Prominent neurological involvement in Dercum disease

“Based on the present findings and data from the literature, neurological and neuropsychiatric manifestations, although not specific, are a relevant and probably under-estimated component of DD. The episode of dizziness followed by left side sensory-motor deficit might have been of vascular origin (namely, a transient ischemic attack), although it is still unclear whether the stroke-like events described in few reports of DD are caused by the condition itself or if their occurrence is merely coincidental. Notably, painful cutaneous lipomatosis has been related to microangiopathy and increased tissue fluid (lipedema),inflammatory reactions and fibrosis. Pain in lipedema is thought to result from hypoxia, inflammation, and necrosisof adipocytes; on the other hand, hyperbaric compression reduces tissue fluid and improves oxygen saturation, resulting in decreased pain in DD. Accordingly, these changes might represent a risk factor for lipohyalinotic andmicroatheromatous degeneration of the small penetrating cerebral vessels, given their limited caliber. The final result may be a modification of the local functioning of the striothalamic arterioles in the basal ganglia, eventually leading to ischemic lesions and enlarged perivascular spaces characteristic of microvascular disease. Systemic features of DD also include early onset spondy-loarthropathy, a finding which is in line with our report and with the occurrence of spine degeneration in DD. Although the etiology of DD is unknown, an inflammatory pathomechanism has been proposed, possibly leading to the release of pain-related neuropeptides, as suggested forother chronic pain syndromes. Finally, DD must be differentiated from other conditions including lipomas. Multiple lipomatosis is principally distinguished by large subcutaneous fatty masses, usually not painful, distributed in a symmetrical fashion, more prevalent in non-obese males and often associated with alcoholism. Sometimes,multiple lipomatosis is inherited as an autosomal-dominant trait, then referred to as FML. Benign adipose tissue tumors (solitary or multiple) are isolated subcutaneous tumors of well-differentiated adipocytes, some of which are painful; conversely, malignant cutaneous adipose tissue neoplasms are exceptionally rare and typically not painful.”

PDF: https://tinyurl.com/yd86s2dt
http://link.springer.com/article/10.1007/s00415-017-8415-1

 

 

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Case Report

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

Differentiating lipedema and Dercum’s disease

K. Beltran and K. L. Herbst

Abstract
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).
RESULTS:
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.
CONCLUSIONS:
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

Mixed generalized/juxta-articular form of Dercum’s disease

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.

http://www.termedia.pl/Journal/-18/pdf-28374-10?filename=Mixed%20generalized%20juxta-articular.pdf

 

 

Lipoma Treatment Review

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.
http://www.clinicaldermatology.eu/materiale_cic/876_3_4/7449_review/article.htm
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.

http://www.clinicaldermatology.eu/materiale_cic/876_3_4/7449_review/article.htm

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