Lipedema - Page Two

Lipodema is a chronic disease of complex causes, many of which we understand little about. These include hormonal imbalance, an inability to metabolise exudate from blood vessels so that those proteins and cells that would normally be metabolised and returned to the circulation are deposited as adipose tissue in the subcutaneous tissue. The blood vessels themselves are affected, venous stasis and vasoconstriction occur and return is diminished especially at the subcutaneous level. This causes arterial constriction which accounts for the cold and often pale skin and leads to an increased lymphatic load(1). The condition worsens over years as tissue channels become progressively narrowed until the condition of a secondary lymphoedema, overlying the original lipodema, may lead to a situation where the patient may be either wheel-chair bound or bedridden. Weight gains can be up to or greater than 250 kgs. At this stage infections and intractable ulcers (or ones that necessitate skin grafts when lack of oxygenation to the skin causes problems with healing) which can be due to the simplest injury e.g. careless donning of a compression garment or other minor trauma, occur with an even higher frequency.

This condition occurs predominantly in women, and can in some cases be familial; the rare cases of men with lipodema always seem to include a hormonal imbalance which should be treated if possible. The macrophages themselves become overloaded and cease to play an active role in protein proteolysis and look like fat cells.

Lipodema is often misdiagnosed as obesity in its earlier stages but the symptoms are clear and distinctly recognisable clinically from this. In its later stages it maybe more difficult to distinguish from lymphoedema, although the case history and distribution of excessive tissue should provide an indication. The overlying condition of lymphoedema may occur in the later stages. Lipodema can occur in the legs, buttocks and also, but not necessarily, in the arms. It does not involve either the feet or hands until the onset of lymphoedema. The mean age of diagnosis is approximately 35-36 years but it usually starts at puberty. Symptoms of onset, however, can start in childhood, and may be clinically detectable in adolescence. Cellulite, which is a very mild form of lipodema, usually occurs towards or after menopause. It has been suggested that a mixture of primary lymphoedema as well as lipodema can occur in some cases.

Differential Diagnosis

Lipodema

There is symmetrical bilateral enlargement of limbs, both in legs (with buttocks involved) or in both legs and arms, but with the obvious enlargement excluding the feet and hands, until the later stages. Enlargement is a gradual but continuous process.
There is no pitting with pressure. The tissue feels more like "rubber", and is not as hard and fibrotic as in a grade II lymphoedema. It does not, in the early stages, reduce with elevation.

There is pain on pressure, especially in the medial aspect of the thigh and the base of the spine. As the disease progresses, pain is often caused by the lightest of touches to the skin, particularly later in the day. Pain is also caused by "self pressure" e.g. crossing the legs or sitting with pressure on the spine. The abdomen may also be painful to pressure.

Stemmer's sign is negative; i.e. a skin fold test done on the second toe. If it comes up as a thin fold test when "pinched", no lymphoedema is present. If it is a "lump" this indicates lymphoedema.

Superficial capillaries are easily damaged i.e. the limb bruises easily.

Skin temperature is lowered. Hands may feel clammy (damp), but limbs are cold. General nourishment of the skin is also affected and may cause "patchy" skin, dry in one place, oily in others in the early stages. Skin elasticity is reduced and it usually has an "orange peel" appearance.

"Pins and needles" are common and movement seems to alleviate these to a large extent. A feeling of dizziness may be present. There seems to be a reduction of venous return in the legs when standing which can lead to fainting; this may be prevented by walking.
There is little or no loss of weight with rigorous diet. Many of these patients have eaten low calorie diets for many years. In some cases their stomachs have been stapled because of misdiagnosis. In some of these cases, this seems to have lead to the onset of stomach cancer. It is not the answer to the problem! Obesity, caused by overeating, does respond to a proper dietary regime; lipodema does not.

Joint pains (especially in knees) are common.
Infection of the limb, either bacterial or mycotic is not normally a problem.

Plantar support is reduced i.e. people have fallen arches.
The shape of lipodema may vary, from a inverted "pear" shape (like a classical Greek column) to a more bulbous shape from the ankles upward. (Figs. 1-2.) This also usually involves the buttocks.
Lymphoscintigraphy i.e. time of clearance of a radio-tracer injected into the feet to the inguinal nodes, is normal.

Secondary Lymphoedema Accompanying by Lipodema in the later stages.

Stemmer's sign becomes positive.

Pressure will cause pitting, and there may be a small reduction with elevation.

Folds of skin will further enlarge and feet will swell (Fig. 4.). If arms are affected hands also will swell. If the top of the body is affected the shoulders, thoracic and neck area may be affected as well.

Infection may become a problem.

Diagnosis can normally be achieved by the taking of a careful case history and clinical observation (see above). In the later stages lymphoscintigraphy may clarify this, but the picture is so different from primary lymphoedema that this should seldom be necessary.

Psychological Problems

All of these patients present with a variety of these problems ranging from lack of self-confidence to lack of confidence in their medical or health workers, often because of misdiagnosis and lack of sympathy, and then to real depression and anxiety and because of their appearance and the lack of understanding of the condition, particularly as their mobility decreases. The whole problem, of course, becomes worse if the onset of lymphoedema further exacerbates the condition.

They are "blamed" for being overweight, told they eat too much or are "cheating" on their prescribed diets. If they are hospitalised for a weight loss program when the situation becomes very serious and they don't lose weight, they are often met by the comment "I am surprised" by their health care professionals and are summarily dismissed as "patients for whom nothing can be done" or "we don't know what is wrong with you" or "you'll just have to live with it". This is not helpful to the patient who should be made aware that the condition is a genetic abnormality and that their obesity is not their fault. Of course, up to a point dieting can help but it will never cure this condition. They obviously, and for good reason, become discouraged and dismayed by their problems, which seem not even to be recognised. When lymphoedema occurs on top of the lipoedema this is a situation which is almost a problem that is so great for them (and again often undiagnosed as such) that they need to be very strong people to cope with it. Sadly, many are not able to do this. Psychological counselling can be helpful; for this poorly understood condition it is seldom offered. Invalid Pensions are not the answer for those that have, until they could not, lead an active and productive life. In many centres in Australia and I am sure worldwide, these patients are turned away from treatment centres (for lymphoedema) as untreatable cases. In some cases they resort to surgery in a final effort to improve their condition. The result of some of these operations (including liposuction and limb reduction) are so appalling that they have to be seen to be believed.

Treatment

Lipodema can be treated and reduced with careful massage to the normal nodal groups after the truncal areas have been precleared i.e. the superficial inguinal and axillary nodes, then gentle superficial drainage towards these. and compression applied to the legs in the form of bandaging as garments. Although not as easy to treat as is lymphoedema, considerable reduction, easing of pain and improvement in mobility, can be of huge physical and psychological benefit to the patient. Compression bandaging is tolerable, especially after a few days of massage. The overlying lymphoedema, if present, can be greatly reduced. The improved mobility will increase the ability to exercise which will help the calf muscles pump and increase venous and lymphatic return. After the initial decongestion by manual drainage permanent compression causes a significant reduction in adipose tissue and also has a positive influence on the disturbed veno-arterial response.

Benzo-pyrones seem to help this condition considerably, presumably by their stimulation of macrophage numbers and activity. Many patients have reported a considerable weight gain when coumarin became unavailable in Australia. Interestingly, the Italian product CellaseneÔ which is recommended for cellulite, contains benzo-pyrones and other plant extracts that work in a similar way to benzo-pyrones, so despite medical scepticism, this may help in these conditions. Unfortunately the cost of these and other available benzo-pyrones are too high for many people who would benefit from them.

The only diet which may help is a very low protein only diet (250 mg per day) (and nothing else, except, of course, water), which will put the body into a state of ketosis where some of the excess fat may be metabolised. However this usually results in weight loss in already lean areas e.g. the waist and often the upper body. Operative procedures do not attack the cause of the problem. Careful liposuction may produce immediate reduction but considering the destruction of tissue it causes, long term results have not been clinically proven. Other reduction operations are contraindicated. Pumps are normally intolerable because of the pain they cause, and there is no published evidence of them ever being successful in this condition.

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