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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.

"Lipedema," a special form of obesity syndrome, represents swelling of the legs due to an increase of subcutaneous adipose tissue. In 12 patients with lipedema of the legs and in 12 healthy subjects (controls), fluorescence microlymphography was performed to visualize the lymphatic capillary network at the dorsum of the foot, at the medial ankle, and at the thigh. Microaneurysm of a lymphatic capillary was defined as a segment exceeding at least twice the minimal individual diameter of the lymphatic vessel. In patients with lipedema, the propagation of the fluorescent dye into the superficial lymphatic network of the skin was not different from the control group (p > 0.05). In all 8 patients with lipedema of the thigh, microaneurysms were found at this site (7.9 +/- 4.7 aneurysms per depicted network) and in 10 of the 11 patients with excessive fat involvement of the lower leg, multiple microlymphatic aneurysms were found at the ankle region. Two obese patients showed lymphatic microaneurysms in the unaffected thigh and in only 4 patients were microaneurysms found at the foot. None of the healthy controls exhibited microlymphatic aneurysms at the foot and ankle, but in one control subject a single microaneurysm was detected in the thigh. Multiple microlymphatic aneurysms of lymphatic capillaries are a consistent finding in the affected skin regions of patients with lipedema. Its significance remains to be elucidated although its occurrence appears to be unique to these patients.

Lipedema never reveals clinical picture of extreme lymphedema-elephantiasis, and skin signs and complications have not been observed. Aim of this paper is to present a case of lipedema with the initial lymphedema in which, after one episode of lymphangiitis and cellulitis, came to the rapid development of lymphedema followed by chyloderma. During the local treatment of extreme chyloderma with excessive exudation, semiocclusive synthetic dressings have been used for moist wound healing. The treatment was completed after 20 weeks with total epithelizsation, without maceration and irritation, without additional spreading of the chyloderma field, without wound infections, with fast and full relief of the pain. Lipedem with extreme lymphedema can be followed by skin complications of lymphedema like chylodermia.

We describe a 52 year-old woman in whom lymphedema primarily of the abdominal wall was superimposed on lipedema resulting in an abdomen of enormous dimensions with marked impairment of ambulation. Treatment consisted of preoperative compression of the legs by an external pneumatic device (Lympha-Press) followed by excision of the lymphedematous abdominal fat pad in conjunction with "debulking" of the right leg. The patient illustrates the extremes of lipedema complicated by lymphedema and the technical difficulties associated with its management.

PURPOSE: Lymphoscintigraphy has emerged as the diagnostic test of choice in patients with suspected lymphedema. To assess the lymphatic circulation of 386 extremities in 188 patients, we prospectively recorded a semiquantitative index of lymphatic transport in addition to visual evaluation of lymphoscintigraphy image patterns. METHODS: Sixty-one male and 127 female patients were studied (mean age 48 years, range 13 to 87 years). Twenty had upper extremity swelling, and 168 had lower extremity swelling. The disease was bilateral in 60 patients. Lymphoscintigraphy was performed by injecting a mean of 503 microCi of technetium 99m-antimony trisulfide colloid subcutaneously into the second interdigital space of the extremity. Time for transport to regional lymph nodes, appearance of lymph vessels and nodes and distribution pattern were scored. These scores were compiled into a modified Kleinhans transport index (TI). To assess the venous circulation, 155 patients underwent evaluation of the venous system by impedance plethysmography, ultrasonography, or contrast venography. RESULTS: The mean TI (+/- SEM) in 79 asymptomatic extremities was 2.6 +/- 0.5, with 66 (83.5%) demonstrating normal lymphoscintigraphy pattern (TI < 5). Patients with clinical diagnosis of lymphedema (n = 124) had a mean TI of 23.8 +/- 1.5; 81.5% of these were greater than 5. Fifty-six patients (30%) had primary and 68 (36%) had secondary lymphedema. (TI of 26 +/- 3.5 and 22.1 +/- 1.9, respectively, p = NS). Patients without any lymphatic transport (TI of 45) were more likely to have cellulitis in their history (p < 0.05). Contrast lymphangiography in six patients correlated with lymphoscintigraphy. Sixty-four patients (34%) had swelling without lymphedema (venous edema, cardiac edema, lipedema, etc.; TI of 1.9 +/- 0.4, p < 0.001). Of the 41 patients with abnormal venous studies, 18 (44%) had an elevated TI. CONCLUSIONS: Semiquantitative evaluation of the lymphatic transport with lymphoscintigraphy reliably depicts abnormalities in the lymphatic circulation. Lymphoscintigraphy excluded lymphedema as a cause of leg swelling in one third of our patients.

Twelve patients with primary lymphedema of the lower limb were examined with computed tomography (CT). A characteristic "honeycomb" pattern of the subcutaneous compartment was seen in 10 of these patients. CT scans in nine other patients with swollen leg secondary to chronic venous disease or lipedema did not show this characteristic pattern. CT may be helpful in the differential diagnosis of a swollen leg, thus obviating venography or lymphangiography.

« La parenté de l'ædeme chronique avec la lipomatose a depuis long lemps frappé les auteurs » ( 1 ) . Le cas actuel que nous publions aujourd'hui, après l'avoir présenté en quelques mots à la Société de Neurologie ( 2) , vient à l'appui de celle rollesion Ichard et L. Lévi. OBSERVATION ( PI. LXV ). Il s'agit d'une brodeuse de 39 ans, Mlle Eugénie B ... 1, venue à notre consul tation de l'hôpital Laënnec pour grosseur anormale des membres inférieurs . Antécédenis héréditaires -- La mère est morte à 25 ans, de tuberculose aiguë (alcoolisme possible). Son père est mort à 56 ans, très probablement d'un cancer du pylore. Eugénie B ... a une scur âgée de 41 ans, bien portante. Une de ses tantes aurait eu , pendant longtemps, de la difficulté à marcher . Antécédents personnels. -- Mile B ... n'a jamais été malade. Elle se souvient que, vers l'âge de 8 ans, elle avait déjà de gros mollets qui faisaient l'admira tion de ses camarades ; néanmoins, en promenade, elle se fatiguait plus vite que ses compagnes. Elle n'a jamais ressenti de douleurs vives dans les mem bres inférieurs. A 14 ans ses règles apparaissent régulières, avec un peu de dysmenorrhée. A 22 ans,, elle s'aperçoit que, sans aucun doute, ses jambes grossissent et non le reste du corps . L'ordème aurait envahi, par étapes, les jambes, les cuisses et les fesses . Elle est examinée et traitée à Lille , à Amiens et à Paris. A Lille elle prend de la tyroïdine ( 2 pilules par jour) , et de l'iode , pendant 15 jours . Elle maigrit sous l'influence de ce traitement.

<p>INTRODUCTION: Lipedema is a progressive connective tissue disease with enlargement of adipose tissue, fibrosis, fluid collection and dermal thickening. Herein, we present a case of lipedema associated with skin hypoperfusion and ulceration in which soft tissue debulking with liposuction improved patients’ symptoms. CASE PRESENTATION: A 39-year-old female presented with asymmetric progressive initially unilateral lower limb swelling with severe pain with subsequent skin ulceration. Conservative management have failed to improve her condition. After excluding other causes and detailed radiologic investigation, lipedema was diagnosed with an associated impaired skin perfusion. Trial of local wound care and compression therapy failed to improve the condition. Subsequent soft tissue debulking with circumferential liposuction and ulcer debridement and immediate compression showed dramatic improvement of the symptoms and skin perfusion. DISCUSSION: The unique nature of this case shed light on lipedema as a loose connective tissue disease. Inflammation and microangiopathies explains the associated pain with hypoperfusion and ulceration being quite atypical and in part might be related to the large buildups of matrix proteins and sodium contents leading to micro-vessels fragility with frequent petechiae and hematoma and subsequent tissue ischemia. Conservative measures like compression therapy plays a significant role in disease course. Surgical debulking with liposuction was shown to be efficacious in reducing the soft tissue load with improvement in limb pain, edema, circumference and skin perfusion that was seen in our patient. CONCLUSION: Lipedema is a frequently misdiagnosed condition with disabling features. Skin involvement in lipedema with potential hypoperfusion was shown and it requires further investigation</p>