Most of the different treatment strategies that exist are based on case reports. Pain associated with Dercum's disease can often be severe. In 2008 I realized I needed to find out what exactly was going on with my body myself. Science is at the heart of good medicine. Once again I had to find a new doctor. In order to properly diagnose the patient, the doctor must first exclude all other possible differential diagnoses. Many will do a yearly list of highly recommended doctors. Researchers have proposed a 'minimal definition' based on symptoms most often part of Dercum's disease: 1) Generalized overweight or obesity. 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 postmenopausal women. A higher concentration of monounsaturated fatty acids was noted in the lipomas. Cowden disease: trichilemmomas and multiple hamartomas and is associated with breast and thyroid carcinoma, Multiple endocrine neoplasia type 1: parathyroid, pancreatic and pituitary tumors. Dercum's is a rare disease that causes painful, fatty growths to appear under the skin, sometimes all over the body. One patient's symptoms were improved with methotrexate and infliximab. Disease at a Glance Summary Adiposis dolorosa is a condition characterized by the growth of multiple, painful, lipomas (benign, fatty tumors). Fascia is the thin layer of connective tissue surrounding organs within the body. In most cases, there doesnt seem to be an underlying cause. Clinical studies are medical research involving people as participants. Treatment usually includes: Learn more about eating a balanced diet and maintaining a healthy body weight. This page is not available in other languages. Swelling of various body areas have been reported. Cyst vs. Lipoma: What Are the Differences? When you meet a new doctor with a great deal of kindness, patience, and humility on your part politely ask them if they might be interested in reading a medical journal article about a rare disease youd heard about and causes you some concern. Orphanet and the National Organization of Rare Disorders also listed the disease.[5]. (n.d.). If you have a problem with fat in any of the diseases listed above, you have a connective tissue disease. (PDF) Dercum's Disease: Estimating the prevalence of a rare painful In contrast, Atkinson et al. If not an expert, how do I find a good doctor? We are dedicated to only providing information that can be traced back to peer-reviewed medical journal articles. The number and severity of symptoms experienced may differ among people with this disease. We do not solicit nor accept donations. Ultrasound and magnetic resonance imaging can play a role in diagnosis. Dercum's Disease: Symptoms, Causes, Diagnosis, Treatment - WebMD There are no unique features that distinguish a lipoma of Dercums disease from other lipomas. A naturally inquisitive doctor is more likely to be equally inquisitive about tracking down the underlying cause of your illness. [5] Although surgical resection or liposuction improves pain, regrowths occur in 50% cases. Enter your email address to receive updates about the latest advances in genomics research. Treatments for gallbladder cancer may include surgery, radiation, and chemotherapy. have suggested that an effect on the central nervous system is more likely, as lidocaine can depress consciousness and decrease cerebral metabolism. However, although it is 20 times more common in women, 16 . My 2nd surgery 28 were removed and it was discovered that I had 2 different types of lipomas. - Drug Monographs Hello Im all in for a Dercums group. As always, if you can't find the information you need, we're happy to hear from you. Meet The Dercum Society. Learn more about gallbladder cancer treatment. Type 2.Multiple lipomas associated with obesity in females.The most common type. Can you give me the name of a Dercum's Disease specialist in Lipomas are a common cause of fatty tissue all over the body. N Engl J Med. Do you know if Mayo", "@colleenyoung I was wondering if there is a way for people with Dercum's Disease, FML, Adiposis", "Welcome @iamrare2, I love the username. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 2040 minutes of CVAC process daily for 5 days. - Evidence-Based Guidance They may include Ehlers-Danlos Syndrome (mine is the Vascular type), Mast Cell Activation Syndrome (MCAS), allergies, Dysautonomia, thyroid issues, fibromyalgia symptoms, lymphedema, positive autoimmune testing and frustration! Although there is no cure for Dercums Disease, your doctor can work with you to develop a treatment plan tailored to your specific, unique health needs. As the aetiology of Dercum's disease is probably not inflammatory, it is plausible that the improvement some of the patients experience when using corticosteroids is not caused by an anti-inflammatory effect. Surgical excision or liposuction of selected lipomas may help, especially when they are in inconvenient locations. J Eur Acad Dermatol Venereol. Ive had 3 really bothersome lipomas removed 2 were angiolipomas. We are rare but there are many more out there! Dercum's Disease: Let's create a support group - Mayo Clinic Connect Dercum's disease mainly occurs in adults and more women are affected than men. We do not solicit nor accept donations. Patients have a multitude of systemic symptoms with variable incidence. This disease is inherited in the following pattern(s): Patient organizations can help patients and families connect. How do you look for it? Dercum's definitely does cause generalized inflammation and many suffer from Mast Cell Activation Syndrome so I'm continually on a variety of MCAS inhibitors (Wikipedia lays out the different classes, Drs. Several articles have also noted an increased risk for depression. Usually, lipomas dont need to be treated unless theyre bothering you. It's not clear why lipomas happen. Adiposis Dolorosa (Dercum Disease) - Medscape A person may develop Dercums disease due to: Other possible causes of Dercums disease include: Research has linked Dercums disease to a range of other health conditions, including: Dercums disease is underdiagnosed, so researchers do not know how frequently it occurs in the general population. Sebaceous Cysts: Symptoms, Diagnosis, and Treatment. [4], Corticosteroids The lumps under the skin can appear anywhere on the body, but they're most often found on the arms, legs, and trunk. It may be due to the growths pressing on nerve cells or inflammation of the fascia. [9], Common treatments for Dercum's disease is directed towards treating the individual symptoms. It's what leads to breakthroughs and what keeps you safe. My 3rd surgical removal was 42. I thought I share a few links in case you haven't already seen them and hopefully provide more information for members with this rare disorder. However, this has not been fully studied beyond a few isolated case studies and cannot be considered as concrete fact. Dercum disease has been reported to occur in families, and in some of these cases it seems to be inherited as an autosomal dominant trait (this means that one out of a person's two copies of the gene, inherited from one parent, is altered). Involvement of other medical care professionals may be called for, such as psychiatrists in bouts of depression, dieticans for weight reduction, and surgeons when a localized lipoma is botherosme Patient support groups can provide invalubale information to the patients and their families. Dercum's disease (adiposis dolorosa): a review of clinical presentation and management, Subcutaneous adipose tissue therapy reduces fat by dual X-ray absorptiometry scan and improves tissue structure by ultrasound in women with lipoedema and Dercum disease. Dercum's disease, or adiposis dolorosa, is a rare disorder which consists of multiple, painful lipomas within the subcutaneous tissue and has a distribution mainly in the abdomen and extremities. [3] Possible causes include genetics (autosomal dominant inheritance), trauma, nervous system dysfunction, disturbances in endocrine system and metabolism of fat, or it maybe an autoimmune disorder. Because your life may depend on it. Painful lipomas. Am J Med Sci 1892; 104: 52135. Pain Med 2008; 9(8): 12246. Others believe its a metabolic problem related to not being able to properly break down fat. https://www.healthline.com/health/dercums-disease#causes. The lipomas can form in any part of the body and range from pea-sized to plum-sized growths. In addition, Skagen et al. [1] Sometimes referred as adiposis dolorosa in medical literature, Dercums disease is more of a syndrome than a disease (because it has several clinically recognizable features, signs, and symptoms that are characteristic of it and that tend to occur together). Diagnosis of Dercum disease (adiposis dolorosa) is made clinically and is a diagnosis of exclusion. Do you know if Mayo Clinic can help? Other symptoms may include weakness, fatigue, and memory disturbances. On the other hand, the pain relief effect was the greatest at about 20 minutes after the end of the infusion. @pamelars60 and @hippygrandma both have mentioned that they have Dercums disease along with several other conditions, including fibromyalgia, and may have some experiences to share. Optimal Therapeutic Approach for Adiposis Dolorosa, Unusual Clinical Scenarios to Consider in Patient Management. Int J Obes (Lond). https://www.healthline.com/health/dercums-disease#causes, https://www.mayoclinichealthsystem.org/locations/eau-claire, https://www.mayoclinic.org/diseases-conditions/lipoma/symptoms-causes/syc-20374470, https://rarediseases.info.nih.gov/diseases/5750/adiposis-dolorosa, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6911249/. If those dont relieve the pain, cortisone shots, numbing agents, massage therapy, or electrical stimulation can help. (2010). We link primary sources including studies, scientific references, and statistics within each article and also list them in the resources section at the bottom of our articles. The origin of the pain is obscure, and the disease is better known as a description of its symptoms rather than as a physiologic or metabolic process. Due to the fact that adiposis dolorosa patients complain of multiple systemic symptoms, ranging from joint pains or low-grade fever to cognitive changes, an extensive work-up including many procedures is often perfotmed. How long have you been living with Dercum's? - Full-Length Features Some researchers have suggested that Dercum disease is an autoimmune disorder (a condition that occurs when the body's immune system attacks normal, healthy body tissue). 2009. pp. Are you under treatment by a doctor? The histologic appearances are similar with an encapsulated fatty deposit . which can in turn will let them see and understand how critical research is needed in this field of medicine. Treatment improving quality of life. Excessive exercise, high-impact activities, and any potential irritants should be avoided whenever possible. Dercum disease is a rare condition that is characterized by multiple, painful fatty tumors that occur chiefly in post-menopausal, obese women of middle age. Recent studies suggest that the lipomatosis in this disease is inflammatory and involves elevated interleukin (IL)-6 and connective tissue was increased fourfold. Please also check out our section on advice for simple lifestyle changes that several patients have found helpful in anecdotal instances. Thanks for sharing your work through Creative Commons! Colleen, as you can tell by my story I have lived with Dercum's years before my diagnosis. [4], Cyclic Variations in Adaptive Conditioning (CVAC) is a method of touch-free cyclic hypobaric pneumatic compression for treatment of tissue edema and, therefore, edema-associated pain. Four cardinal symptoms have sometimes been used as diagnostic criteria: Other potential signs of the disease include: However, as it is unclear which symptoms are cardinal and which symptoms are minor signs in Dercum's disease, it is unclear which should be used as diagnostic criteria. This page was last edited on 24 April 2023, at 02:04. Topics AZ Living with Dercums Disease can be a very painful and difficult challenge. [12][13], Dercum's disease most commonly appears between the ages of 35 and 50 years of age. It has been recognized by the World Health Organization in ICD-10. However, although it is 20 times more common in women, 16 percent of the reported cases are males and it can also occur in people who are not obese. There are two main types of clinical studies: People participate in clinical trials for a variety of reasons. Other lesions that may mimic a lipoma include: Dercum disease seems to benefit from the patient losing weight. Reference: Data from the Newborn Screening Codingand Terminology Guide is available here. performed an EEG during the administration of intravenous lidocaine. (A well-written differential diagnosis of adiposis dolorosa and its management.). 30 Apr 2012; 7: 23. Connect with thousands of patients and caregivers for support and answers. [6], The disease was described for the first time by an American neurologist Francis Xavier Dercum, who headed the University of Pennsylvanias Neurological Clinic and was the personal physician to President Woodrow Wilson. Multiple studies have found that traditional analgesics rarely have much affect, with opioids unfortunately being the most effective option for most patients. We hope you'll take a look! Any use of this site constitutes your agreement to the Terms of Use and Privacy Policy and Conditions of Use linked below. Fallahian F, Ardestani A, Pranckevicius E, Raut CP, Tavakkoli A, Sheu EG. Type 3. Painful subcutaneous plaques in Dercum disease. By Adrienne Dellwo People should note, however, that some medications and surgery may only provide temporary relief. Dominant means that only one copy of the responsible gene (causal gene) must have a disease-causing change (pathogenic variant) in order for a person to have the disease. What is Dercum disease? Intralesional lidocaine with cortisone to the lipomas have been also used, Oral lidocaine mexilletine an antiarrythmic in a dose up to 900mg per day monitoring ECG to rule out any arrythmia is required, Neuroleptics in particular pregablin in doses up to 300mg per day, Tricyclic antidepressants, such as amitriptyline and nortiptyline, Selective serotonin noradrenaline reuptake inhbitors, such as duloxetine and venlafaxine and mirtazapine. continue to be rare until primary doctors start to report patients who suffer from this to CDC, Nord or others who need to know about us. The cause of the pain is unclear and it has been suggested to be due to pressure on nerves in the lipoma, but histologic findings do not show any neural damage or proliferation. Researchers have not found specific risk factorsfor example, working a certain kind of job and being exposed to chemicalsthat make lipomas more likely to happen. The pain could be wide spread and not just involve the lipomas. Some people might have genetics that make them more likely to get lipomas than others. Dercum's Disease - KAREN L. HERBST, PHD, MD Nervous system diseases are usually diagnosed and treated by neurologists. Journal of Surgical Research. Registration is free. Youve viewed {{metering-count}} of {{metering-total}} articles this month. The disease has not been proven to be an autoimmune disorder or any other disorder. Although rare, the diagnosis may not include obesity. Learn more. In 2004 and with a new doctor and with more lipomas my new doctor agreed that I needed to see a surgeon. Your provider might want to do other tests to look for or rule out other conditions that can cause lumps before diagnosing the lump as a lipoma. Dont miss out on todays top content on Dermatology Advisor. Hello @iamrare2, Welcome to Connect. Dercums disease causes multiple, painful growths to occur throughout the body. However, in another patient with Dercum's disease, the effect of methotrexate was discreet. After treatment, there was a significant decrease in pain as measured by the Pain Catastrophizing Scale and the Visual Analogue Scale, but there was no change in pain quality by the McGill Pain Questionnaire. It usually appears between 35 and 50 years of age. We would like to hear your feedback as we continue to refine this new version of the GARD website. The lipomas are located primarily on the trunk region and on the extremities close to the trunk. After all, the principles of good medicine are the same whether youre treating a cold, cancer, or Dercums Disease. Females are 530 times more likely to develop Dercums disease than males. Skinmed. Intralesional steroids with lidocaine has been helpful, in the authors experience; however, there has been a report of a flare of the disease with the use of a high dose of corticosteroids. We avoid using tertiary references. Doctors do not know what causes the condition, but genetics may play a role. @colleenyoung I was wondering if there is a way for people with Dercum's Disease, FML, Adiposis Dolorosa and Lipomas to have it's own support group? The prevalence of Dercum's disease was found to range from 150 to 150,000 in the US population. It usually appears in postmenopausal women aged around 45-60 years . Its important to note, liposuction should not be used for the removal of lipomas. Meaning its root origin comes from a genetic mutation which affects the formation of fat cells. Iwane et al. If you have any concerns with your skin or its treatment, see a dermatologist for advice. Treatment of adiposis dolorosa is symptom specific and targeted towards pain relief . Do not assume that lumps and bumps are just lipomas. Some conditions may be mistaken for Dercums disease. This photo contains content that some people may find graphic or disturbing. If fibromyalgia is also an underlying problem, liposuction is not recommended. Dercum's disease (adiposis dolorosa) - Cambridge Core Surgical excision of many lipomas is not usually thought to be helpful and risks surgical complications. A recent case report of use of infliximab and methotrexate demonstrated significant improvement, upon discontinuation of the drug the patient experienced recurrent lipoma pain. This material may not be published, broadcast, rewritten or redistributed in any form without prior authorization. In this section we'll do our best to help provide basic advice about how to find the best doctor possible to help steer you through the diagnostic process and perhaps ultimately help you achieve the best quality of life as a Dercum's patient. Once the diagnosis is etsablished, it is important to inform the care takers to avoid unnecessary procedures, such as extensive surgical approaches, that may endanger an obese patient and will lead to complications, such as infections and worsening of this debilitating disease. Oral corticosteroids with prednisone 20mg per day reduced pain. Dercum disease - also known as Adiposis Dolorosa, Anders' syndrome and Dercum-Vitaut syndrome - is a rare condition that is characterized by multiple, painful fatty lipomas (benign, fatty tumors) that occur chiefly in post-menopausal, obese women of middle age. For example, blood tests can be used to check for genetic conditions and infections. Go to the Chronic Pain Support Group. Dercum's Fat Disorders Resource Society Learn About Scientific Research, We get a lot of questions from patients and their families. 2015;94(24):e950. 1031-8. Learn About Scientific Research, We get a lot of questions from patients and their families. If you have any friends in the area who also have complicated health issues even if theyre totally different than yours ask them for referrals too. [4] It is five to thirty times more common in women than in men. Dercum disease is also known as adiposis dolorosa. A rare condition called Dercum's disease can cause large, very painful lipomas. Biochemical analysis of fatty acid in the lipomas of Dercum patients demonstrated higher rate of monounsaturated fatty acids than saturated ones in comparsion to healthy subjects with lipomas. 151-9. We recommend the first one listed, as its the most complete introduction to Dercums Disease. [1], The onset of Dercum's Disease can be rapid or insidious and progressive, beginning most often in the third decade. Lipomas, DD, FML, will It causes fatty growths to develop underneath the skin or, in some cases, deeper inside the body. Although the pain itself varies, it may have the following characteristics: A person may also experience brain fog or fatigue during a pain flare-up. Other disorders related to, but different from, Dercums disease include: There is no cure for Dercums disease. KAREN L. HERBST, PHD, MD - Home Page for Karen L. Herbst, Ph.D., M.D. The next steps will depend on whether you have other health conditions, and whether the lipomas are bothering you. ClinicalTrials.gov, an affiliate of NIH, provides current information on clinical research studies in the United States and abroad. Hidradenitis suppurativa. However, there were no changes in the Pain Disability Index or Pittsburgh Sleep Quality Index. [4] Currently, there is a lack of scientific data on the use of integrative therapies for the treatment or prevention of Dercum's disease. Cortisone shots have also been shown to be effective in temporarily reducing the chronic pain. Nonetheless, it might also depress cerebral activity that could lead to increased pain thresholds. Some research suggests that having an injury to a part of the body might make a lipoma more likely to form there. The differential diagnosis of painful subcutaneous nodules in an obese female suspected with adiopsis dolorosa: Multiple lipomas can occur in other diseases, such as familial multiple lipomatosis and multiple symmetric lipomatosis ( Madelung`s disease). Adiposis dolorosa is 20 times more common in females. He referred me to different doctors to rule anything out. Complete your request online or contact us by phone. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. Intravenous lidocaine 400mg every other day provided pain relief for 10 hours to several months. However, the disease has been reported as showing an autosomal dominant pattern of inheritance in some families (ie the children of someone with Dercum disease have a 1-in-2 likelihood of also developing it.). It would make it simple for us to find the group and easier to reach out to each other. Doctors arent sure what causes Dercums disease. Dercum disease - also known as Adiposis Dolorosa or Anders' syndrome and is a rare condition that is characterized by multiple, painful fatty lipomas (benign, fatty tumors) that occur chiefly in women of middle age. For more information, please visit our lipoma section. 2) Chronic pain in the adipose tissue. If they show a smile and accept the opportunity, youll know theyre a good doctor. The glee of learning new things is an instantly recognizable sign of a compassionate, patient-centered doctor. Dercums disease is a rare, underdiagnosed disorder of the fat tissue. Why? I left the office crying never to see him again. Author: Dr Emily Ryder, Dermatology Registrar, Hamilton, New Zealand; Chief Editor: Dr Amanda Oakley, Dermatologist, Hamilton, New Zealand, May 2014. Dercum's Disease: Let's create a support group | Page 2 | Mayo Clinic

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