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Lipedema is a chronic vascular disease almost exclusively of female sex, characterized by the deposit of fat on the legs, with an "Egyptian column" shape, orthostatic edema, hypothermia of the skin, alteration of the plantar support, and negativity of Stemmer's sign. The etiology and pathogenesis of this disease are still the object of study, and therapy is very difficult. Various authors have described morphologic and functional alterations of prelymphatic structures and of lymphatic vessels. The big veins remain untouched in the phlebograms and an alteration of the skin elasticity is demonstrated. The present authors have studied by dynamic lymphoscintigraphy 12 women patients suffering from lipedema, and compared the results with those of 5 normal subjects and 5 patients suffering from idiopathic lymphedema who were sex and age matched with the patients suffering from lipedema. The patients suffering from lipedema showed an abnormal lymphoscintigraphic pattern with a slowing of the lymphatic flow that presented some analogies to the alterations found in the patients suffering from lymphedema. A frequent asymmetry was also noticed in the lymphoscintigraphic findings that is in contrast to the symmetry of the clinical profile.

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.

Edemas of the leg sometimes pose problems for diagnosis. Invasive procedures like lymphography or phlebography are either difficult to perform or might endanger the lymphatics. The value of magnetic resonance imaging was assessed in 20 patients with lymphedema, lipedema and phlebedema. Images of patients with lipedema showed homogenous enlarged subcutaneous tissue. In lymphedema a honeycomb pattern in the subcutaneous tissue was observed; in phlebedema there was an increase of fluid within the muscle. Magnetic resonance imaging is useful in differentiating lymphedema, lipedema or phlebedema.

Microlymphatics of human skin form two superposed networks. The superficial one located at the level of dermal papillae may be visualized by fluorescence microlymphography. Microlymphatics fill from a subepidermal depot of minute amounts of FITC-dextran 150,000. In primary lymphedema with late onset the depicted network with vessels of normal size is significantly larger than in healthy controls, whereas in congenital lymphedema (Milroy's disease) microlymphatics are aplastic or ectatic (diameter > 90 microns). Lymphatic microangiopathy with obliterations of microvessels develops in chronic venous insufficiency, in lipedema (preliminary results) and after recurrent erysipelata. In healthy controls microlymphatics are permeable to FITC-dextran 40,000 and impermeable to the larger molecule 150,000. Preserved fragments of the network in chronic venous insufficiency exhibit increased permeability to FITC-dextran 150,000. After visualization of the vessels by the fluorescent dye microlymphatic pressure may be measured by the servo-nulling technique. First results indicate that microlymphatic hypertension contributes to edema formation in patients with primary lymphedema.

The authors assessed the use of magnetic resonance imaging in differentiating lymphedema, phlebedema, and lipedema of the lower limb. They examined 14 patients: five with lipedema, five with lymphedema, and four with phlebedema. T1- and T2-weighted transaxial sequences were performed before administration of gadolinium tetraazacyclododecane-tetraacetic acid (DOTA) and T1-weighted spin-echo sequences were performed after administration of Gd-DOTA in each patient. Images of patients with lipedema showed homogeneously enlarged subcutaneous layers, with no increase in signal intensity at T2-weighted imaging or after Gd-DOTA administration. Patients with phlebedema had areas containing increased amounts of fluid within muscle and subcutaneous fat. In lymphedema, a honeycomb pattern above the fascia between muscle and subcutis was observed, with a marked increase in signal intensity at T2-weighted imaging. After Gd-DOTA administration, there was only a slight increase in signal intensity in the subcutis in lymphedema and phlebedema and a moderate increase in signal intensity in muscle in phlebedema.

Lipedema is a common disease in the usual clinical practice. None organic description about the clinical symptoms and signs associated to this condition has been published. Fifty women with lipedema have been examined by the authors, and incidence rates of symptoms and signs have been emphasized. The following signs and symptoms were constantly reported: "Egyptian column", elastic edema, negative Stemmer's sign, alterated plantar support, cutaneous hypothermia. Some others were frequently found: ecchymosis, spontaneous pain, liposclerosis on the thigh, hypodermic hyperalgesia and pain on the internal face of the knee. Moreover, the two most relevant differential diagnosis as well as their two variant's clinical features (mixed lipedema and "thin women" lipedema) have been described.

The use of a single axial slice through the mid calf in the differential diagnosis of a swollen leg is described. This is a very simple quick non-invasive investigation. Venous obstruction results in an increase in the cross sectional area of the muscle compartment. The subcutaneous fat layer is normally homogeneous; in obesity or lipoedema it is increased but remains homogeneous. In lymphoedema fluid collects in the interstitial spaces which become very prominent on CT images. In chronic lymphoedema a honeycomb pattern is seen as a result of increase in the interstitial tissue due to fibrosis. Popliteal cyst extensions result in fluid collections between muscle planes. Haematomas have higher attenuation, and are intramuscular. The findings in 64 patients and 10 controls are presented and the literature is reviewed.

Lipodystrophy, almost exclusively seen in female patients, causes psychological problems at an early age. In later life, additional complaints are heavy painful legs, edema, and varicose veins. Nearly all patients suffer from alimentary adiposity. Dermolipectomies in general are not advisable because of the resulting large scars and the risk of damaging the lymphatic system. Subcutaneous lipectomy according to the Illouz method can provide good results. Weight loss is a good alternative to liposuction of the upper legs. After sufficient weight loss, only additional liposuction of the trochanteric area and the medial side of the knee is needed. No unpleasant scarring of the leg results. Unfortunately, older patients often require a skin reduction. The results of surgery in the lower legs were, except in 2 patients, good to excellent. The results in the upper legs were disappointing because 9 of 11 patients gained weight again after surgery. Considering this, the preferable treatment now is liposuction of the lower legs, medial side of the knee, and the trochanteric area. Only in cases of ptotic skin on the medial side of the upper legs is skin reduction without lipectomy indicated. Lipodystrophy suggests a disappearance of the subcutaneous fat. When this occurs in the upper part of the body, it is called progressive lipodystrophy. Lipodystrophy is known as an abnormality of the lower half of the female body, swollen by deposition of subcutaneous fat and determined by heredity. It occurs more frequently in the lower social classes and is often accompanied by an alimentary obesity which is a psychological reaction to the disturbed body image.(ABSTRACT TRUNCATED AT 250 WORDS)

Indirect lymphography by subepidermal infusion of newly developed nonionic, dimeric contrast media (e.g., Iotrolan) opacifies peripheral lymphatics of the skin. Using this method we examined 159 patients with primary and secondary lymphedema, chronic venous insufficiency, and lipedema and compared the findings to normal individuals. A variety of characteristic patterns were uncovered. The technique causes little patient discomfort and takes on the average only 30 minutes.

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.

Lipedema of the lower extremities are diagnosed on the basis of clinical findings . An exact differentiation is necessary since therapeutical considerations are depending on the pathophysiology of the diseases. In lipedema (Allen and Hines) as weil as in erythrocyanosis cruris puellarum (Klingmüller) an examination of the state of the lymphatic vessels is justified. Using isotope lymphography the lymphatic capacitY, of the lower extremities can be assessed without problem. As demonstrated a disturbance of lymphatic transport is not regularly present in these two syndroms. Therefore we can not consider them as lymphedema. The frequent appearance of unilateral lymphostasis with definite clinical differenccs of both extremities indicate on the other band that lipedema is not seldom superimposed by the lymphedema .

Patients with lipoedema of the typus rusticanus Moncorps show a skin elasticity deficit of the skin of the calf. This is partly due to the derma oedema in the skin of these patients and seems partly to be due to an intrinsic connective tissue defect in the skin of such patients. The auteurs put forward the hypothesis that also present calf muscle pump dysfunction in these patients is the result of a connective tissue defect of the fascia of the muscular compartment, as an expression of a more generalized connective tissue defect.

A 22 yr old woman with bilateral symmetrical enlargement of her lower extremities since the age of 11 is reported. A diagnosis of lipedema of the legs was made on the basis of history, physical examination, biopsy and phlebography. Lipedema of the legs should be included in the differential diagnosis of symmetrical nonpitting edematous lower extremities. According to Allen and Hines, the characteristic points to be made for a diagnosis of lipedema of the legs included the following: almost exclusively seen in women; always bilateral and symmetrical with minimal involvement of the feet; minimal to absent pitting edems; all parts of the limbs are involved simultaneously; persistent enlargement despite elevation of the extremities. 16% of their patients gave a family history of the disorder; 40% complained of pain in the lower extremities; and approximately half of the patients were obese. The age of onset was variable, from childhood to the sixth or seventh decade. There was no racial preponderance. No patient gave a history compatible with progressive lipodystrophy. Treatment included diet, diuretics, tight stockings, rest and elevation, and massage, but was unsatisfactory in most cases.

Lipoedema was first described by Allen and Hines (1940), and it is characterised by fat legs and orthostatic edema. Generalised obesity may be presend or absent, the mean weight of five illustrative patients in their paper ws 154.5 lbs. Allen and his co-workers (1951) then reported 119 cases of lipoedema at the Mayo Clinic from 1937 to 1946. The condition affects women almost exclusively. Hines (1952) states that the diagnosis of lipoedema can be made easily from observation: (1) The characteristic symmetrical distribution of fat in the lower half of the body, excepting the feet, and (2) the oedema of varying degrees in the more dependent portions of the legs. The condition is briefly mentioned by Martin et al. (1956). The condition is often confused with vascular diseases affecting lower extremities, and lymphoedema (Wold et al., 1951). Furthermore, these workers consider that lipoedema can be distinguished from lipodystrophy progressiva by the extensive loss of subcutaneous fat in the upper half of the body associated with deposition of fat in the buttocks and lower extremities (Whittle, 1944). Two patients are presented with lipoedema, associated with diabetes mellitus. The possibility that lipoedema may be a variatn of lipodystrophy progressive is discussed.