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Dercum's disease

David Hao, Akintomi Olugbodi, Nneka Udechukwu, Anthony A Donato
We present a case of a 39-year-old man who presented with chronic bilateral upper extremity pain associated with innumerable angiomyolipomas that developed 5 years after a motor vehicle accident involving his upper extremities. Our case notes the rare nature of painful adipose tissue deposits and the diagnostic challenges.
March 28, 2018: BMJ Case Reports
Sehem Ghazala, Jawad Bilal, Elle Ross, Irbaz Bin Riaz, Bobby Kalb, Karen L Herbst
BACKGROUND: Dercum disease is a rare disorder of painful subcutaneous adipose tissue masses typically presenting as a constellation of signs and symptoms affecting most organs, including slow lymphatic flow and fatty liver. METHOD: The University of Arizona Institutional Review Board considered this report exempt after patient consent. Multislice, multisequence magnetic resonance imaging (MRI) of the abdomen and pelvis was performed prior to and after d-amphetamine, with and without intravenous gadolinium...
January 31, 2018: American Journal of Medicine
K Beltran, K L Herbst
BACKGROUND: People with lipedema or Dercum's disease (DD) can have a similar distribution of excess painful nodular subcutaneous adipose tissue (SAT), making them difficult to differentiate. METHODS: Case series of 94 patients with DD, 160 with lipedema and 18 with both diagnoses (Lip+DD) from a single clinic in an academic medical center to improve identification and differentiation of these disorders by comparison of clinical findings, prevalence of type 2 diabetes (DM2), hypermobility by the Beighton score and assessment of a marker of inflammation, Total complement activity (CH50)...
February 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
Eugeniusz J Kucharz, Justyna Kramza, Przemysław Kotyla, Anna Kotulska
Dercum's disease is a rare disorder with subcutaneous formation of fatty tissue (lipomas) with symptoms of pain, fatigue, stiffness, weakness and in some cases arthritis. The rarity of this disease causes that it is not taken into consideration in the rheumatological differential diagnosis, so this short report draws attention to this rare disease.
2016: Reumatologia
Uwe Wollina, Birgit Heinig, Dana Langner, Andreas Nowak
Juxta-articular adiposis dolorosa is a rare subtype of Dercum's disease. It manifests mainly on the medial parts of the knees. Pain and impaired mobility are common symptoms. We report on four females (aged between 52 and 83 years) who suffered from juxta-articular adiposis dolorosa for more than 10 years. These patients were successfully treated by dermolipectomy resulting in dramatically improved pain and mobility. Adverse effects and complications were minor with a lymph fistula in a single patient which was treated by surgery...
September 2015: Wiener Medizinische Wochenschrift
Sabina Martinenghi, Amelia Caretto, Claudio Losio, Marina Scavini, Emanuele Bosi
Dercum's disease is a rare condition of painful subcutaneous growth of adipose tissue. Etiology is unknown and pain is difficult to control. We report the case of a 57-year-old man with generalized diffuse Dercum's disease, who improved after the treatment with transcutaneous frequency rhythmic electrical modulation system (FREMS). Treatment consisted in 4 cycles of 30 minutes FREMS sessions over a 6-month period. Measures of efficacy included pain assessment (visual analogue scale, VAS), adipose tissue thickness by magnetic resonance imaging, total body composition and regional fat mass by dual-energy X-ray absorptiometry, physical disability (Barthel index), and health status (Short Form-36 questionnaire)...
June 2015: Medicine (Baltimore)
Jonelle M Petscavage-Thomas, Eric A Walker, Stephanie A Bernard, Jennifer Bennett
PURPOSE: Adiposis dolorosa (Dercum's disease) is a condition of benign, painful subcutaneous lipomatous lesions associated with weakness, endocrine and lipid abnormalities, and mental disturbances. There is little information documenting the cross-sectional imaging findings that differentiate it from lipomatous and neoplastic soft tissue masses, or massive localized lymphedema. The purpose of this study was to provide a radiological case series of adiposis dolorosa. METHODS: A 10-year retrospective review of the picture archiving and communications system was performed...
June 2015: Skeletal Radiology
Panta Rouhani Schaffer, Christopher S Hale, Shane A Meehan, Jerome L Shupack, Sarika Ramachandran
We report a 46-year-old woman with a nine-year history of obesity; chronic diffuse pain in the adipose tissue of her medial upper arms, lower trunk, and thighs; multiple biopsy-proven lipomas and angiolipomas; and a lipomatous pseudomass of the breast. Her systemic symptoms included generalized weakness, fatigue, memory impairment, and arthralgias. Although some of the lesions were tender, most were only appreciated with palpation. Her clinical history and histopathologic data suggested adiposis dolorosa (Dercum's disease)...
December 2014: Dermatology Online Journal
Dina A Patel, Kenneth G Swan
Francis Xavier Dercum (1856-1931) is known primarily for his prominence in the field of neurology and for his identification of "Adiposis Dolorosa", known as Dercum's disease. His brilliance, however, extends well beyond neurology. Born and raised in Philadelphia to parents of American and European descent, Dercum's natural curiosity oriented his interests toward medicine and philosophy. His scholarship flourished, and much recognition came his way. He died in Benjamin Franklin's Chair, closing what would be his last session as President of the American Philosophical Society...
March 2014: Annals of Clinical and Translational Neurology
John C Rasmussen, Karen L Herbst, Melissa B Aldrich, Chinmay D Darne, I-Chih Tan, Banghe Zhu, Renie Guilliod, Caroline E Fife, Erik A Maus, Eva M Sevick-Muraca
OBJECTIVE: Investigational, near-infrared fluorescence (NIRF) lymphatic imaging was used to assess lymphatic architecture and contractile function in participants diagnosed with Dercum's disease, a rare, poorly understood disorder characterized by painful lipomas in subcutaneous adipose tissues. METHODS: After informed consent and as part of an FDA-approved feasibility study to evaluate lymphatics in diseases in which their contribution has been implicated, three women diagnosed with Dercum's disease and four control subjects were imaged...
October 2014: Obesity
B J Tins, C Matthews, M Haddaway, V N Cassar-Pullicino, R Lalam, J Singh, P N M Tyrrell
AIM: To describe ultrasound and magnetic resonance imaging (MRI) features of adiposis dolorosa, Dercum's disease, and to evaluate the MRI features prospectively against a large number of MRI examinations. MATERIALS AND METHODS: Institutional review board approval for this study was obtained. The imaging features at MRI and ultrasound of 13 cases of adiposis dolorosa (nine female, four male; age range 32-72 years) were reviewed. MRI findings typical for adiposis dolorosa were proposed and prospectively evaluated on 6247 MRI examinations performed over a period of 8 months...
October 2013: Clinical Radiology
Krzysztof Łabuzek, Sebastian Liber, Dariusz Suchy, Bogusław Okopień
In this case report, we describe a patient with Dercum's disease who was successfully managed with metformin. The administration of metformin reduced pain intensity from 9/10 to 3/10 and favorably affected the profile of inflammatory cytokines (i.e., TNF a, IL-1β, IL-6, and IL-10), adipokines (i.e., adiponectin, leptin, and resistin), and β-endorphin. Because each variable was affected moderately by the drug, in the range of 20 - 30%, it follows that these effects are additive, i.e., they act independently of each other...
June 2013: International Journal of Clinical Pharmacology and Therapeutics
Emma Hansson, Jonas Manjer, Henry Svensson, Håkan Brorson
Dercum's disease is characterised by obesity, pronounced pain in the adipose tissue, and a number of associated symptoms. Liposuction has been suggested as a treatment. However, the effect on quality-of-life after liposuction in Dercum's disease has never been investigated. The objective of this study was to examine the quality-of-life in Dercum's disease before and after liposuction. A total of 114 women fulfilling the clinical criteria of Dercum's disease were included. Of the 114 women, 53 were operated on with liposuction and 61 were Dercum controls...
September 2012: Journal of Plastic Surgery and Hand Surgery
E Hansson, J Manjer, H Svensson, M Åberg, B Fagher, R Ekman, H Brorson
Dercum's disease (adiposis dolorosa) is characterised by adiposity and chronic pain in the adipose tissue. It has been proposed that conditions encompassing chronic pain have altered concentrations of neuropeptides involved in pain transmission. The aim of this investigation was to examine whether patients with Dercum's disease have abnormal concentrations of different neuropeptides. In cerebrospinal fluid (CSF) and in plasma (P) from 53 patients with Dercum's disease substance P-like immunoreactivity (SP-LI), neuropeptide Y-like immunoreactivity (NPY-LI), β-endorphin-like immunoreactivity (β-END-LI), calcitonin gene-related peptidelike immunoreactivity (CGRP-LI), met-enkephalin-like immunoreactivity (m-ENK-LI), vasoactive intestinal polypeptide-like immunoreactivity (VIP-LI), somatostatin (SOM-LI), γ2-melanocyte-stimulating hormone-like immunoreactivity (γ2-MSH-LI), and dynorphin-like immunoreactivity (DYN-LI) were measured...
2012: Reumatismo
Emma Hansson, Henry Svensson, Håkan Brorson
BACKGROUND: Dercum's disease is characterised by pronounced pain in the adipose tissue and a number of associated symptoms. The condition is usually accompanied by generalised weight gain. Many of the associated symptoms could also be signs of depression. Depression in Dercum's disease has been reported in case reports but has never been studied using an evidence-based methodology. The aim of this study was to examine the presence of depression in patients with Dercum's disease compared to obese controls that do not experience any pain...
2012: BMC Psychiatry
Marie-Christine Vantyghem, Anne-Sophie Balavoine, Claire Douillard, Frédérique Defrance, Lucile Dieudonne, Fanny Mouton, Christine Lemaire, Nicole Bertrand-Escouflaire, Marie-Françoise Bourdelle-Hego, Fabrice Devemy, Anne Evrard, Dominique Gheerbrand, Caroline Girardot, Sophie Gumuche, Christine Hober, Hélène Topolinski, Blandine Lamblin, Bénédicte Mycinski, Amélie Ryndak, Wassila Karrouz, Etienne Duvivier, Emilie Merlen, Christine Cortet, Jacques Weill, Dominique Lacroix, Jean-Louis Wémeau
The spectrum of adipose tissue diseases ranges from obesity to lipodystrophy, and is accompanied by insulin resistance syndrome, which promotes the occurrence of type 2 diabetes, dyslipidemia and cardiovascular complications. Lipodystrophy refers to a group of rare diseases characterized by the generalized or partial absence of adipose tissue, and occurs with or without hypertrophy of adipose tissue in other sites. They are classified as being familial or acquired, and generalized or partial. The genetically determined partial forms usually occur as Dunnigan syndrome, which is a type of laminopathy that can also manifest as muscle, cardiac, neuropathic or progeroid involvement...
June 2012: Annales D'endocrinologie
Emma Hansson, Henry Svensson, Håkan Brorson
UNLABELLED: DEFINITION AND CLINICAL PICTURE: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum's disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches. CLASSIFICATION: We suggest that Dercum's disease is classified into: I...
April 30, 2012: Orphanet Journal of Rare Diseases
Karen L Herbst
Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD...
February 2012: Acta Pharmacologica Sinica
Jennifer K Yee, Susan A Phillips, Kambiz Allamehzadeh, Karen L Herbst
BACKGROUND: Elevated stearoyl-CoA desaturase activity has been described in obese states, with an increased desaturation index (DI) suggesting enhanced lipogenesis. Differences in the DI among various phenotypes of abnormal adiposity have not been studied. Abnormal accumulation of subcutaneous adipose tissue occurs in rare adipose disorders (RADs) including Dercum's disease (DD), multiple symmetric lipomatosis (MSL), and familial multiple lipomatosis (FML). Examining the DI in subcutaneous fat of people with DD, MSL and FML may provide information on adipose tissue fatty acid metabolism in these disorders...
2012: Lipids in Health and Disease
Emma Hansson, Henry Svensson, Unne Stenram, Håkan Brorson
BACKGROUND: Dercum's disease (DD) is characterised by obesity and chronic pain (> 3 months) in the adipose tissue. The pathogenesis of DD is unknown, but inflammatory components have been proposed. In previous reports and studies, an inconsistent picture of the histological appearance of the adipose tissue in DD has been described. The aim of this investigation was to examine the histological appearance of adipose tissue in patients with DD, with particular focus on inflammatory signs...
2011: Journal of Inflammation
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