Lipedema

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Nosology and clinical pictures

Lipedema can be defined as a localized swelling of the lower limbs, which is bilateral, symmetrical, with a soft consistency, that develops due to the build-up of adipose tissue, in a “chaps-style” fashion, namely starting from the hips down to the ankles. Lipedema is a nosological entity with unknown aetiology, which, prevailingly, affects the female population. According to recent epidemiological studies, it affects 11% of women. It frequently has its onset when the girl has her menarche, or during menopause, or pregnancy. Despite a low incidence in men, some cases have been reported that are characterized by a significant sex hormone dysfunction and/or liver function impairment. In particular, some authors have reported a higher incidence of lipedema following head traumas and surgeries for pituitary adenomas.

Lipedema may either progress upward or downward. It normally begins around the hips and thighs, and progresses to the lower limbs. Conversely, the typical sign of lipedema - edematous-fibrosclerotic panniculitis – has a much later onset. In advanced cases, particularly in elderly, non-treated patients, subcutaneous nodular formations may be observed, that result out of sclerotic processes of the subcutaneous connective tissue. In severe cases, immobility is the direct consequence of elephantiasis of the lower extremities. The skin generally has a normal color, except for those cases in which lipedema is associated with a particular form of erythrocyanosis (erythrocyanosis crurum puellarum). In some cases, hemispheric adipose pads are present at the level of the knee joint. Occasionally, lipedema of the lower limbs is accompanied by lipedema of the upper limbs, with swelling typically spreading from the shoulders down to the wrist. In some rare cases, conversely, lipedema affects the upper extremities only.

Some other significant features characterizing lipedema are described here below.

  • • Edematous fluid often builds up in the affected extremity, in particular in the hot season and in the second half of the day. The Lipedematous region has a soft consistency, painful when manually pressed. Conversely, healthy individuals or lymphedema patients feel no pain under this manual pressure.
  • Small traumas that normally leave no signs in healthy tissues can cause bruises in the lipedematous region, or haematomas in subcutaneous adipose tissue.
  • Lipedema is a frequent cause of strong emotional distress: the patient feels uncomfortable with her unpleasant and unshapely look, which has a negative effect on her quality of life and may cause depression, which, in turn, leads to additional complications.

Physiopathology

Adipose tissue is a particular type of connective tissue formed by adipose (adipocytes) cells, which, by joining together, form lobules separated by fibrous connective tissue partitions. Each single adipose cell has its own blood supply through a capillary system, as well as its own innervation mediated by adrenergic nervous fibers. Further, it is maintained that adipose tissue also has its own sensory innervation. Lymphatic papillaries are present at the level of the fibrous partitions, but not between adipocytes. Few reticular fibers between adipose cells make up the initial (or pre-lymphatic) lymphatic drainage system.

Microangiopathy, which takes place in the adipose tissue leading to higher protein permeability and, at the same time, more fragile capillaries, is one of the triggering mechanisms of lipedema. As a consequence of higher permeability, high-protein content fluid builds up in the surrounding cell area, while bruises are due to higher capillary fragility.

The soft consistency of adipose tissue may be associated with neurogenic inflammation, that further worsens the microangiopathy. Some authors believe that this extremely soft texture is caused by a damaged autonomic nervous system. According to this theory, this dysfunction could lead to a wrong interpretation by the body of protopathic sensory inputs (linked to pressure, temperature, or posture), which is a well-known condition, for example, in Sudeck-Leriche Syndrome.

Pericellular fluid build-up is associated with a dilatation of the pre-lymphatic drainage system, thus allowing only a very slow fluid outflow through initial lymphatic vessels. A typical pathologic impairment also involves cutaneous lymphatic capillaries. In particular, the capillary wall is highly permeable. Some investigations with fluorescent microlymphangiography have shown aneurysm-like formations in association with evidence of numerous capillary segments with an extremely small gauge.

It is thus possible to understand that dilated pre-lymphatic vessels, coupled with morphological and functional dysfunctions of lymphatic capillaries, may progressively damage lymphatic drainage functions. In addition to the above, dysfunctions in lymphangion (which is the anatomic-functional unit of the lymphatic collector, namely the segment of lymphatic vessel located between two valves) motor activity have also been observed. In early-stages of the condition, assessed by lymphography using oil-based iodinated contrast agent, suprafascial lymphatic collectors with a wavy, so called “corkscrew” shape have been observed. In cases associated with elephantiasis, progressive immobility caused by the disease has a damaging effect on lymph formation and on lymphangion motor activity. Also, isotopic lymphography has shown that the lymphatic pump ages more quickly.

Furthermore, in lipedema, skin resiliency is severely decreased, while skin compliance (skin stiffness, expressed in mmHg, measured with a special device) is increased. All this has the following severe consequences:

  • The skin loses its helping role as a venous pump in the lower extremities. In healthy individuals, when in standing position, venous pressure in the back of the foot is 100 mmHg; during walking, this pressure drops to 30 mmHg. This mechanism fails to function properly in lipedema patients. Hence, the resulting passive hyperemia increases ultrafiltration volume. Due to higher skin compliance, a higher volume of interstitial fluid is necessary to raise interstitial pressure. Therefore, lymphatic drainage function is further impaired and, as a consequence, a key mechanism against edema development fails to work.
  • Another major factor leading to disease progression is the absence of any venoarteriolar reflex. In healthy individuals, this reflex normally implies vasoconstriction in upright standing posture: in turn, the ensuing reduction in the perfused capillary area decreases the ultrafiltrate volume in the lower extremities. This reflex, which is lacking in lipedema, is a major defense mechanism against the development of edemas.
  • Macrophages, which can scavenge plasma proteins outside lymphatic vessels, are rarely present in adipose tissues. For this reason, fibrosis between adipocytes quickly develops with formation of collagen fibers.

Pitting edemas - leaving a dent in the skin after pressing the area with a finger - often develop in lipedema, during the second half of the day and when the weather is hot. The reason being that higher temperature leads to reactive hyperemia, which, in turn, increases the lymphatic fluid load. If a water retention edema is associated with lipedema, lymphatic vascular failure becomes manifest. Indeed, a good lymph outflow is enhanced by properly functioning valves in the lymphatic system, thus preventing gravitation reflux. Therefore, in lipedema, on one hand, the lymphatic load – e.g. water and proteins - is increased, while, on the other hand, lymph formation and lymphangion motory activity are impaired, and, on top of this, the lymphatic valvular system is severely insufficient.

Complications and association with other diseases

Complications linked to patients:

  • Anorexia nervosa
  • Bulimia

Complications caused by physicians:

  • Diuretics/laxatives
  • Lipectomy
  • Liposuction
  • Sclerotherapy (varicose veins)
  • Varicectomy without absolute indication
  • Gastric banding, gastric bypass

Natural complications:

  • Lipo-Lymphedema
  • Lipedema + idiopathic cyclic edema syndrome
  • Lipedema + arthrosis
  • Lipedema + chronic venous insufficiency

Differential diagnosis

 

Lipedema

Lymphedema

Lipolymphedema
Lympholipoedema

Unilateral

No

Yes

No

Bilateral

Yes

Possible

Yes

Bilateral-Symmetrical

Yes

Rare

Rare

Bilateral-Asymmetrical

No

Frequent

Frequent

Foot

No

Affected

Affected

Stemmer sign

Negative

Positive

Positive

Skin folds

Normal

Deep

Deep

Softness

Yes

No

Yes

Bruises

Yes

No

Yes

Erysipelas

No

Yes

Yes

Angiosarcoma

No

Possible

Possible

Therapy

It is worth underlining that lipedema is not exclusively a patient’s “subjective problem”, nor is it only an issue of excessively fat lower limbs, nor is it a mere constitutional change, as it is sometimes maintained: it is rather a disease that needs to be properly treated in order to avoid likely complications.

Combined Decongestion Therapy (CDT) - featuring manual lymphatic drainage, the use of adequate elastic compression and/or proper short stretch bandages, therapeutic exercises, and a careful skin hygiene - is not initially recommended when lipedema is associated with obesity, which, in turn, is generally associated with some major anatomic-functional dysfunctions of the cardiovascular, pulmonary, and muscular-skeletal systems and, in particular, with arterial hypertension, congestive heart failure, diabetes mellitus, hyperlipidemia, Pickwick syndrome, arthrosis of the hip, knees or feet. Therefore, in those cases in which lipedema is associated with obesity, CDT can be started after having duly treated all the other clinical conditions linked to obesity, not only with medical-conservative therapies, but also through an adequate weight loss program, as well as, and in particular, with surgery. As a matter of fact, treating obesity is essential for CDT, associated with microsurgical treatment, to be successful (Figure 1,A-B).

Surgical treatment of obesity has experienced a real breakthrough following the design and introduction of Biliopancreatic Diversion (BPD) by Professor Nicola Scopinaro and his collaborators of the Clinical Surgery of the University of Genoa. This approach has significantly improved the outcome of obesity surgical treatment, because it provides an actual solution to this disease, through a functional regulation of food intake and energy absorption. Significant weight loss is associated with stable glycemic values and cholesterol levels in the totality of cases.

Figure 1, A-B: Elephantiasis of the right lower limb, in obese patient, treated with combined decongestion therapy and derivative lymphatic-venous microsurgery, coupled with appropriate obesity treatment.