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Adiposis Dolorosa

What is Adiposis Dolorosa?

Adiposis dolorosa is a rare disorder affecting fatty tissue under the skin. Affected individuals present with fatty growths, known as lipoma, found most commonly on the torso, upper parts of the limbs, and buttocks. These fatty growths form under the skin (subcutaneous) and feel like firm nodules and are typically painful. The associated pain can be severe and may come and go or persist. Adiposis dolorosa is associated with obesity and can cause other symptoms such as general weakness and tiredness, cognitive symptoms, and digestive problems. While there is a genetic link, most cases are not hereditary.

 

Adiposis dolorosa is a rare disorder affecting fatty tissue under the skin. Affected individuals present with fatty growths, known as lipoma, found most commonly on the torso, upper parts of the limbs, and buttocks. These fatty growths form under the skin (subcutaneous) and feel like firm nodules and are typically painful. The associated pain can be severe and may come and go or persist. Adiposis dolorosa is associated with obesity and can cause other symptoms such as general weakness and tiredness, cognitive symptoms, and digestive problems. While there is a genetic link, most cases are not hereditary.

Acknowledgement of Adiposis Dolorosa has not been added yet.

The prevalence of adiposis dolorosa is unknown. However, it affects women more frequently than men. Most commonly, it appears in overweight women between the ages of 35-50. Rarely, children have been affected.

Synonyms for Adiposis Dolorosa has not been added yet.

The cause of adiposis dolorosa is not known. There are many hypotheses proposed but none have been confirmed. These hypotheses include dysfunction in the nervous system, the endocrine system which is responsible for hormone production to regulate body functions, trauma, and abnormal metabolism of fat. 

 

Some suggest that adiposis dolorosa may be an autoimmune disorder where the immune system redundantly attacks healthy tissue. However, autoimmune disorders are expected to cause inflammation above normal levels. Inflammation is an immune response that can cause tissue damage if it persists. In individuals affected by adiposis dolorosa, inflammation appears to be similar to otherwise healthy, obese individuals. This provides evidence against the autoimmunity hypothesis. In some cases, the use of high dosages of a class of drugs called corticosteroids has been associated with adiposis dolorosa. Corticosteroids are anti-inflammatory drugs that reduce the symptoms associated with inflammation. 

 

There is some evidence suggesting that there is a genetic link. For example, there are families with more than one case of this disorder. In those cases, adiposis dolorosa appears to be autosomal dominant. An autosomal dominant disorder is a disorder that is caused by the presence of one defective gene. Each person inherits two copies of genes, one from each parent. While in some cases, if one copy of a gene is defective, the other copy can compensate. In other cases, the presence of one defective copy is enough to cause dysfunction. This is known as an autosomal dominant mode of inheritance. Despite this evidence, most cases of adiposis dolorosa are not inherited, and no specific genes have been identified.

 

The pain associated with the fatty nodules is also not understood. It is possible that these fatty growths press on nearby nerves and cause pain and weakness.

 

Adiposis dolorosa can be classified into four subtypes. The generalized diffuse type is characterized by general pain in fatty tissue around the body without any apparent lipomas. In the generalized nodular type, fatty tissue is generally painful and lipomas are present. Pain in the fatty tissue is more pronounced around the lipomas. In the localized nodular type, pain is localized to the lipomas. In the juxta-articular types, fatty tissue is painful near large joints.

The most common initial symptom is the growth of painful nodules that are sensitive to touch. The quality of this pain is described as burning, stabbing, and aching. These nodules vary in size from small to large.

 

Other symptoms include swelling, especially in the hands, and sudden, unexplained weight gain.  Generalized fatigue and weakness, bruising easily, headaches, irritability, sleep disturbances, depression, memory and concentration problems, flushing, shortness of breath, rapid heart rate, and gastrointestinal problems are among other symptoms. 

Them diagnosis of adiposis dolorosa is clinical and based on the presence of generalized obesity and chronic, painful fatty tissue just below the skin or multiple painful fatty nodules. The presence of other associated symptoms can help with diagnostic certainty. 

 

While adiposis dolorosa is primarily diagnosed based on symptoms, a biopsy of the fatty nodules can be helpful. During a biopsy, a small piece of the painful tissue is removed and examined to confirm that it is a lipoma. 

 

Although not common, ultrasound and magnetic resonance imaging (MRI) have been used as diagnostic tests. Ultrasound and MRI can visualize multiple fatty nodules under the skin if they are present. 

 

The goal of the management of adiposis dolorosa is pain reduction. Although none of the currently available treatments have successfully led to permanent pain relief, some treatments have temporarily reduced pain. Liposuction and surgical removal of painful lipomas has been helpful in some cases. However, lipomas may recur or pain reduction effects of these treatments decrease over time. Traditional pain medications can also provide some pain relief. Recently, transcutaneous frequency rhythmic electrical modulation system (FREMS) has been effective for pain reduction. In FREMS, electrodes are placed on the skin and send electrical signals to the nerves and interact with live tissue and can lead to pain reduction.

In individuals with joint pain, weight reduction can alleviate their pain, but weight reduction generally is not an effective treatment. Medications that increase the amount of water that is excreted through urination (diuretics) can reduce swelling of the hands and fingers. 

 

Adiposis dolorosa is a chronic disease and tends to progress and worsen over time. The associated pain is difficult to manage and usually does not respond well to treatment. 

 

Tips or Suggestions of Adiposis Dolorosa has not been added yet.

Genetic and Rare Disease Information Centre. Adiposis dolorosa. 2016. https://rarediseases.info.nih.gov/diseases/5750/adiposis-dolorosa

 

Cook JC, Gross GP. Adiposis Dolorosa (Dercum, Anders Disease) [Updated 2019 Apr 3]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507867/

 

Genetic Home Reference. Adiposis dolorosa. 2019. Available from https://ghr.nlm.nih.gov/condition/adiposis-dolorosa#synonyms

 

Tins B, Matthews C, Haddaway M, Cassar-Pullicino V, Lalam R, Singh J, et al. Adiposis dolorosa (Dercum’s disease): MRI and ultrasound appearances. Clinical Radiology. 68: 1047-1053. http://dx.doi.org/10.1016/j.crad.2013.05.004

 

Hansson E, Svensson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management.  Orphanet Journal of Rare Diseases. 2012; 7. Available from https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-7-23


 

Hao D, Olugbodi A, Udechukwu N, Donato A. Trauma-induced adiposis dolorosa (Dercum's disease). BMJ Case Reports. 2018. DOI: 10.1136/bcr-2017-223869

 

Bouchra A, Leguilchard F,  Benhamou C. Dercum’s Disease (Adiposis Dolorosa): A New Case-Report. Joint Bone Spine. 2004; 71(2): 147-9. doi:10.1016/S1297-319X(03)00139-8

 

Hansson E, Svensson H, Brorson H. Liposuction may Reduce Pain in Dercum's Disease (Adiposis Dolorosa). Pain Medicine. 2011;12(6): 942-52. doi: 10.1111/j.1526-4637.2011.01101.x

 

Campen R, Mankin H, Louis D, Hirano M, Maccollin M. Familial occurrence of adiposis dolorosa. Journal of the American Academy of Dermatology. 2001;44(1):132-136. DOI: 10.1067/mjd.2001.110872

Martinenghi S, Caretto A, Losio C, Scavini M, Bosi E. Successful Treatment of Dercum's Disease by Transcutaneous Electrical Stimulation: A Case Report. Medicine (Baltimore). 2015;94(24):e950. doi:10.1097/MD.0000000000000950

painful thighs Created by jhewels
Last updated 7 Feb 2010, 04:17 AM

Posted by jhewels
7 Feb 2010, 04:17 AM

Hi i am new to this site, and also suffer from Dercum's. i have recently had 20 lipomas removed and more have grown bak but the pain is worst in my inner thighs. i have other areas that cause pain but for me that is the worst location. i am having more removed this month. the dematologist tells me I am his lipoma girl. It is hard to convince others about this real disease. there is a doc who says he knows about it but when he talsk you know he doesn't that can be irritating. and trying to convince others how painful this disease can be is awful. we should not have to go through this but we must if we will help ourselves or others down the line. i take medication that helps with the pain but some days it's not enough. i have spoken to the doctor who specializes in dercum's and she is great and understanding but she lives in San Diego so i cannot go there. but if it comes down to it I will travel just to have a consult with here. she is willing to look at my pathology when i have it sent to her. I read that people with this disease have larger fat cells than in others without this disease. It is sad that people will just see the fat and not the disease that caused the fat. I am overweight and have typeIII Dercums. I have a set of doctors who are treating me for this disease and I am trying to get the word around as much as I can. well i hope to chat with other members. Till the goodnight!

this explains so much Created by LaurieH
Last updated 1 Jul 2009, 01:12 PM

Posted by LaurieH
1 Jul 2009, 01:12 PM

Hi- I have been dealing with unusual leg pain now for about 5 years. I am 48. I am also just finishing treatment for breast cancer. For years I just assumed that I probably had fibromyalgia, because my older sister suffers terribly from it. But the small lumps in my thighs have grown, and at times swell so much that walking is difficult. The pain is sometimes unbearable (usually at night), and I cannot sleep without the aid of a pain killer. I believe I have found my problem, and intend to take the information to my physician. If anyone knows of anything that can bring relief, that is not a narcotic, please let me know. Please email me at houdashelt_laurie@yahoo.com. Use A D in the subject so that I wont discard it. Thanks so much

not diagnosed but I think I have this. Created by maryla
Last updated 2 Jun 2009, 06:32 PM

Posted by BDcouture
2 Jun 2009, 06:32 PM

I have the reference for the medical survey that I mentioned earler. Go to www.lipomadoc.org , then click on "Adiposis Dolorosa" on the menu on the left of the page. Then scroll down to the references. In reference #2, which is the citation for Dr. Herbst's article, there is a link for a .pdf of the article itself. I encourage everyone with this condition to print out the article, read it, and take it to your docor(s) and beg them to read it. It is the best, most complete survey about this condition. All the other articles that I have found contain misconceptions and errors, such as the assertion that lipomas cannot occur on the feet: silly!

Posted by maryla
28 May 2009, 10:07 PM

Thanks for responding....yes, I am a middle aged, overweight drug seeking woman..LOL. I am allergic to NSAIDS, so that has put kind of a damper on what I can take for pain. I am currently taking vicodin, and have been for about 7 years. That has been for the fibromyalgia and osteoarthritis pain.....and for what I now believe is the dercums. I realized that most of the pain started with the lump (louie is it's name)..LOL I was diagnosed before that with chronic fatigue, as I have extreme fatigue and weakness that followed a severe flu. It was like I could never recover. I was in bed for about a year. Then I got a little better, but the pain started. The pain has been progressing. As I read through the symptoms of Dercum's, it was like reading my life story. The other interesting aspect of this, is my mother has developed lumps in her upper arms and now shoulders that are very painful....she is 90, but they started some years back. she got the same thing...those are fatty tumors, but are not usually painful....so no further action was taken. Now I read that this is hereditary, through matriarchal lines. My doc has always said that the fibromyalgia was kind of atypical. Have you had any imaging done to find the lipomas? Or, I guess I would be interested in your story.....you can email me if you would like...dancingdingovt@yahoo.com. Just put dercums in the subject so I will know not to erase it......if anyone else would like to tell my their history with this illness, please do...I also have lots of questions....

Posted by BDcouture
28 May 2009, 03:26 PM

Visit www.lipomadoc.org. (might be .com??) This site is run by Dr. Karen Herbst, a research endocronologist, who has conducted a very detailed, accurate medical survey on Dercum's Disease. This survey is the best, most accurate and most comprehensive article on this disorder that I have found. The results of this survey are on her site. Print it out and take it to your doctor!!!!! Chondromalacia of the knees and irritable bowel disease are very commonly associated with Dercum's (I have both!) Fatigue and muscle weakness are also common. Getting a diagnosis is extremely helpful. For one, it makes doctors pay attention to your symptoms instead of assuming that you are a fat, middle aged crazy woman seeking pain meds (I am not assuming that you are!). For another, it helps you learn how to take care of yourself: lots of rest, prescription NSAIDS for pain and flareups, and a very low carbohydrate diet are extremely useful.

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painful thighs

Created by jhewels | Last updated 7 Feb 2010, 04:17 AM

this explains so much

Created by LaurieH | Last updated 1 Jul 2009, 01:12 PM

not diagnosed but I think I have this.

Created by maryla | Last updated 2 Jun 2009, 06:32 PM


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