Vatanchi M. Cutaneous Lipomas. Dercums disease affects females more often than males with medical publications citing the disease as 5-30 times more common in women. Adiposis dolorosa, also known as Dercums disease, is characterized by painful lipomas that usually appear on the trunk and upper and lower proximal extremities. Dercums Disease, on the other hand, is very rarely known by most medical professionals. Medscape Updated: Dec 15, 2017. Weve done our best to try to facilitate the work of other researchers as best we can over the years, but thats really the best we can do. As always, we encourage medical professionals to consult both our Articles section and PubMed for additional information. WebA rare disorder is a disease or condition that affects fewer than 200,000 Americans. They may also do tests to rule out other medical issues that share some of the same symptoms. This rule of thumb is doubly important in the Dercums community. The Society is an informal gathering of Dercums patients, their families, and doctors, dedicated to making scientific information about the condition more readily available to Think of it this way. Surgical excision of lipomas may temporarily relieve symptoms although the generation of inflammation (part of the healing process) during and after the surgery may induce more lipomas to develop in that area. No specific treatment exists for Dercums disease. As such, legitimate medical research is performed under the auspices of large organizations and/or respected medical schools. Please, trust your gut. However, almost all people with Dercums disease have painful lipomas that grow slowly. Unlike Dercums disease, the lipomas do not cause pain. Angiolipomas may be inherited in an autosomal dominant manner. Our founder, Ms. Lawver, is happy to pay the small yearly fees for our domain registration and web hosting. Then the third type is a combination of the two, with both lipomas and painful diffuse fat. Reduction of the inflammation associated with infection with the hepatitis C virus may also have provided benefit. In addition to Ms. Lawver, several very kind physicians and researchers have been instrumental in verifying the data provided on this website. He is not, however, an ongoing contributor. To make matters even more complicated, most Dercums patients often also have a wide array of additional diagnoses. Anecdotal information is used sparingly and will always be clearly identified. In most cases, there doesnt seem to be an underlying cause. Lets deconstruct this question a little bit further. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. Angiolipomas are known to be painful and hence people with angiolipomas are said to have Dercums disease, angiolipomatosis type. Lippincott Williams & Wilkins. However we have discovered in well-documented clinical research that Dercums mutated fat cells are incredibly unique. We wouldnt even want to try! No, we do not. All rights reserved. Average disease duration was 2 years (minimum of a few months and maximum of 9 years). Seemingly seeking celebrity over science. You can learn more about how we ensure our content is accurate and current by reading our. Autosomal dominant inheritance in adiposis dolorosa (Dercums disease). We hope you'll take a look! Well review the research on whether CBD oil is effective. This is truly senseless and must be changed. Therefore wed like to caution Dercums patients everywhere not to jump to conclusions or get frustrated by an apparent lack of quick action in the medical community based on either individual cases or small amounts of anecdotal evidence. More often than not, Dercums patients go through at least a four year period between when they first suspect they may be ill and when theyre finally diagnosed. The risks are too steep. Our founder readily acknowledges she is not a doctor, but has done her best to bring together the best and brightest doctors to help make medical information more accessible and easy to understand for the sake of Dercums patients, their families, and the public at large. In order to get answers for you as quickly as possible, we've compiled a page filled with the most vital information to satisfy your most urgent questions. 2013; 25:00199. BMJ Case Rep. 2018; :bcr-2017-223869. Swelling occurs for no apparent reason, though on imaging, the lymphatic system can be slow to pump in the painful limb compared to the less painful limb, or frank lymphedema may be present. But if you happen to find any published medical journal articles you feel we may have overlooked, please feel free to send it to us. Please be wary, however. DOI: Debra Sullivan, Ph.D., MSN, R.N., CNE, COI, rarediseases.org/rare-diseases/dercums-disease/, fat-disorders-2017.squarespace.com/dercums/, mayoclinic.org/diseases-conditions/lipoma/symptoms-causes/syc-20374470, 12 Things Only Someone with Chronic Pain Would Understand. Yes. J Plast Surg Hand Surg. Dercum disease, also known as adiposis dolorosa, is a rare disorder of Heather Lawver founded the Dercum Society in 2005 shortly after she was diagnosed with the disease by a team of doctors from (what was then known as) the National Naval Medical Center at Bethesda and the National Institutes of Health. Dercums disease mainly occurs in adults and more women are affected than men. The content from our original website (the Dercums Disease and Diagnosis sections as seen on the menu above) was all essentially translated (for lack of a better word) from the work of Dr. Hkan Brorson & Dr. Birger Fagher of the University of Malm, as well as Dr. Francis Dercums original published works on the disease. McKusick VA. ed. Lange U, Oelzner P, Uhlemann C. Dercums disease (Lipomatosis dolorosa): successful therapy with pregabalin and manual lymphatic drainage and a current overview. Phone: 203-744-0100 Additional symptoms may include muscle spasms and stiffness. However, we are learning more about the three different distinct types of the disease which seem to appear at different rates in each gender. Powered by NORD, the IAMRARE Registry Platform is driving transformative change in the study of rare disease. However several published case studies and a great deal of anecdotal reports seem to indicate that even low doses of steroids can lead to an increase in lipoma formation and/or a general worsening of symptoms. Our founder developed perfectly symmetrical painful lipomas on the soles of each foot, right where there were plastic nubs on her favorite pair flip-flops, digging into her foot ever so slightly with each step. The size and number of lipomas varies from person to person. The neck and shoulders are usually not affected. Buck DW, et al. Related Pages. Additional symptoms may occur in individuals with Dercums disease including fatigue, generalized weakness, a tendency to bruise easily, headaches, irritability, and stiffness after resting, especially in the morning, gastrointestinal symptoms, palpitations and shortness of breath. ID Cases 2020;19:e00682. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. Its even more difficult when the few doctors that do know about Dercums dont know that it can present itself in three very distinct patterns. Again, if you cant find answers, youre more than welcome to contact us! Additional reports in the medical literature have linked Dercums disease to a variety of conditions including arthritis, high blood pressure (hypertension), congestive heart failure, sleep disorders, dry eyes, and myxedema, a condition due to an underactive thyroid that is characterized by dry skin, swelling around the lips and nose, mental deterioration and infection. Neither is Fibromyalgia, Lipedema, Lymphedema, or anything else writ large an early form of Dercums Disease. Adiposis Dolorosa. Its vitally important that you not attempt to diagnose yourself. Trauma-induced adiposis dolorosa (Dercums disease). Certain case studies and anecdotal reports seem to indicate that some patients believe a small percentage of their tumors seem to coincide with some form of trauma, either blunt force or repeated. But again, we know the root causes of both Lipedema and Lymphedema. Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORDs mission. With all of the various ways in which Dercums can affect the body, its not surprising that it can have a unique domino effect in each patient. Please, do your research and always insist on science-based medical information. I read in an article that average life expectancy for DD is 52. Symptoms of the following disorders can be similar to those of Dercums disease. Others believe its a metabolic problem related to not being able to properly break down fat. Your message will go directly to our founder. Or it could be the result of a hormone or nervous system problem. Weve also witnessed a fair amount of bullying of those who disagree, as well as an eagerness to exclude any information/data that runs contrary to certain closely held opinions or beliefs of those at the top. White spots on your nipples are usually harmless. Youre told to exercise, but nothing helps. Research Last updated: Ms. Lawver is intensely grateful for the assistance and generosity of the many doctors over the years who have contributed to this website and the cause of raising awareness and understanding of Dercums Disease. Tins BJ, Matthews C, Haddaway M, Cassar-Pullicino VN, Lalam R, Singh J, Tyrrell PN. National life tables for many countries are published by the WebDercum's disease is considered a chronic condition and can have long-term impacts on First off, anecdotal evidence meaning just you alone, or just your friends, or just a small handful of patients is not enough information to equal concrete fact. If you have Dercums disease, consider working with a pain management specialist as well as a mental health professional for added support. Clinical presentation. Also I would like to know if any of you have flare ups like mine. Consider working with a pain management specialist to find the safest combination thats most effective for you. (Lond) 2017; 41:240-245. All of this we find deeply troubling and as such, we can not in good conscience support it. (It breaks our hearts to report, that question is a direct quote from a recent email, and is a paraphrased quote from far too many others. However, in one reported case in the medical literature, the use of high-doses of corticosteroids was linked as a possible cause of the disease. Dercum's Disease Last Updated: 5 December 2022 Focused Ultrasound Therapy Focused ultrasound is a rapidly evolving, noninvasive, therapeutic technology with the potential to improve the quality of life and decrease the cost of care for patients with Dercums disease which is also known as Adiposis Dolorosa which translates to A Podcast For The Rare Disease Community, Rare Disease Cures Accelerator (RDCA-DAP), Policy Statements & Letters to Policymakers, Stay Informed With NORDs Email Newsletter, Launching Registries & Natural History Studies, https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/, http://www.emedicine.com/derm/topic839.htm, http://www.emedicine.com/derm/topic242.htm, https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/, https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/, https://rarediseases.org/patient-assistance-programs/caregiver-respite/, Learn more about Patient Assistance Programs >, https://rarediseases.org/non-member-patient/american-chronic-pain-association/, NIH/National Institute of Diabetes, Digestive & Kidney Diseases, https://rarediseases.org/non-member-patient/nih-national-institute-of-diabetes-digestive-kidney-diseases/, Learn more about Patient Organization & Membership >. Data are for the U.S. Life expectancy at birth. See how to do it right and avoid side effects. 1997;27:1141. I have episodes of severe pain that is mostly in one area like it is probably new growth, hot flashes with excessive sweating, cold chills, lowgrade Anecdotal evidence is interesting, it can be used to help guide further medical research, but it should not be taken as a grand sign from above that all the answers have been found. Please note that NORD provides this information for the benefit of the rare disease community. Weve also personally seen and experienced those marketing opportunities clouding the advice given to others, including toward patients that have not been physically examined by the medical professional(s) proffering that advice in an online setting. Herbst KLH. The same goes for Lipedema, Lymphedema, et cetera. However, no one should fault you for being your own advocate and trying desperately to find answers to the chronic pain and misery youve been dealing with. Webpage 2 Journal of the american osteopathic college of Dermatology AOCD 2902 N. Baltimore St. Kirksville, MO 63501 800-449-2623 FAX: 660-627-2623 www.aocd.org COPYRIGHT AND PERMISSION: Written permission must be obtained from the Journal of They have been denied a healthy normal life because of the constant pain brought on by this disease and have suffered the frustrations of the futility of their efforts to become well and whole. That said, in this case, the anecdotal evidence can caution us to be careful about avoiding injury, which in general isnt bad advice anyway. Theres no standard criteria for diagnosing Dercums disease. In most cases, Dercums disease appears to occur spontaneously for no apparent reason (sporadic), though causes of inflammation should be sought. But unfortunately it is often either misdiagnosed or undiagnosed. We do know that there are three distinct presentations of the same underlying disease. Charging exorbitant fees for consultations. As always, if you can't find the information you need, we're happy to hear from you. In the past few years, The National Institutes of Health have started a few research protocols to look into Dercums Disease. In addition, small specific areas known as tender points are typically painful when pressure is applied to them. What Causes White Spots to Form on Your Nipple? Absolutely. The average life expectancy figures for the most common types of dementia are as follows: Alzheimers disease around eight to 10 years. If users are not very carefully monitored and pre-treated with enriched oxygen, the risks are near certain of causing. Your diet doesnt seem to affect your size. In order for your doctor to know they can trust the information literally with your life, they need to know the information has been obtained by following rigorous scientific standards, and that the data has been carefully reviewed by a panel of respected experts. If youd like to take a look, please, The best person to help you with the chronic, debilitating pain of Dercums Disease is a qualified pain management specialist a doctor trained in the intricate details of treating pain. Some individuals may develop hundreds of small lipomas that do not cause symptoms (asymptomatic), and are often found in similar locations on one side of the body compared to the other side (symmetric). Frequently Asked Questions, The Dercum Society was founded by Heather Lawver, herself a sufferer of the disease. Prenatal. This final type seems to be the most common as documented by current medical research. Angiolipomatosis is a rare disorder characterized by multiple lipomas with increased numbers of blood vessels that contain fibrin clots, and larger numbers of mast cells. Again, it is not a toy and is nothing to be taken lightly. The symptoms from some diseases may begin at any age. If you have Dercums, the growths of fatty tissue (lipomas) may appear all over your body. Chronic Kidney Disease Life Expectancy by Stage . Int J Obes. If you have been diagnosed, be sure to print out a few medical journal. As you will see in our Introduction and throughout those sections, weve included as many footnotes as possible to the relevant sections. The root gene that leads to the mutation of fat cells in Dercums Disease has yet to be discovered. We also welcome any recommendations, suggestions, ideas, or critiques you have that might help us improve the website or in any way contribute to our mission of raising awareness and making medical information more widely accessible to the general public. WebIt most often shows up between the ages of 45 and 60. Campen RB. WebThis is a disease that could result, at best, in a lifetime of chronic pain, or at worst in The upper limb was affected first in 39 cases, the lower limb in 14 cases, both upper and lower limbs in 11 cases and bulbar onset in 21 cases. As such, such people arent even allowed to go scuba diving for that very reason. This is the very first time in our over 10 year history that we have ever addressed this. Unfortunately we dont have the capacity to keep track of the various doctors throughout the country and the world who are at least familiar with the disease. Alternative approaches including acupuncture, hypnosis, biofeedback, and cognitive behavior therapy have also been tried for individuals with Dercums disease. Attempting to fast forward through the vital clinical diagnostic process for Dercums Disease of carefully ruling out other possible causes. The exact cause of Dercums disease is unknown. Throughout this site we will examine what peer-reviewed medical data is currently available about Dercums Disease, provide links to peer-reviewed published medical journal articles, offer a glimpse into the Dercums diagnostic process, address several common myths, and do our best to offer advice for simple lifestyle changes that seem to produce some anecdotal improvements in the quality of life of current Dercums patients. Unfortunately, like most answers regarding Dercums Disease, there just hasnt been enough medical research to know for certain. Our hope, though, is that you will read through the articles as much as you are able. Dercums cannot be adequately diagnosed if medical professionals arent even aware of its existence. If you suspect you may have Dercums Disease or any other possible lipid disease, please contact your doctor immediately and request that they search PubMed (an international database of peer-reviewed medical journal articles) for additional information. Another type presents without any lipomas, but rather a unique pattern of painful diffuse fatty tissue collecting on the thighs, upper arms, and a distinct shape on the belly and buttocks. Email: [emailprotected], Some current clinical trials also are posted on the following page on the NORD website: Throughout the process of translating those medical articles, Ms. Lawver was guided by an informal group of doctors from (what was then known as) the National Naval Medical Center in Bethesda, Maryland. Given all of that, it appears that liposuction is a whole lot of risk for very little and very fleeting relief. Skinmed. Lipedema: A relatively common disease with extremely common misconceptions. Rheumatol Int. For instance, when entering a hyperbaric chamber in a hospital, it is vital that you wear only natural fibers and have absolutely nothing on you that could possibly cause a spark, as there is a hugely increased risk of explosion and fire due to the enriched oxygen. McGevna LF. Because your life may depend on it. Naturally, as you know, Dercums Disease presents with symptoms that are incredibly similar to many other diseases. Dercums disease. Cannabidiol (CBD) oil, made from cannabis, is sometimes used for chronic pain. But much like all placebo effects, its short lived. We are dedicated to only providing information that can be traced back to peer-reviewed medical journal articles. The two are not the same, despite presenting similarly in some ways. We vehemently reject it and refuse to associate with anyone who would dare devalue human life so callously. 2005 - 2023 WebMD LLC, an Internet Brands company. All work was done on a strictly volunteer basis and Heather paid and continues to pay for the websites hosting entirely on her own. Fibromyalgia is a chronic disorder characterized by pain throughout the muscles of the body and abnormally persistent episodes of fatigue. NORD strives to open new assistance programs as funding allows. Here's what may be causing them and what you can do to ease your symptoms. Of course you are free to support anyone that you choose. Fibromyalgia is not uncommon in people with Dercums disease. Our goal was to translate into laymans terms the information found within the original published work of Dr. Francis Xavier Dercum, as well as Dr. Hkan Brorson & Dr. Birger Faghers landmark multi-year study of the disease, Portrait of a Medical Enigma, published in The Journal of the Swedish Medical Association on April 10, 1996. With input from doctors, researchers, and the US Food & Drug Administration, NORD has created IAMRARE to facilitate patient-powered natural history studies to shape rare disease research and treatments. There really isnt too much we need. Upon further digging, she could find only two sources of any information; 1) a single rant published on an Australian blog, lamenting how painful the disease is, and 2) a medical journal article published entirely in Swedish, which turned out to be the work of Dr. Brorson and Dr. Fagher. Im curious to know how old some of you are and how long you have had DD. Meaning youre likely to have a mix of normal, healthy fat, and the uniquely mutated Dercums fat cell. Meaning, oftentimes theres no concrete abnormal test result doctors can point to for the reason behind either Dercums pain or Fibromyalgia pain. First described in 1892 by the American neurologist Francis Xavier Dercum at Jefferson Medical College in Philadelphia, Pennsylvania, Dercum disease (adiposis dolorosa) is an unusual progressive syndrome of unknown etiology characterized by multiple painful lipomas that arise in adult life, most often affecting obese Yet if youre temporarily feeling euphoric, you cant even safely judge or realize that youre putting yourself in danger. All rights reserved. Allergies are not an early form of a cold. Cherish it. Suite 310 Some individuals with Dercums may experience swelling of various areas of the body, especially the hands, but often one arm or leg may be swollen and therefore more painful. (2016). Available at: https://www.omim.org/entry/103200 Accessed Jan 6, 2020. (2012). Some cases of Dercums disease have run in families and several reports in the medical literature cite the possibility that the disorder may be inherited as an autosomal dominant trait in these cases (FML type or angiolipomatosis type). The lipomas can be the size of a pea or as large as a grape, plum or fist. Acta Pharmacol Sin. According to a review in the Orphanet Journal of Rare Diseases, Dercums disease is anywhere from 5 to 30 times more common in women. As such, most doctors no longer recommend liposuction for Dercums patients as a means of treating pain. South Dartmouth (MA): MDText.com, Inc.; 2000-2019 Dec 14. 55 Kenosia Avenue The report shows the disparity in life expectancy between men and Learn About Scientific Research, We get a lot of questions from patients and their families. Patients must rely on the personal and individualized medical advice of their qualified health care professionals before seeking any information related to their particular diagnosis, cure or treatment of a condition or disorder. 1998;243:197-201. Interferon alfa-2b induced long-term relief in two patients with adiposis dolorosa and chronic hepatitis C. J Hepatol. Onset of this disorder is usually during adolescence. And yet despite all of this, after a wide array of tests, your doctors cant seem to explain why you have these strange myriad symptoms. The ultimate goal of IAMRARE is to unite patients and research communities in the improvement of care and drug development. There is a solid reason medical research is conducted using very large numbers of patients compared against similarly large numbers of control groups. Dermarolling, aka microneedling, is the art of erasing unwanted acne scars, wrinkles, and stretch marks. Because certain systems of the body rely on fat cells to function properly, its understandable that a collection of mutated fat cells could alter however slightly those bodily systems. See additional information. If you are a medical professional, a basic understanding of this disease will help your patients who are searching for help and relief from chronic pain. Theres even some question if perhaps the pain relief might have been a surgical variant of the placebo effect after the physical and emotional investment in surgery, a psychosomatic return in slightly lessened pain is a possibility. (2015). Cumulatively, there are more than 7,000 rare diseases affecting more than 30 million Americans. Again, this is just our opinion. Familial occurrence of adiposis dolorosa. As such, we have worked very hard to ensure that every ounce of data on this website can either be traced directly back to peer-reviewed medical journal data, or is clearly labeled when and if we include anecdotal information. Several disorders are characterized by the development of benign (noncancerous) growths consisting of fatty tissue (lipomas) including Proteus syndrome, PTEN hamartoma syndrome, and Gardner syndrome. Unfortunately our founder regularly receives unkind, forceful emails from their supporters, demanding she take the website down and even going so far as to include name-calling and threats of gossip, black-balling, and diminishing her personal reputation. Both diseases present with chronic, widespread pain with a seemingly unknown, untestable cause. National Organization for Rare Disorders: Dercums Disease., Fat Disorders Research Society: Dercums Disease., National Human Genome Research Institute: Learning about Dercum Disease., Orphanet Journal of Rare Diseases, Review of Dercums Disease and proposal of diagnostic criteria, diagnostic methods, classification and management., National Center for Advancing Translational Sciences/Genetic and Rare Diseases Information Center: Adiposis Dolora.. The most current research has been conducted by The University of Malm, The University of Lund, and the National Institutes of Health. More research is necessary to determine the long-term safety and effectiveness of interferon alfa-2b as a potential therapy for this disorder. Yes, the slower path tests our patience. Thank you very much for your generosity! Campen R, Mankin H, Louis D, et al. Familial multiple lipomatosis is a rare genetic disorder characterized by the formation of multiple growths of fatty tissue (lipomas) that most often affect the trunk, arms and upper leg. We encourage everyone from the average person to all medical professionals to browse through this website and educate themselves about this disease. Subcutaneous Adipose Tissue Diseases: Dercum Disease, Lipedema, Familial Multiple Lipomatosis and Madelung Disease. Tollfree: (800) 411-1222 You gain weight disproportionately, but you cant explain why. Those guidelines are vital for the health and safety of others and cannot be taken lightly. Thats precisely why the clinical diagnostic process is so vital, in order to rule other potential dangerous diseases out. These classifications include: Theres no cure for Dercums disease. As always, feel free to contact us by clicking here. WebTechnically speaking, Dercums Disease is classified as a genetic lipid disease. One case reported in the medical literature was linked to the use of high-doses of corticosteroids and some cases have been linked to infection including Lyme disease or Valley fever (coccidioidomycosis). Injections of corticosteroids have also been used to treat individuals with Dercums disease. Some experts think Dercums may come from a mutated fat gene thats passed down in families. WebThe Dercum Society was founded by Heather Lawver, herself a sufferer of the disease. Life expectancy is less if the person is diagnosed in their 80s or 90s. Beltran K, Wadeea R, Herbst KL. The exact reason for pain associated with Dercums disease is unknown but may occur because the lipomas press on nearby nerves or because the fascia is inflamed. We do not mention their names out of kindness, but sadly, rarely has that kindness been returned. If youre diagnosed with Dercums disease, your doctor may classify it based on the size and location of your lipomas. Copyright 2023 NORD National Organization for Rare Disorders, Inc. All rights reserved.