Finally, as a more advanced type with greater affectation, Lipo-Lymphedema may appear, in which lipedema (accumulation of adipocytes) is associated with lymphedema (accumulation of lymphatic fluid). The affectation of feet below the ankle is characteristic, with swelling of liquid on the sides and below the ankles.
In our everyday clinical practice we have sometimes observed variations of these types, such as lipedema in the thighs and calves not affecting the hips.
1. Bilateralism
Lipedema is always bilateral.
However, thorough examination will show that one of the limbs is always slightly more affected than the other. Patients occasionally report clearly that one of the legs is more painful and swollen than the other.
2. Pain and increased sensitivity
Lipedema might be painful on deep palpation and might cause discomfort on examination by superficial touch.
Although lipedema is described as painful in most studies and papers, in our experience, lipedema is not always painful, especially in its early stages and first years of progression.
Nevertheless, patients frequently refer swelling and discomfort in the legs, even when there is no pain.
It is very common that swelling and discomfort increase with heat and menstrual periods.
3. Disproportion
Lipedema typically causes disproportion between the upper and lower halves of the body.
It generally starts in the lower limbs. Seldom will lipedema affect only the arms.
It most commonly starts from hips to thighs and/or knees (60%), but it is also common from hips to ankle (30 %). Less than 10 % of the cases are Knee to ankle.
4. Fat pads of lipedema.
From Stage II of lipedema, increase in fat is observed at the inner and back parts of the knee. Fat pads of lipedema.
5. Sign of Cup (Sign of CUFF)
When lipedema affects the lower half of the legs, the deposits of fat end abruptly just above the ankles, creating a kind of “cup rim” which has been called the Sign of Cuff or the Sign of Cup.
If lipedema progresses into lipo-lymphedema the area below the ankles is affected too. The Sign of Cuff begins to disappear due to swelling of the feet.
6. Sign of medial below the knee joint compression (Simarro’s sign)
It is a pathognomonic sign of lipedema. (If it is not present it means it is not lipedema). It appears even at early stages when nothing hints lipedema.
It is present even when lipedema does not affect the lower half of the legs. It is of utter importance to determine which is the medial below the knee joint area and carry out a skin pinch. If it is painful it is lipedema.
Any other fatty area must be pinched with the same degree of intensity and depth, in order to check that it is not painful.
This sign is already present at the early stages of lipedema. It is really important to enable early diagnose of the disease when everything “seems normal”.
As we have already mentioned, one of the limbs will be more severely affected than the other. That is why pinching would be more painful in the most affected limb.
7. Cellulitis above the kneecap
Whenever intense cellulitis is visible above the kneecap and in the thighs, lipedema must be suspected and ruled out.
Cellulitis above the kneecap generally produces folds of fat which drop over the kneecap, covering it almost completely.
8. Inheritance
Lipedema is a hereditary disease which affects women mainly.
It might not affect every family member but it is always transmitted.
Most studies state that there is a family history of lipedema in 16%-45% of the cases.
Our hypothesis is that lipedema is a hereditary condition in all cases. The reason why a family history of lipedema might not always be found is that the condition occasionally, skips 1 or 2 generations. Besides, it might also be difficult to find a family history both in small families and in families with a reduced number of women, although lipedema might have existed.
In our clinical practice we have seen cases in which a mother suffers from Stage I lipedema whereas her daughter suffers from Stage III lipedema. This mother would have never been diagnosed if her daughter had not been affected by the condition.
Another reason why sometimes is difficult to find a family history of lipedema is that although lipedema is hereditary, the Stage is not. (Both parents suffering from lipedema might mean a higher likelihood of suffering a more advanced Stage of lipedema).
Finally, lipedema is related to eating habits. Advanced Stages of lipedema are linked to overweight and obesity. A patient with unhealthy eating habits might undergo more advanced stages of lipedema than her ancestors with good eating habits.
9. Capillary fragility.
Erythematous areas present more capillary fragility. Because of this, from Stage II lipedema onwards, it is extremely infrequent not to find varicose veins. Besides, it is very common that spider veins are present at ´fat pad` level.
On the other hand, capillary fragility means bruising easily even due to minor traumatisms.
10. Non-pitting edema.
If you press on a swollen area and an indentation or pit remains, it is called pitting edema. It happens because the skin and underlying fat tissue has excess fluid trapped, causing edema.
Pitting edema occurs in conditions which involve significant fluid retention.
As lipedema is a collection of fat with an increase in number and volume of adipocytes, fluid retention would not be severe enough to cause pitting edema.
Except in the case of Stage IV (Lipo-lymphedema) pitting edema will not be common, being a clear ground for distinguishing between lipedema and lymphedema in which is usual.
CURRENT LIPEDEMA TREATMENTS
1. Nutritional treatment
Due to poor knowledge of lipedema it is very frequent that patients are treated with diets.
Lipedema responds badly and poorly to weight loss. Dieting patients do not lose weight evenly from their whole body, but only from the upper part. Paradoxically, there are women with anorexia with enlarged lower limbs.
Weight loss from dieting does not have a good aesthetic outcome in lipedema.
On the other hand, even through bariatic surgery (Gastric Reduction or Roux-en-Y Gastric bypass) weight loss does not always occur in the area affected by lipedema.
2. Physiotherapy and Sport
Manual lymphatic drainage, mechanical lymphatic drainage, decongestive theraphy and/or compression therapy are the most common treatments. They are usually effective in the medium and long term. They are not curative, which means treatment must be maintained and repeated indefinitely.
On the other hand, exercising may exacerbate pain in the lower limbs and fluid builds up in the supramalleolar area. Because of this, many patients with lipedema are reluctant to do exercise. Only swimming is accepted by these patients, however, it has poor effectiveness as a treatment.
3. Surgical treatment
Currently, surgical intervention is done by Tumescent Liposuction and/or by Water-Jet Assisted Liposuction (WAL). In both cases, using techniques, medical equipment and supplies different from the ones typically used in liposuction. This technique has been developed by German surgeons who have the greatest expertise using it.
Thanks to this technique, the lymphatic ducts and nodes are not damaged during liposuction.
According to the latest studies, the surgery achieves a clear improvement in the short and medium term (4-8 years). However, the same studies suggest that there might be a relapse in the long term in untreated areas..
Years after leg surgery, lipedema may appear in other parts of the body. Our experience with ultrasound diagnosis shows that fat in lipedema usually has a wider distribution than it may seem in an initial clinical assessment. (It may affect the lower half of the abdomen and buttocks).
The number of procedures required will depend on the amount of fat to be removed. Usually between 2 and 6 surgeries may be needed, spacing out the procedures from 1 to 6 months.
Any surgical technique (except W.A.L) is absolutely inadvisable in lipo-lymphedema and in advanced Stage III cases. Even W.A.L should not be performed without a previous weight loss treatment. Likewise, W.A.L will not eliminate the lymphedema component in lipo-lymphedema, however, it will improve it.
Using conventional liposuction techniques might worsen the condition if the surgery affects the lymphatic ducts. This is especially likely to happen in liposuctions in which fat close to the lymphatic chains (inner thigh and inner and outer calf) is removed.