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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.

METHODS: Twenty-four healthy subjects and 16 patients with lymphedema and lipedema were studied with MRI and ultratomography. RESULTS: In chronic lymphedema, ultrasonography revealed a statistically significant increase of the subcutaneous fat without difference in skin thickness as compared to the healthy subjects. MRI revealed in lymphedema a statistically significant increase of skin thickness + subcutaneous tissue + muscular mass (p = 0.048); in lipedema, a statistically significant increase of skin thickness and subcutaneous tissue (p < 0.0001) as compared to the healthy controls. CONCLUSIONS: MRI offers strong qualitative and quantitative parameters in the diagnosis of lymphedema and lipolymphedema, while ultrasonography is expected to improve its diagnostic efficiency with the aid of high frequency echo with more sophisticated resolution apparatus. Age, weight and height of the patient as well as duration of the disease do not seem to affect the above-mentioned parameters.

15% of the patients of a special clinic for lymphological diseases had a lipedema. In 97% it was located in the legs and in 31% also in the arms. In 66% it was located only in the legs and in 3% only in the arms. Combinations of lipedema of the leg with phlebedemas have been seen in 2% and with a lymphedema in 1%. The differential diagnosis to lipohypertrophy, adiposis and lymphedema is given. The therapy with liposuction and physical therapy of edema, combination of manual lymphatic drainage and compression, will be discussed.

Early terms of lymphostasis in lipedema can be detected with lymphoscintigraphy. A normal examination almost certainly excludes a lymphatic component. Indirect lymphography is only used to rule out morphological abnormalities of lymph vessels. If a lymphoscintigraphic study is normal indirect lymphography is not indicated.

The "Lipedema" or "Fatedema" is conditioned by a slight mechanical obstruction of the small lymphatic vessels by the increasing pressure of the growing fat tissue. This lymphostasis in a normal lymphatic vessel system arises only with women and always symmetrically and conducts to typical complaints. Therapeutically, apart from loss in weight, only lymph drainage therapy is in a position to remove the complaints of edema.

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.

In a review of 250 cases of lymphedema of the lower extremity, 9 patients were noted to share unique similarities in their history and physical findings. Although these patients had mild swelling in their pretibial areas and were all referred with a diagnosis of lymphedema of the legs, their findings differed significantly from the usual patient with either congenital or acquired lymphedema. Notably, the lower extremity swelling was always bilateral and symmetrical in nature and never involved the feet. Skin changes characteristic of lymphedema were not found, and consistent fat pads were present anterior to the lateral malleoli in each patient. These findings are representative of a clinical entity known as lipedema, which is distinct from lymphedema and for which treatment may be different.

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.

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 txochanteric 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. Nearly all patients have severe feelings of inferiority because people mock and laugh at them; thus, the alimentary component of the obesity is a form of compensation for lack of love. By dieting, the adiposity disappears only partly because the typical deformity of lipodystrophy remains. I believe that in the lipodystrophy patient a lipedema may develop resulting from lymphatic and venous disease, which may arise later in life. Younger patients' complaints are mainly psychological and result from a disturbed body image, whereas older patients are troubled by pain and leg weariness. Treatment consists of dieting, subcutaneous lipectomy by liposuction, and prescription of elastic stockings.

An elderly patient with a swollen leg or legs can present a challenging problem in differential diagnosis. Although most swollen legs are the result of congestive heart failure, thrombophlebitis, cellulitis, or lymphedema, many other conditions should also be considered.

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.

Leg swelling is often of edematous nature. The most important differential diagnosis lies in the distinction between venous or lymphatic forms of edema. An increased vascular permeability and alterations in blood composition have also to be taken into account. A particular entity is the lipedema. Next to an accurate history, specific inspection and palpatory criteria permit to distinguish the various forms. Tests for venous function, laboratory and technologically investigative techniques increase diagnostic accuracy. Lymphedema can only be diagnosed by an exact clinical diagnosis.

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)