Lipedema is an uncommon disorder characterized by localized adiposity of the lower extremities, often occurring in females with a family history of the condition. The adiposity extends from hips to ankles and is typically unresponsive to weight loss.
It is important to distinguish Dercum’s disease (adiposis dolorosa) from lipedema, which is characterized by multiple painful lipomatous tumors, differing from the diffuse soft fatty deposition seen with lipedema.
***DERCUM’S DISEASE: AN OVERVIEW OF CLASSIFICATION, CLINICALPRESENTATION, DIAGNOSTIC CRITERIA AND MANAGEMENT
Dercum’s disease is a rare disorder described by generalized obesity with painful adipose tissue. The clinical symptoms presented were multiple painful fatty masses, fatiguablity, swelling of fingers, morning stiffness, cognitive dysfunction, headache, anxiety, rapid heartbeat, shortness of breath, bloating, constipation, easy bruisability, joint aches, muscle aches, mood swing, delirium and dementia. Dercum’s disease affects women more frequently than men. Elevated erythrocyte sedimentation rate, alpha-1 antitrypsin, orosomucoid, haptoglobin, compliment factors C3.C4, Clq and Cls have been found in Dercum’s disease. Differential diagnosis includes Fibromyalgia, Madelung’s, Familial multiple lipomatosis, Proteus syndrome, Weber-christian disease, Neurofibromatosis type1, Frohlich syndrome, Lipodystrophia and Metabolic disorders. The diagnosis is made clearly when the differential diagnoses have been excluded. The main goal of treatment in Dercum’s disease includes the pain reduction with surgical interventions (liposuction, excision), pharmacological therapies (analgesics, membrane stabilizing agents, corticosteroids, calcium channel modulators, methotrexate and infliximab, Interferon α-2b) and other alternative such as Rapid cycling hypobaric pressure and Frequency Modulated Electromagnetic Neural Stimulation. We propose a review on definition, classification, pathophysiology, diagnostic methods and treatment.
Rapid Weight Gain: A Common Presentation of Adipose Tissue Disorders
Weight gain has long been assumed to be a consequence of impaired energy balance due to poor feeding habits and low physical activity. We examined 40 patients who were referred for rapid weight gain or difficulty losing weight without obvious endocrine or non-endocrine causes. Most of the subjects also complained of fatigue and chronic pain that localized to the subcutaneous fat tissue. We examined our subjects systematically for subcutaneous adipose tissue accumulation in the upper back, deltoid regions, upper arms, trunk, abdomen, hips, buttocks and thighs. We also palpated for lipomas and fat nodules, and assessed abnormal or exaggerated fat distribution in unusual locations. Discrete lipomas in the abdomen, hips, buttocks and upper arms were frequenlty found in patients with rapid weight gain. More than half of our subjects have a painful adipose tissue disorder, adiposis dolorosa, (Dercum’s disease), mostly the nodular subtype. Three subjects had bilateral mastectomy because of mastalgia, 2 subjects have sciatic pain from lipoma growth in the spine, and 2 subjects had been hospitalized for panniculitis. The majority of the subjects have concurrent lipoedema, causing hip and thigh pain. History of previous surgery to remove lipomas is common, and tissue removed surgically in 3 subjects revealed angiolipomas. Other subjects who present with rapid weight gain have non-painful multiple symmetric lipomatosis, familial multiple lipomas, or generalized obesity. In conclusion, many subjects who present for rapid weight gain have abnormal lipomatous growth, which needs to be distinguished from generalized obesity. Dercum’s disease, or painful adipose tissue disorder, is a generally unrecognized condition that presents as rapid weight gain usually with chronic pain. The etiology of adiposis dolorosa remains to be determined.
Nothing to Disclose: A. A. Jamalallail, M.B.B.S., A. U. Rehman, A. Kaur, MBBS, B. C. Villafuerte, MD.
– See more at: http://press.endocrine.org/doi/abs/10.1210/endo-meetings.2016.OABA.8.FRI-644#sthash.oEbUXfe5.dpuf
Juxta-articular adiposis dolorosa (Dercum’s disease type IV): report of four cases and treatment by dermolipectomy
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.
Sci-Hub link: http://dabamirror.sci-hub.io/813967979df12375078741c4def28def/wollina2015.pdf
(2013) Knee dermolipectomy is a simple procedure with good results and few complications that improves the quality of life for obese patients. http://www.ncbi.nlm.nih.gov/pubmed/23254908
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.
*Case reports are generally considered a type of anecdotal evidence. Given their intrinsic methodological limitations, including lack of statistical sampling, case reports are placed at the foot of the hierarchy of clinical evidence, together with case series. https://en.wikipedia.org/wiki/Case_report