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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).

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.

PURPOSE: To evaluate the accuracy of computed tomography (CT) scan imaging in distinguishing lymphedema from deep venous thrombosis (DVT) and lipodystrophy (lipedema) in patients with swollen legs. MATERIAL AND METHODS: CT scans of the lower limbs were performed in 55 patients with 76 swollen legs (44 lymphedemas, 12 DVT and 20 lipedemas). Thirty-four normal contralateral legs were also similarly evaluated. Primary lymphedema was verified by lymphography or lymphoscintigraphy, whereas secondary lymphedema was documented by a typical clinical history. DVT was established by ultrasound Doppler imaging. The diagnosis of lipedema was made with bilateral swollen legs where lymphoscintigraphy and Doppler examination were both unremarkable. Qualitative CT analysis was based on skin thickening, subcutaneous edema accumulation with a honeycombed pattern, and muscle compartment enlargement. RESULTS: Sensitivity and specificity of CT scan for the diagnosis of lymphedema was 93 and 100%, respectively; for lipedema it was 95 and 100%, respectively; andfor DVT it was 91 and 99%, respectively. Skin thickening was found in 42 lymphedemas (95%), in 9 DVT (75%), and in 2 lipedemas (16%). Subcutaneous edema accumulation was demonstrated in 42 legs (95%) with lymphedema and in 5 (42%) with DVT but in none with lipedema. A honeycombed pattern was present only in lymphedema (18 legs or 41%); muscle enlargement was present in all patients with DVT, in no patient with lipedema, and in 4 (9%) with lymphedema. CONCLUSION: Edema accumulation is readily demonstrated with plain CT scan and is not present in lipedema. Specific CT features of the subcutaneous fat and muscle compartments allow accurate differentiation between lymphedema and DVT.

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.

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 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.

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.