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Madelung's Disease...

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Benign Symmetric Lipomatosis
Madelung's Disease
Lanois-Bensaude Syndrome

MSL, Multiple Symmetrical Lipomatosis, also known as Benign Symmetric Lipomatosis or Madelung's Disease and Lanois-Bensaude Syndrome is a metabolic condition characterized by the growth of fatty masses around the face, back of the head, neck, upper arms, abdomen, back and upper leg in a very specific pattern or distribution. Unlike the usual lipoma, these benign, fatty masses are not enclosed within a membranous capsule with very distinct boundaries. It is because of this characteristic, as well as the absolute symmetry in their occurrence that the condition is often dismissed as simple obesity.

There are, however, other systemic manifestations which are part of this syndrome, but they tend to be treated as discrete medical problems. This often leads to simultaneous medical investigations and possible treatment by a wide variety of specialists. Quite often, lab tests fall within normal ranges. This leads to the frustrating and demoralizing situation of feeling ill and weak, with no identifiable cause. In addition there is a steady increase in weight, despite all attempts at dieting, which often leads to the judgment of "a non compliant patient." This, and the frequent reference to psychological explanations leads to an escalating sense of fear and stress which only worsens the condition.

Some of the other physical symptoms aside from the peculiar weight distribution include: neurological problems in the upper and lower extremities, including weakness, and areas of anesthesia (decreased sensory feeling) and paresthesias (strange sensations as if something were crawling over the skin, or burning liquid were running down an extremity;) as well as autonomic nervous system manifestations such as unusual flushing and sweating (particularly after eating,) wide fluctuations in blood pressure and heart rate, adult onset asthma, glucose intolerance, gastrointestinal problems (particularly nocturnal diarrhea), and foot problems such as ulcers on the plantar surface of the foot or spontaneous fractures of small bones.

Up until now, the condition has been regarded as very rare, with the highest incidence reported in males and in particular those with alcoholic background. There have been some cases reported among women as well as in nonalcoholic males and females. Since, however, the condition seems to manifest itself more during mid life, an obvious question is how many women have this condition with their medical problems being disregarded as psychosomatic or just manifestations of menopause.

Many physicians have not heard of this condition. Some who have, regard it as primarily a cosmetic entity, with relief only achieved by liposuction or other surgical procedure to "debulk" the fatty masses. In many instances, surgical intervention is complicated by the tendency of these lipoma to be non encapsulated, invading the tissue and structures around them. The tumors also tend to be more vascular than normal adipose tissue resulting in more bleeding than one might suppose as well as post operative oozing of sero-sanguineous fluid. In some instances after debulking surgery, the tumors tend to return over a relatively short period of time.

The lipomatous masses which are highly vascular, have a rubbery consistency and a propensity for manifesting their presence on the left side of the body prior to the right. Areas around the head and neck are the primary sites with those on the torso developing as the condition progresses. Occasionally, the extremities are involved, usually the upper arm and upper leg. In some instances the lower arm on the anterior surface is involved. Rarely if ever are the fatty masses seen below the knee.

Because of the symmetry, regular physical exam may not identify the condition. The location of the masses, a history of weight gain, even in a reduced calorie intake, extreme fatigue and muscle achiness, unexplained pain over the muscles, hypersensitivity to touch or minor trauma, weakness of the extremities and severe unexplained cramping over the sites where the lipomatous masses most commonly occur are indicators that there is cause for further investigation.

Blood studies and tend to be quite normal. CAT scan and MRI (in particular) demonstrate the presence of the masses. Biopsy of a fatty mass with underlying muscle tissue, and the identification of specific pathology in both, is significant in confirming a diagnosis.

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