical therapy should be commenced (complete decongestive therapy), with surgery reserved for a small number of cases.