Adipocytic tumors form a significant proportion of all benign and malignant musculoskeletal masses. This chapter focuses on the pathophysiology and clinical and imaging characteristics of the different benign and malignant subtypes presented according to the current WHO classification (Fletcher et al., World health organisation classification of tumors of soft tissue and bone, 4th edn. IARC Press, Lyon, 2013). MRI is emphasized throughout as this imaging technique, in particular, is most useful in this tumor group due to its ability to specifically characterize normal adipose tissue.
A 60-year-old woman affected by rheumatoid arthritis (RA) reported a 12-month history of progressive growth of multiple, painful and symmetrically distributed subcutaneous nodules affecting forearms, thighs and lower back. In the last months the size and the number of nodularities markedly increased, most recently with the appearance of a new lump every other week. These painful swellings had first appeared in the left forearm, with a single lesion progressively increasing in size, but later on similar lesions occurred in the other arm. On examination, multiple, tender and movable subcutaneous nodules with an increased consistency of sizes varying from 0.5–1.8cm were present on both forearms, thighs and lower back. The overlying skin did not show any abnormality but pain was evoked upon palpation with light pressure. The patient described the pain as burning, dull and persistent, slightly reduced during the night, ranging from 5 to 8/10 on a numeric rating scale in the last week. The histologic examination of a nodule of the left forearm showed only adipose tissue. Dercum’s disease (adiposis dolorosa) is a rare disorder of unknown etiology, first described in 1888, characterized by the development of multiple painful nodules of adipose tissue on the trunk and proximal parts of the extremities, most commonly affecting obese postmenopausal women.
Devis Benfaremo, Michele Maria Luchetti, Armando Gabrielli
Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy
Prominent neurological involvement in Dercum disease
(Abstract not provided, article behind paywall)
52 year old obese postmenopausal female presented with complaints of multiple painful lumps over the legs and thighs of 2 years days duration. Clinical examination revealed multiple tender plaques and nodules over both the legs and thighs of different sizes. Biopsy suggestive of lipoma. These features were diagnostic of a rare lipomatosis, dercum’s disease. https://www.omicsgroup.org/journals/dercums-diseaseadiposis-dolorosa-2376-0427-1000252.pdf
K. Beltran and K. L. Herbst
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.
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).
Differences between groups were by Student’s t-test with α of 0.05. The Lipedema Group had significantly greater weight, body mass index (BMI), gynoid distributed nodular SAT, and fibrotic and heavy tissue than the DD Group. Hypermobility was significantly higher in the Lipedema (58±0.5%) than DD Group (23±0.4%; P<0.0001). DM2 was significantly greater in the DD (16±0.2%; P=0.0007) than the Lipedema Group (6±0.2%). Average pain by an analog scale was significantly higher in the DD (6±2.5%) than the Lipedema Group (4±2.1%; P<0.0001). Fatigue and swelling were common in both groups. Easy bruising was more common in the Lipedema Group whereas abdominal pain, shortness of breath, fibromyalgia, migraines and lipomas were more prevalent in the DD Group. The percentage of patients with elevated CH50 was significantly positive in both groups.
The significantly lower prevalence of DM2 in people with lipedema compared to DD may be due to the greater amount of gynoid fat known to be protective against metabolic disorders. The high percentage of hypermobility in lipedema patients indicates that it may be a co-morbid condition. The location of fat, high average daily pain, presence of lipomas and co-morbid painful disorders in DD patients may help differentiate from lipedema.
International Journal of Obesity accepted article preview 18 November 2016; doi: 10.1038/ijo.2016.205 http://www.nature.com/ijo/journal/vaop/naam/abs/ijo2016205a.html
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
The clinical and imaging features are consistent with focal adiposis dolorosa likely associated with long-term glucocorticoid use. Adiposis dolorosa (Dercum’s disease) refers to painful fatty tissue in an overweight or obese individual. The epidemiology, etiology and pathogenesis of adiposis dolorosa are not clear but long-term glucocorticoid use for rheumatologic diseases has been associated with focal adiposis dolorosa.
Full Text & Images: http://journals.aace.com/doi/pdf/10.4158/EP161236.VV
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