DERCUM’S DISEASE OR MORBUS (Mb) DERCUM

 

A considerable amount of patients suffering from Mb Dercum have often met with pain during a long time, and also consulted several doctors before finally getting the correct diagnosis.

 

Sometimes the diagnosis ”fibromyalghia” has been made, but that disease causes an obvious soreness over certain so-called “trigger points”, symmetrically placed above the muscle attachments on both halves of the body. Mb Dercum, however, shows a marked soreness in the flesh in general, and specially in the adipose tissue of the upper arms, the inner thighs and knees, the outer thighs (so-called riding-breeches thighs), the belly and the behind. Therefore the name of  ”fat tissue rheumatism” (adiposis dolorosa) has been introduced. Besides, Dercum patients often suffer from extreme pain in the skeleton.

 

Many women (only 5% men) have had to struggle to be believed, not the least by medical service and regional insurance offices. Why? The reason is that the medical service has strictly established routines into which you necessarily have to fit. In case your blood tests should show no abnormalities you will run the risk of being classified as mentally ill.

 

The prescriptions given are often without any effect on these symptoms, and the Dercum patient will most likely be considered as a hypochondriac, ”burnt out”, neurotic or some other suspicious diagnoses.

 

When a patient is complaining about ”pain in every limb” the doctors often presume that medicines against rheumatism will help. Normal painkillers, however, have little or no effect on the pain in the adipose tissue or the skeleton. If a painless condition should be achieved, drugs like morphine or similar medicines could be required.

 

The social insurance authorities have very often distrusted the patients. Without the correct diagnosis and lack of support from the medical care, as well as the authorities involved, it is not at all surprising that patients get depressed and sometimes need psychopharmacological drugs. Instead of support, reactions like: ”We were right. After all, the symptoms have psychological reasons”, are frequent. As the diagnosis is very often doubted, the patients may be denied their illness benefits, due to that the physicians, employed by the social security, do not believe the diagnosis given by the patients’ doctors. This will of course lead to severe economic problems for the patients.

 

The symptoms may aggravate in course of time. As a routine, patients are offered physical therapy. Unfortunately, this practice is of no use to the Dercum patient. Most patients with Mb Dercum has not shown any improvement. On the contrary, the symptoms are known to grow worse during the treatment, and some patients have not even been capable to return to work. The most important ethical rule for the medical care is “not to harm a patient”. It should be noted that the social security staff and its physicians very often lack medical responsibility and cannot even be reported to the Responsibility Council, if the experiment of rehabilitation ends badly. If the patient has got a faulty diagnosis, different damages (e.g. psychological) may be caused due to incorrectly prescribed treatment.

 

Chronic pain exists in many different types. One type derives from the tissues and could be blocked by e.g. morphine. This type of pain is Mb Dercum. Other types of pain are neurogene and psychogene. Patients with Mb Dercum or fibromyalghia are often classified as patients with psychological problems. However, a more realistic view can be seen today. The information about Mb Dercum in medical literature has during the years been extremely deficient. That is very astonishing as the Dercum disease was described for the first time in 1888, by the famous American professor of psychiatry, Francis Dercum. That happened even before the detection of fibromyalghia, which was described in Germany in about the year of 1900 and was re-detected in the US in about 1980. Professor Dercum was during his last years active as president Wilson’s physician.

 

Is has been reported that there are patients who show a remarkable recovery if taking “sugar pills” (placebo). However, these experiments on Dercum patients have not shown any effects at all on the pain. This has been certified in a randomised study that shows that Mb Dercum is not a self-imagined illness. Mb Dercum has been classified by the WHO (according to ICD-10) with the number of E88.2.  Mb Dercum is a typical female disease. Statistically, there are proportionally 20 women to one man, who have the disease. Mb Dercum often appears when the patient has reached 20-35 years of age, sometimes in the teens or in the patient’s middle age. Hence it is not an illness that starts in the menopause. Quite often it is inherited from grandmother/mother to daughter. Mostly the pain sneaks on the patient.  At an early stage of the illness the patient suffers from pain after minor efforts; later on the pain comes even during pauses. After a transitory period of local pain (e.g. in the hips, the upper arms and the small of the back), a general pain follows. A quick and prominent weight increase of about 20-30 kilos during the first year is not unusual. This signifies a great suffering for the patients, especially as knees and feet are afflicted, and they have also difficulties to walk because of the swellings on the inside of the thighs and knees, as well as the skeletal pain in the feet, lower limbs and thighbones.

 

It is not possible for these patients to loose weight. Several Dercum patients have tried to fast or follow the advice of Weight Watchers with little or no result. There also are patients who have had their stomachs reduced (VBG-operation) to loose weight in order to diminish the loading of the joints. The operation to loose weight is successful, but the patients still have their pain.

 

In Malmö-Lund a project with liposuction started in order to ease the pain. It had quite a good effect for about 80% of the patients. The results of this operation are under evaluation. Unfortunately a liposuction cannot give any relief for pains in the skeleton. On the contrary, some patients have reported that the pain in the skeleton has increased while the pain in the flesh has decreased. Before and after the operation measurements were made when the patient’s adipose tissue was “pinched” with a special instrument connected to a so-called dolorimeter till the pain became unbearable. Three months after this operation the pain was reduced to about 50%. Two years thereafter the pain had increased a little but was remarkably less than before the operation.

 

Not every patient gets overweight as an effect of Mb Dercum. Some patients suffer from pain in the already existing adipose lumps though they have no overweight. However, these lumps tend to increase. Associated systemic phenomena are e.g. chronic fatigue, Sjögren-like dryness, fumbling hands and fingers, swollen hands and feet, sleeplessness, stiffness, skeleton pain and a tendency to bruise easily.  Moreover, it is not rare that the patients develop a depression as people around constantly distrust them!  Occasionally there are patients who have periods with a very high temperature – up to 39ºC – and at the same time more pain.  Some patients often have a headache, difficulties with the memory and loss of concentration. It has also been noted that the risk of infections increases. The pain very often grows worse in course of time, especially on the thighs, knees, upper arms, belly and behind. Naturally it is difficult to move with a big, sore belly. If the patient has a work where it is necessary to sit down it is highly troublesome due to the pain in the behind and the caudal vertebra. Sometimes it is even difficult to go by bus or car.

 

The pain is not similar to that of fibromyalghia and above all it is worse. However, there are patients who have fibromyalghia as well. This fact has caused the doctors to believe that it is the same illness. The pain that is related to Mb Dercum is very intense, burning, shooting, smarting. Many patients describe the pain like “being scalded”, “walking on crushed glass” or “that the pain runs through the body”. Mostly the pain is symmetrical.

 

Common painkillers like Magnecyl (containing acetylsalicylic acid), Orudis (containing ketoprofen) or Voltaren (containing aceclofenac) have good effects on patients with Rheumatoid Arthritis  (RA) but not on the Dercum patients and often there is a tendency to gastric problems and even ulcers. Now there are new medicines without gastric influence. It must be clarified that the pain may increase if people around always distrust the patient. Therefore it could be good to take some light anti-depressive medicine just to stand the situation, although this medicine in itself does not ease the pain.

 

Many patients, but not all, feel relief by warmth and soft massage. Acupuncture is known to have only a slight or no effect. The pain increases by pressure or if wearing too tight clothes. Also during menstruation the pain is said to be worse. If the patients put on weight the pain gets worse. It can be local or diffuse.

 

Where then is the pain situated? By examining with palpitation it is clarified that the pain is definitely located to the fat. However, it can also be deeper in the organism over the skeleton and the periosteums. By removing some fat the pain diminishes markedly. But, nevertheless, it is difficult to say from where the pain emanates. Maybe the nerve fibres are bad. The pain increases by a light pressure and loading. It may be local, e.g. round thighs, knees and elbows, or diffuse and generalized in the fat. Sometimes it is evident in lipomas (adipose bulbs) that can be so sore that they have to be surgically eliminated. Many patients have an ordinary fat distribution over the body, like the women in Ruben’s paintings, while others have the so-called “riding breeches” fatness, which is easy to control by liposuction.

 

Overweight and pain in the fat and skeleton are not the only symptoms. The patients often suffer from dryness in their mouths and eyes, which rather frequently develops into a classical Mb Sjögren. Sometimes also inflammations round the tendons occur. Occasionally the pain can develop into fibromyalghia, but as the over-lying fat is very sore it is impossible to determine whether fat or tendons are aching. Thyroid gland functions (mostly under-function), migraine, aching ribs and ache round the heart (many patients have gone to the hospital, thinking that they have got a heart attack), irregular troubles in the large intestine, sore caudal vertebra, wrist pains and pains in the genitals often accompany Mb Dercum.

 

Overweight in itself is often a great handicap. Beside the wear and tear on the joints the Mb Dercum patients, due to the overweight, sometimes suffer from high blood pressure, diabetes and an increasing cholesterol value. The breathing may be insufficient followed by lack of oxygen. Soreness over the cranium, collarbones, breastbone, lumbar region and the tibias are also common. Pains in the jawbone make the patient think that there might be dental problems. Sometimes pain behind the eyes also occurs as the eyes are embedded in fat. All these symptoms make the patients handicapped and their lives can be compared to the lives of patients suffering from severe chronic RA. The RA patients surely have deformed joints, but can often be helped by mitigating medicine. However, that is of no use for the Dercum patients.

 

Mb Dercum patients have problems to stand, walk, especially up the stairs. It is necessary to stop and rest to be able to continue. All this pain leads to suffering in the social and sexual life. The patients feel victimized and are often exhorted to loose weight with little or no result. The efforts of loosing weight often cause the patients to go hungry. The pain in the hands is an obstacle when e.g. nursing babies. Some patients even have problems to go to the toilet and to climb into and out of the bathtub.

 

In contrast to fibromyalghia the sedimentation rate is sometimes high (20-50 mm) and by electrophoresis of the plasma, inflammations are seen explaining the high sedimentation rate. Dercum patients have been compared to other persons weighing up to 150-160 kilos and it is to be seen that the fat cells in Dercum patients are much bigger than those of other obese persons, and these cells also tend to grow even bigger.

The heat production (measured by a micro calorimeter) in the fat cells of a Dercum patient is higher than that of other obese persons with the same size of the fat cells.

 

A neuropeptide associated with pain, neuropeptide P, is slightly increased in the spinal cord liquid of the Dercum patient, as distinguished from that of the fibromyalghia patient and lower than that of other, healthy overweight persons. By microscopic investigations inflammatory cells can be seen round the vessels. The fatty acids building up the fat have a different percent distribution with Dercum patients than with healthy overweight persons with an increased value of monosaturated fatty acids.

 

The ability of working is very often quickly diminished. The modern rehabilitation, which is forced upon the patients - swimming, work-out and various working-tests - definitively take no consideration to the nature of the disease. It is clearly proved that even slight efforts make the symptoms worse. Instead, the Dercum patients often need psychological support because of all the distrust shown by the social authorities and the society during many years. People who don’t look ill or handicapped in any way are often told how healthy they look. But, not all diseases are visible, not even diabetes or a debuting cancer. We must learn to stop the mobbing that de facto exists even if it is not meant to be. Never say to a person “you cannot be ill – you look so healthy”!

 

 

TREATMENT SUGGESTIONS

 

The Dercum patient must be helped with many things in the daily life. The home often needs to be adjusted to the needs of the handicapped person. An elevator may be necessary, as well as special water taps, easy handled household equipments, wrist supports, soft cushions, orthopædic shoes and perhaps a car adjusted for the needs of the handicapped patient. It is recommendable that some patients can get a reduced dentist fee due to the dryness in the mouth something that may cause caries.

 

It is also important to keep a diet excluding some types of fat. Medicine to soothe the pain is necessary but has to be taken with care! A ”harmless” medicine is Alvedon (containing paracetamol) together with Doloxene (containing dextropropoxiphen) or Dexofen (containing dextropropoxiphen-napsylat) or Nobligan (containing tramadole-hydrochloride). But, these medicines often have little or no effect. A solution is to take a medicine containing codeine. It must be pointed out to the patient to take a pause in using these strong medicines, e.g. a couple of days per week or to change medicines every now and then. Other types of morphine-like medicines are sometimes required, though there are few patients who need these medicines. A good medicine is Somadril (containing carisoprodole) that eliminates the muscle tensions, and also here it is necessary to be precautious. When the patient suffers from insomnia, or sleeps very little, a medicine to induce sleep is preferable. There are e.g. Imovane (containing zopiclone) or Stilnoct (containing zolpidem) that help the patient to fall asleep. It is very rare that the patients get addicted to the prescribed medicines.

 

It is sometimes possible to loose a little weight and to receive a lighter breathing with Ephedrine chloride. This medicine has an effect on 50% of the patients. It increases the muscular work by stimulation of the nerve hormone nor-adrenaline and it also decreases the appetite. There have been no positive results after taking common slimming pills, e.g. Xenical (containing ortistate) that makes 30% of the fat-intake to pass directly through the intestines.

 

Many kinds of health foods are tested by the patients but without any remarkable results.

 

The facts above are given by doctor Birger Fagher, M.D. and specialist, to the Swedish Dercum Society.

 

 

Summary

 

In contrast to fibromyalghia patients, who usually are normal-weight and whose pain originates from muscle bellies and tendon insertations, patients with Mb Dercum have their pain located mainly to the abundant adipose tissue and skeleton. The pain is very resistant to treatment. Liposuction induces a postoperative decrease of sensibility in treated areas. Inflammation plays a part in the syndrome. The inflammatory cell process is supported by increase of several plasma proteins. Adiposis dolorosa has a spectrum of associated systemic phenomena, e.g. chronic fatigue, sporadic bouts of low-grade fever, Sjögren-like dryness and swollen hands and feet. Placebo has not effect on the pain. Many patients have been subjected to mobbing from the health care system and insurance authorities in Sweden (Fagher, B., M.D. on behalf of the Dercum Society in Sweden).  

 

 

A MB DERCUM PATIENT’S EXPERIENCE

 

·         Chronic and prolonged pain in the whole body. The pain moves and varies from day to day

·         Loss of concentration, absent minded

·         Abnormal tiredness that is an obstacle for daily activities

·         Depressed, not self-confident, nervous and irritable

·         Cannot live like before. Intestinal and urinal troubles. In need of a toilet close by

·         Sleepless, often waking up at night or  - the opposite - sleeping too much

·         Weak. The muscles cannot be strained

·         Feeling stress without reason, dizziness and nausea

·         Numbness, irritation in muscles and skin, headache

·         Pain and soreness, difficulties to wear clothes

·         Powerlessness and sorrow not to be believed

·         Sometimes an abrupt loss of sexual lust

·         Sjögren-like dryness in eyes, mouth and genitals varying from day to day

·         Cannot perform monotonous movements

·         Difficulties to sit and stand too long

·         Stiff and swollen mostly round the joints, worse in the mornings, aching jaws

·         Weather sensitive, especially cold and damp

·         Want to be social, but have no power. Often say no to social life

·         Mostly positive and happy. Often looking alert though the feeling is the opposite

·         The disease cannot be seen from the outside

 

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