What Is Dercum's Disease

[matt]

Found this article from the long gone site dercumshope.org:

Article by Denise Loving, MS

Dercum’s disease is a progressive disorder that causes the growth of painful adipose (fat) tissue. The painful fat can be in distinct lumps and nodules, or it can be more diffuse and harder to tell from ordinary fat. A few other names used for Dercum’s disease are adiposis dolorosa, adiposa dolorosa, lipomatosis dolorosa, neurolipomatosis, or Dercum’s syndrome.

Diagnosis

It can be very difficult to get a diagnosis if one has Dercum’s. The disease is so rare that it is little known among the medical community. There are no laboratory tests that establish or rule out Dercum’s disease, although some tests used in research show different average values from people without any illness.

Many people with Dercum’s are misunderstood by almost everyone, and some have been accused of being a hypochondriac or even psychotic. When the symptoms “don’t make sense” some doctors assume the cause is psychiatric rather than organic. However, more doctors are becoming aware of Dercum’s disease.

Diagnosis requires a history of at least three months of chronic pain in fatty tissue, either constant or recurring. Some articles add (1) generalized obesity, which is not always present (2) asthenia (loss of strength) and excessive fatigue and (3) mental disturbances, including depression, confusion, emotional instability, epilepsy, and dementia. Many people with Dercum’s report depression, occasional confusion (often called “brainfog”) and memory problems. Fortunately epilepsy and dementia seem to be quite rare. Much of the literature reports that Dercum’s occurs most often in obese postmenopausal women. However, it does occur in teenagers and young adults, and about one in twenty patients are male. While weight gain is common, there are many people with Dercum's who have never been overweight. Losing weight will not decrease the pain or the size of the lumps.

Other diseases which may be confused with Dercum’s include familial multiple lipomatosis, which is an inherited condition of usually non-painful lipomas, lipodystrophy, and Madelung’s disease (also known as multiple symmetric lipomatosis, among other names). In those conditions some lipomas (abnormal growth of fatty tissue) may be painful because they press on nerves, but most are not. In Dercum’s disease most or all lipomas and other fat deposits are tender or painful, with the pain often increasing over the space of years. In particular, bumping a Dercum’s lump or lipoma usually hurts, and pinching one usually hurts a lot, much more than one would expect. Sometimes lipomas in Dercum’s are angiolipomas - lipomas which contain many blood vessels.

Painful fat can occur anywhere on the body, including the head and neck. Some people have tender or painful fat deposits literally from the top of their head to the soles of their feet. They are almost always in the subcutaneous fat layer, although it seems that at times they can invade other tissues. It appears that not a lot is known about how often that happens.

Dercum’s has been classified into three types, based on the location and character of the painful fatty tissue. One can have a mixture of two types, such as type 2 and type 3. These are:

Type I, or the juxta-articular type, with painful folds of fat on the inside of the knees and/or on the hips, in rare cases only evident in upper-arm fat.

Type II, or the diffuse, generalized type, where widespread pain from fatty tissue is found, apart from that of type I, also often in the dorsal upper-arm fat, in the axillary (armpit)and gluteal (buttocks) fat, in the stomach wall, in dorsal (side) fat folds, and on the soles of the feet. This is the most common type. The painful fat can be spread out to resemble ordinary obesity at first glance, and can also occur as distinct lumps arising from the subcutaneous fat.

Type III, or the lipomatosis, nodular type, with intense pain in and around multiple lipomas, sometimes in the absence of general obesity; lipomas are approximately 0.5-4 cm, soft, and attached to the surrounding tissue.

Many patients with type III Dercum's report small rubbery nodules, which feel rather like corn or marbles under the skin. These often grow together into chains or clumps of lumps. These seem to occur most often in the arms, abdomen, and rib area.

The pain from Dercum’s disease is often described as aching, burning or stabbing. Pain is common when a lipoma is bumped, pressed, or pinched, but can occur at any time. Sometimes even the lightest pressure can be painful, and only loose clothing can be tolerated. Pain can also be felt in the skeleton, as well as the fatty tissues.

The intensity of the pain varies from mild discomfort to excruciating pain. Pain often increases during menstruation, and decreases with warmth and high barometric pressure. Infections often make the pain worse. Stress, both physical and psychological, appears to make the symptoms of Dercum’s worse. Unfortunately, just having Dercum’s disease adds a lot of stress to life.

Other Common Problems

Other problems which commonly occur with Dercum’s disease include the following.

Swellings that appear and often recede for no apparent reason. The swellings can resemble retained water, but they are non-pitting. That is, firm pressure by a thumb doesn't cause a pit that slowly becomes level. These swellings can leave the skin loose and inelastic (saggy skin, or pendulous skin folds), or hardened. In some cases it appears that as or after the swellings subside, adipose tissue (fat) increases under the skin, and at times nodular lipomas can be felt later.

Sleep disturbances and difficulty getting to sleep are common. Many patients find they need ten or more hours of sleep each day to function. Missing sleep can make the pain worse, and more pain makes sleep even more difficult.

Numbness, tingling and burning in the limbs, particularly the hands and feet. This seems to occur when a lipoma presses on a nerve. It can be constant or only noticed when the limb is in certain positions. It can disappear without treatment at times, presumably when temporary swelling is putting additional pressure on the area. This seems to be relatively rare, however. In most cases it won’t subside without removal of the offending lipoma(s).

Thyroid problems may occur. This is most commonly an underactive thyroid, but overactive thyroid has also been noted.

Other problems are also reported, such as fibromyalgia, carpal tunnel syndrome, tendonitis, irritable bowel, and pain in the tailbone and vulva.

There have been reports of death due to cardiac (heart) and pulmonary (lung) complications, but little appears to be known about this.

Dercum’s and Cancer

It is very rare that cancers of the fatty tissue (liposarcomas) start in an existing lipoma. There doesn’t appear to be any higher chance of developing cancer because one has Dercum’s disease.

What is the Cause?

No one knows what causes Dercum’s disease, but it appears to be hereditary in some families. From the way it seems to be transmitted in those families, there is a single gene that mutates. In most familiar hereditary diseases, such as cystic fibrosis, to have the disease a person needs two copies of the defective gene. Assuming that Dercum’s is caused by a mutated gene, it seems that it only takes one defective copy of the as-yet-unknown gene to cause the disease. Most people with Dercum’s disease don’t have any known relatives that also have it; in those people it is possible that a mutation occurred in the egg or sperm cell before fertilization. There are also various degrees to which Dercum’s affects people, and there may be relatives which have Dercum’s in a very mild form that is never detected.

You will find articles about treatment options, support groups, and other useful information elsewhere on this website.

Bibliography

Brorson H, Fagher B.
Dercum’s disease. Fatty tissue rheumatism caused by immune defense reaction?
Lakartidningen 1996 Apr 10;93(15):1430, 1433-6
(Translation by Ruth Weaverat http://dercums_data.tripod.com/brorson.html)

Campen R, Mankin H, Louis DN, Hirano M, Maccollin M.
Familial occurrence of adiposis dolorosa.
J Am Acad Dermatol 2001 Jan;44(1):132-6

Fagher B, Monti M, Nilsson-Ehle P, Akesson B.
Fat-cell heat production, adipose tissue fatty acids, lipoprotein lipase activity and plasma lipoproteins in adiposis dolorosa.
Clin Sci (Lond) 1991 Dec;81(6):793-8
available online at http://dercums_data.tripod.com/fat_heat.html

Macaron N, Arbiser L
Adiposis Dolorosa
eMedicine.com (online) avaliable at http://www.emedicine.com/derm/topic839.htm

Motzer, I
Dercum's for Dummies
online, available at www.dercumshope.org/dercum_for_dum.php

National Organization for Rare Disorders, entry on Dercum Disease
online, available at http://www.rarediseases.org/search

[Klaus]

Hi Denise,
you are angry that you have a Dercum illness. How do you know that you have one? Have you any expierences what is Dercum disease and what is lipoma?

It is very seldom that somebody can give you answers, based on what you have provided.
Is it possible to give more detail, and let me know in which country do you live? Maybe I can provide you with more details.

Regards

Klaus

[Dmac5018]

I am new to this subject. I have however been doing lots of research. This is a frustrating process. Many Docs dismiss this as unimportant. From someone who has had multiple lipomas removed, most of which were quite painful. They appeared very quickly shortly (within 2 months) after completing the current treatment for hepc. What I noticed first was extreme, consistent itching close to prior injection sites. The second thing I became aware of were very fast(within 2 weeks) growing lumps that continued to itch. The first being removed right away. Two weeks later more appeared. These were in a long line. All have been found in abdominal area. Once reached a certain size the itching stopped. Post(many yrs) surgically menopausal, overweight, fibro myalgia,as well as many other seemingly connected conditions. Interested in whatever others have found in relation to this condition. Thank You.

[Guest]

Hi Denise,
you are angry that you have a Dercum illness. How do you know that you have one? Have you any expierences what is Dercum disease and what is lipoma?

It is very seldom that somebody can give you answers, based on what you have provided.
Is it possible to give more detail, and let me know in which country do you live? Maybe I can provide you with more details.

Regards

Klaus

Klaus:

Hi. Denise definitely has a well documented case of classic Dercum's syndrome.

She has posted a lot of information about her own case, but the post quoted above was not intended to inform about her own symptoms. I don't think she is seeking assistance at this time.

Regards.....

[maria]

I too discovered the painful itchy lumps during my Hep C treatment. Since completing my treatment they have spread everywhere. Of course the Doctor wasn't even interested in the pile of the research I brought in on Dercums

[Cristina]

Hello! Was interested to find out how everything progressed and how you have treated your Dercum's...
Its been four months since i started feeling excrutiating abdominal pain, starting 2 days before period and ending about 2 days after - in a certain area where i can feel a lump. For the rest of the time i can just feel that somehing is there and sometimes a wierd movement...
After two ultrasounds and an MRI the doc still didnt reach a conclusion, It is very frustrating!!!
The comments of the 'specialists' reading the scans are 'existing lump but all normal' (!!!!)

I cant live with this thing... the pain is just out of control

[matt]

Hi Cristina! Have you seen/read everything at lipomadoc.org? Here's a pdf that suggests the following for treatments:

Evidence-Based Treatment (alphabetic order)

• Bariatric surgery: Improves co-morbidities but weight loss may be minimal while the lipomas remain.
• Cycling hypobaric pressure: The Cyclic Variations in Adaptive Conditioning™ (CVAC™) process
• improved pain and mental functioning in ten people with DD.
• Infliximab and methotrexate: Improved pain and induced weight loss in a single woman with DD.
• Interferon alpha-2b: Induced pain relief in two patients with hepatitis C infection.
• Lipoma resection: Reduces pain but the lipomas may recur; inhibit recurrence and seroma formation with compressionafter resection for 6 weeks.
• Lidocaine: Topical and intravenous preparations have been used with variable success.
• Liposuction: Reduces pain and improves quality of life.
• Manual lymphatic drainage combined with compression garments and pregabalin (pain medication) reduced pain and weight.
• Mexilitene: After intravenous lidocaine, mexilitene was able to maintain pain relief.
• Metformin: See Case study in the original PDF. May either not work for pain relief, or may lose its effectiveness over time.

I'd also suggest taking some antitumor lipoma supplements as they may lessen the inflammation and reduce the pain.