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.
Differential diagnosis excluded fibromyalgia, benign symmetric lipomatosis, lipedema, adenolipomatosis, and panniculitis.
Dercum’s disease appears in nodular, generalized and juxta-articular forms. In this short report, we report a patient with mixed generalized/juxta-articular form. The patient responded well to medication with paracetamol administered in low doses. We recommended continuation of this treatment as well as monitoring for diabetes or glucose intolerance. Metabolic disturbances are common in patients with Dercum’s disease.
The following link provides a review of lipoma excision techniques and possible alternative technologies that have been tested or are being developed. Of the reviewed methods, liposuction is the most effective overall in removing adipose tissue, but is hindered by associated costs. Deeper lipomas may be ablated with laser technologies, although larger incisions are required. Additionally, study results of ultrasound and pharmaceutical methods are promising, but require FDA approval before becoming a prevalent practice.
Conclusion: Although lipoma affects a large portion of the population, the majority of removal procedures for subcortical lipomas are still performed with a standard set of scalpels and scissors. Unfortunately, the standard methods can result in significant scarring. The most effective adipose tissue removal method that is FDA approved is liposuction, but the required equipment and training are cost prohibitive for many surgeons. New methods are desired to effectively remove an entire lipoma with reduced scarring and costs. Ultrasound and pharmaceutical methods have shown promise, but still require FDA approval. Laser technologies have been effective for deeper lipomas, but require very large incisions. Our society and the industry would benefit from the development of a novel, directed approach to lipoma excision, which may utilize a combination of components from the many emerging technologies.
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