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"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.

OBJECTIVES: The purpose of this study was to define the relationships between the short saphenous vein (SSV) and the fasciae of the leg, including the muscular fascia (MF) and the membranous layer (ML) of the subcutaneous tissue. METHODS: Fascial relationships of the SSV were evaluated by means of dissection in 30 cadaveric limbs and by means of duplex sonography in 270 healthy limbs from living subjects. RESULTS: All along the leg, the SSV courses in a flat compartment delimited by the MF and the ML. Neither results from dissection nor results from sonographic examination demonstrated piercing of the MF by the SSV. A hyperechoic lamina similar to a ligament connects the SSV to the fasciae by which it is encased. An SSV tributary and collateral vessels course out of this space and are devoid of any fascial wrapping. CONCLUSIONS: The SSV does not correspond to the classical description of a "superficial" vein. In fact, from the anatomical point of view, the SSV is an interfascial vein, because it is encased by two connective fasciae, just like the greater saphenous vein. Fascial relationships of the SSV suggest that muscular contraction potentially influences the caliber and hemodynamics of the SSV. In addition, the ML is arranged as a sort of mechanical shield that could counteract dilative pathologic conditions in varicose limbs.

Venous oedema, which occurs almost exclusively in the legs, develops due to increased venous pressure following valvular insufficiency, and is marked by blue discolouration, varicosities and, in chronic forms, by brown discolouration of the skin resulting from the deposition of haemosiderin. It is possible to confirm the condition by phlebological instrumental diagnosis. Primary lymphoedema also almost always occurs in the legs. It is caused by underdevelopment of lymphatic vessels, but the skin colour remains normal. It is recognisable by Stemmer’s sign and characteristic thickening of the skin over the toes resulting from subcutaneous protein fibrosis. Lymphoscintigraphy may be required for diagnosis in rare cases. Venous oedema and lymphoedema are found in both sexes, uni- or bilaterally. If bilaterally then usually asymmetrical. In contrast, lipoedema occurs in women only, thickening is always symmetrical, and the skin has a normal colour as in lymphoedema. The predisposing condition for lipoedema is lipohypertrophy of the extremities, a congenital accumulation of adipose tissue in the extremities that results in a disproportionate physical form with a relatively slim trunk. In one third of cases lipoedema is also observed in the arms. In contrast to lymphoedema, the hands and feet characteristically remain free of thickening and oedema.

Diagnosis and treatment of the <<Lipedema Syndrome>> are now as ever connected with problems for the general physcician, but also for the experts in lymphology. Numerous open questions regarding etiology, diagnosis, and course of the disease as new therapetic concepts have lead to the idea, to include that subject again as one of the main topics into the program of the annual meeting of the German Society of Lymphology

The role of operative management of "symptomatic" varicose veins in patients with lower extremity lymphedema or lipedema is controversial. We reviewed the clinical outcome of 261 patients between 1989-1997 at the Földiclinic with lower extremity lymphedema (68 patients), lipo-lymphedema or lympho-lipedema (103 patients) or lipedema (90 patients) who had undergone operation for varicose veins. In each group, the results were dismal as leg swelling worsened or was unchanged in greater than 90% whereas symptoms such as heaviness, fatigue, cramps (termed varicogenic symptomatology) were improved in less than 10%. These findings support that operations for varicose veins in the legs of patients with lymphedema, lipedema, or combinations of these disorders should be undertaken only if there is an absolute indication present (ascending phlebitis and/or bleeding). Otherwise, complete decongestive physiotherapy is still the best treatment approach for these groups of 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.

In order to develop further the criterias of the ultrasonographic diagnosis of the lip- and lymphedema, also in the demarcation of the phlebedema, we examined 100 patients in our practice. In addition to the specific “sonomorphology”of the lip- and lymphedema we found a different reaction of the subcutaneous tissue of the lower leg in painful and not painful lipedemas examined with compression-sonography. A more precise demarcation of the cutis from the subcutis was made possible with a new 13 MHz linear probe. The spontaneous painful lipedema shows a thickened subcutis with increased echogenity and is only compressible at 10 to 20 percent, while the not painful lipedema is compressible at about 50 percent. In patients with lymphedema the typical echoless gaps showed no colour coding and were also not compressible. Especially because of the low time needed, the non-invasive method and the lack of radiation the ultrasonographic description, measurement and compression of the subcutaneous tissue of the lower leg is useful for diagnosis, the objective control of therapeutic strategies and the estimation of the clinical symptoms – especially in cases of lipedema – and also for the etiology – especially in cases of lymphedema. The sonography is superior to the clinical examination and should be the second step in diagnosis of all nonsystemic forms of edemas of the legs.

Lipedema represents a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs with consecutive development of lymphostasis and lymphedema. The aim of this article was to review one clear case of lower limbs lipedema, of unusual occurrence and appearance, which was associated with dermatomyositis. A moderately manifested lipedema in 8 years old little girl was reported with its expressive segmental distribution to upper and lower legs, without significant increase in its size during last 10 years and without signs of lymphostasis. The hereditary influence was not confirmed. Histological examination of lipedematous tissue revealed significant presentation of immune component of the disease. According to the available literature, association between lipedema and dermatomyositis, lower limbs lipedema with segmental distribution as noticed above and its appearance as a consequence of corticosteroid therapy have not yet been published.

A major cytogenetic subgroup among human lipomas is characterized by translocations involving the HMGIC gene at 12q15. In the context of an ongoing research program aiming at the elucidation of the functional consequences of HMGIC translocations in the etiology of lipomas, we have isolated a novel human gene, LHFP (lipoma HMGIC fusion partner), that acts as a translocation partner of HMGIC in a lipoma with t(12;13). The LHFP gene was mapped to the long arm of chromosome 13, a region recurrently targeted by chromosomal aberrations in lipomas. By Northern blot analysis, a transcript of 2. 4 kb was detected in a variety of human tissues. We assembled a cDNA contig containing the entire coding region of LHFP. Nucleotide sequence analysis of the composite LHFP cDNA revealed an open reading frame encoding a protein of 200 amino acids. The predicted human LHFP protein is almost identical to a translated mouse EST that covers almost the entire LHFP coding region. In addition, BLAST searches revealed that the LHFP protein belongs to a new protein family consisting of at least four or five members. In the lipoma studied, the expressed HMGIC/LHFP fusion transcript encodes the three DNA binding domains of HMGIC followed by 69 amino acids encoded by frame-shifted LHFP sequences. LHFP is the second translocation partner of HMGIC identified in lipomas and represents a candidate target gene for lipomas with 13q aberrations.

The sign of the thickened cutaneous fold of the second toe is typical for the early and differential diagnosis of a primary ascending lymphedema without false positive findings. It appears in the late stages of the descending lymphedema.

The anatomical and functional status of the epifascial and subfascial lymphatic compartments was analyzed using two compartment lymphoscintigraphy in five groups of patients (total 55) with various forms of edema of the lower extremities. Digital whole body scintigraphy enabled semiquantitative estimation of radiotracer transport with comparison of lymphatic drainage between those individuals without (normal) and those with leg edema by calculating the uptake of the radiopharmaceutical transported to regional lymph nodes. A visual assessment of the lymphatic drainage pathways of the legs was also performed. In patients with cyclic idiopathic edema, an accelerated rate of lymphatic transport was detected (high lymph volume overload or dynamic insufficiency). In those with venous (phlebo) edemas, high volume lymphatic overload (dynamic insufficiency) of the epifascial compartment was scintigraphically detected by increased tracer uptake in regional nodes. In patients with deep femoral venous occlusion (post-thrombotic syndrome). subfascial lymphatic transport was uniformly markedly reduced (safety valve lymphatic insufficiency). On the other hand, in the epifascial compartment, lymph transport was accelerated. In those patients with recurrent or extensive skin ulceration, lymph transport was reduced. Patients with lipedema (obesity) scintigraphically showed no alteration in lymphatic transport. This study demonstrates that lymphatic drainage is notably affected (except in obesity termed lipedema) in various edemas of the leg. Lymphatic drainage varied depending on the specific compartment and the pathophysiologic mechanism accounting for the edema. Two compartment lymphoscintigraphy is a valuable diagnostic tool for accurate assessment of leg edema of known and unknown origin.

The anatomical and functional status of the epifascial and subfascial lymphatic compartments was analyzed using two compartment lymphoscintigraphy in five groups of patients (total 55) with various forms of edema of the lower extremities. Digital whole body scintigraphy enabled semiquantitative estimation of radiotracer transport with comparison of lymphatic drainage between those individuals without (normal) and those with leg edema by calculating the uptake of the radiopharmaceutical transported to regional lymph nodes. A visual assessment of the lymphatic drainage pathways of the legs was also performed. In patients with cyclic idiopathic edema, an accelerated rate of lymphatic transport was detected (high lymph volume overload or dynamic insufficiency). In those with venous (phlebo) edemas, high volume lymphatic overload (dynamic insufficiency) of the epifascial compartment was scintigraphically detected by increased tracer uptake in regional nodes. In patients with deep femoral venous occlusion (post-thrombotic syndrome). subfascial lymphatic transport was uniformly markedly reduced (safety valve lymphatic insufficiency). On the other hand, in the epifascial compartment, lymph transport was accelerated. In those patients with recurrent or extensive skin ulceration, lymph transport was reduced. Patients with lipedema (obesity) scintigraphically showed no alteration in lymphatic transport. This study demonstrates that lymphatic drainage is notably affected (except in obesity termed lipedema) in various edemas of the leg. Lymphatic drainage varied depending on the specific compartment and the pathophysiologic mechanism accounting for the edema. Two compartment lymphoscintigraphy is a valuable diagnostic tool for accurate assessment of leg edema of known and unknown origin.