-
Arquivos Brasileiros de Cardiologia Nov 2018
Topics: 1-Alkyl-2-acetylglycerophosphocholine Esterase; Adiposis Dolorosa; C-Reactive Protein; Child; Cholesterol, HDL; Dyslipidemias; Female; Humans; Lipomatosis; Lipoproteins; Pain; Rare Diseases
PubMed: 30484519
DOI: 10.5935/abc.20180191 -
BMJ Case Reports Mar 2018We 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...
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.
Topics: Accidents, Traffic; Adipose Tissue; Adiposis Dolorosa; Adult; Amitriptyline; Analgesics, Non-Narcotic; Angiomyolipoma; Baclofen; Chronic Pain; Clonidine; Diagnosis, Differential; Humans; Ibuprofen; Male; Muscle Relaxants, Central; Tomography, X-Ray Computed; Upper Extremity
PubMed: 29592996
DOI: 10.1136/bcr-2017-223869 -
Plastic and Reconstructive Surgery.... Sep 2016Lipedema, or adiposis dolorosa, is a common adipose tissue disorder that is believed to affect nearly 11% of adult women worldwide. It is characterized most commonly by...
Lipedema, or adiposis dolorosa, is a common adipose tissue disorder that is believed to affect nearly 11% of adult women worldwide. It is characterized most commonly by disproportionate adipocyte hypertrophy of the lower extremities, significant tenderness to palpation, and a failure to respond to extreme weight loss modalities. Women with lipedema report a rapid growth of the lipedema subcutaneous adipose tissue in the setting of stress, surgery, and/or hormonal changes. Women with later stages of lipedema have a classic "column leg" appearance, with masses of nodular fat, easy bruising, and pain. Despite this relatively common disease, there are few physicians who are aware of it. As a result, patients are often misdiagnosed with lifestyle-induced obesity, and/or lymphedema, and subjected to unnecessary medical interventions and fat-shaming. Diagnosis is largely clinical and based on criteria initially established in 1951. Treatment of lipedema is effective and includes lymphatic support, such as complete decongestive therapy, and specialized suction lipectomy to spare injury to lymphatic channels and remove the diseased lipedema fat. With an incidence that may affect nearly 1 in 9 adult women, it is important to generate appropriate awareness, conduct additional research, and identify better diagnostic and treatment modalities for lipedema so these women can obtain the care that they need and deserve.
PubMed: 27757353
DOI: 10.1097/GOX.0000000000001043 -
Medicine Jun 2015Dercum'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...
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). After FREMS treatment the patient's clinical conditions significantly improved, with reduction of pain on the VAS scale from 64 to 17 points, improvement of daily life abilities (the Barthel index increased from 12 to 18) and amelioration of health status (higher scores than baseline in all Short Form-36 domains). Furthermore, we documented a 12 mm reduction in subcutaneous adipose tissue thickness at the abdominal wall and a 7040 g decrease in total body fat mass. FREMS therapy proved to be effective and safe in the treatment of this rare and disabling condition.
Topics: Adipose Tissue; Adiposis Dolorosa; Body Composition; Body Mass Index; Disability Evaluation; Electric Stimulation Therapy; Health Status; Humans; Male; Middle Aged
PubMed: 26091459
DOI: 10.1097/MD.0000000000000950 -
Annals of Clinical and Translational... Mar 2014Francis 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...
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. From anatomy, histology, pathology, and neurology to teaching, writing, and philosophy, Dercum's life was that of a man for all seasons.
PubMed: 25356401
DOI: 10.1002/acn3.36 -
Obesity (Silver Spring, Md.) Oct 2014Investigational, near-infrared fluorescence (NIRF) lymphatic imaging was used to assess lymphatic architecture and contractile function in participants diagnosed with... (Clinical Trial)
Clinical Trial
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. Each participant received multiple intradermal and subcutaneous injections of indocyanine green (ICG, total dose ≤400 µg) in arms, legs, and/or trunk. Immediately after injection, ICG was taken up by the lymphatics and NIRF imaging was conducted.
RESULTS
The lymphatics in the participants with Dercum's disease were intact and dilated, yet sluggishly propelled lymph when compared to control lymphatics. Palpation of regions containing fluorescent lymphatic pathways revealed tender, fibrotic, tubular structures within the subcutaneous adipose tissue that were associated with painful nodules, and, in some cases, masses of fluorescent tissue indicating that some lipomas may represent tertiary lymphoid tissues.
CONCLUSIONS
These data support the hypothesis that Dercum's disease may be a lymphovascular disorder and suggest a possible association between abnormal adipose tissue deposition and abnormal lymphatic structure and function.
Topics: Adiposis Dolorosa; Female; Humans; Indocyanine Green; Infrared Rays; Lymphatic Diseases; Lymphatic System; Middle Aged; Optical Imaging; Pain; Phenotype; Subcutaneous Fat
PubMed: 25044620
DOI: 10.1002/oby.20836 -
BMC Psychiatry Jul 2012Dercum's disease is characterised by pronounced pain in the adipose tissue and a number of associated symptoms. The condition is usually accompanied by generalised...
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.
METHODS
A total of 111 women fulfilling the clinical criteria of Dercum's disease were included. As controls, 40 obese healthy women were recruited. To measure depression, the Montgomery Åsberg Depression Rating Scale (MADRS) was used.
RESULTS
According to the total MADRS score, less than half of the patients were classified as having "no depression" (44%), the majority had "light" or "moderate depression" (55%) and one individual had "severe depression" in the Dercum group. In the control groups, the majority of the patients were classified as having "no depression" (85%) and a small number had "light depression" (15%). There was a statistically significant difference for the total MADRS score between the two groups (p = 0.014).
CONCLUSION
The results indicate that the patients with Dercum's disease are more likely to suffer from depression than controls.
Topics: Adiposis Dolorosa; Adult; Aged; Case-Control Studies; Depressive Disorder; Female; Humans; Middle Aged; Obesity; Psychiatric Status Rating Scales; Severity of Illness Index
PubMed: 22759645
DOI: 10.1186/1471-244X-12-74 -
Orphanet Journal of Rare Diseases Apr 2012DEFINITION AND CLINICAL PICTURE: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose... (Review)
Review
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. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form - a form with pain in and around multiple lipomas IV. Juxtaarticular form - a form with solitary deposits of excess fat for example at the medial aspect of the knee.
EPIDEMIOLOGY
Dercum's disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum's disease has not yet been exactly established.
AETIOLOGY
Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma. DIAGNOSIS AND DIAGNOSTIC METHODS: Diagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded.
DIFFERENTIAL DIAGNOSIS
Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours. GENETIC COUNSELLING: The majority of the cases of Dercum's disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum's disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition. MANAGEMENT AND TREATMENT: The following treatments have lead to some pain reduction in patients with Dercum's disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum's disease should be treated in multidisciplinary teams specialised in chronic pain.
PROGNOSIS
The pain in Dercum's disease seems to be relatively constant over time.
Topics: Adipose Tissue; Adiposis Dolorosa; Chronic Disease; Female; Humans; Male; Obesity; Pain; Rare Diseases; Severity of Illness Index
PubMed: 22546240
DOI: 10.1186/1750-1172-7-23 -
Journal of Pain Research Aug 2010Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in...
Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia. Sequential compression relieves lymphedema pain; we therefore hypothesized that whole body cyclic pneumatic hypobaric compression may relieve pain in AD. To avoid exacerbating hyperalgesia, we utilized a touch-free method, which is delivered via a high-performance altitude simulator, the Cyclic Variations in Altitude Conditioning™ (CVAC™) process. As a pilot study, 10 participants with AD completed pain and quality of life questionnaires before and after 20-40 minutes of CVAC process daily for 5 days. Participants lost weight (195.5 ± 17.6-193.8 ± 17.3 lb; P = 0.03), and bioimpedance significantly decreased (510 ± 36-490 ± 38 ohm; P = 0.01). There was a significant decrease in scores on the Pain Catastrophizing Scale (P = 0.039), in average (P = 0.002), highest (P = 0.029), lowest (P = 0.04), and current pain severity (P = 0.02) on the Visual Analogue Scale, but there was no change in pain quality by the McGill Pain Questionnaire. There were no significant changes in total and physical SF-36 scores, but the mental score improved significantly (P = 0.049). There were no changes in the Pain Disability Index or Pittsburgh Sleep Quality Index. These data present a potential, new, noninvasive means of treating pain in AD by whole body pneumatic compression as part of the CVAC process. Although randomized, controlled trials are needed to confirm these data, the CVAC process could potentially help in treating AD pain and other chronic pain disorders.
PubMed: 21197318
DOI: 10.2147/JPR.S12351 -
The Journal of Headache and Pain Dec 2010A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back....
A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back. Six weeks prior to the presentation to our service, she developed severe occipital headache refractory to drug treatment. She improved after bilateral greater occipital nerve blocks. She was subjected to bilateral greater occipital chemical neurolysis which has given her complete pain relief.
Topics: Adiposis Dolorosa; Diagnosis, Differential; Female; Headache Disorders; Humans; Middle Aged; Neuralgia; Scalp; Spinal Nerves
PubMed: 20811764
DOI: 10.1007/s10194-010-0253-9