Subcutaneous adipose tissue therapy reduces fat by dual X-ray absorptiometry scan and improves tissue structure by ultrasound in women with lipoedema and Dercum disease.

Lipoedema is painful nodular subcutaneous adipose tissue (SAT) on legs and arms of women sparing the trunk. People with Dercum disease (DD) have painful SAT masses. Lipoedema and DD fat resists loss by diet and exercise. Treatments other than surgery are needed. Six women with lipoedema and one with DD underwent twelve 90-min sessions over 4 weeks. Body composition by dual X-ray absorptiometry scan, leg volume, weight, pain, bioimpedance, tissue size by caliper and ultrasound were analysed before and after SAT therapy by paired t-tests. There was a significant decrease from baseline to end of treatment in weight, 87.6 ± 21 to 86.1 ± 20.5 kg (P = 0.03), leg fat mass 17.8 ± 7.7 to 17.4 ± 7.6 kg (P = 0.008), total leg volume 12.9 ± 4 to 12 ± 3.5 L (P = 0.007), six of 20 calliper sites and tissue oedema. Pain scores did not change significantly. By ultrasound, six women had 22 hyperechoic masses in leg fat that resolved after treatment; five women developed seven new masses. Fascia improved by ultrasound after treatment. SAT therapy reduced amount and structure of fat in women with lipoedema and Dercum disease; studies are needed to compare SAT therapy to other therapies.

Trauma-induced adiposis dolorosa (Dercum's disease).

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.

Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods, classification and management.

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.

[Fatter through lipids or water. Lipohyperplasia dolorosa versus lymphedema]. / Dicker durch Fett oder Wasser. Lipohyperplasia dolorosa vs. Lymphödem.

Lipohyperplasia dolorosa and lymphedema are completely different disease entities, which are both, however, classified under lymphology. While in lipohyperplasia dolorosa a congenital lipid distribution disorder leads to a high volume insufficiency and the corresponding clinical symptoms, lymphedema is characterized by a congenital transport incompetence of the vessels or acquired disorders of transport capacity. Both lymphedemas of different genesis are familial volume alterations of the affected regions and the increase in volume is irreversible if not exclusively still in stage I or II. According to current knowledge the solid increase in volume by lymphedema is due to a malfunctioning biomechanism by which the release of additional proteoglycans in the homeostasis system of the fluid in the interstital space plays an important role. Removal of this tissue and the sponge-like substance of proteoglycans is the aim of therapeutic approaches. Manual lymph drainage and compression can evacuate the sponge but not remove it. Lymphological liposculpture is a successful dermatosurgical measure even for secondary lymphedema. Reduction of the necessity of complex hemostasis therapy to 20% of the initial value and an adjustment of the affected extremity on the healthy side, represent a clear improvement in quality of life of patients. The same dermatosurgical method, lymphological liposculpture, has been known for many years to fulfil the successfully proven purpose for the treatment of lipohyperplasia dolorosa by the removal of subcutaneous fatty tissue, present as hyperplasia and not hypertrophy. Tenderness and the necessity for complex hemostasis therapy are no longer present or no longer necessary after lymphological liposculpture for lipohyperplasia dolorosa. This condition is permanent because the congenital fatty masses do not reoccur following surgical removal. Lipohyperplasia dolorosa is therefore curable by lymphological liposculpture. For secondary lymphedema a drastic improvement in quality of life of the patient can be achieved by this method which is demonstrated by the adjustment of symmetry of the extremities and reduction or even avoidance of complex hemostasis therapy.

Dercum's disease: an unusual presentation.

Dercum's disease, also known as adiposis dolorosa, is a rare disease characterized by the accumulation of painful subcutaneous deposits of mature adult fatty tissue around the thighs, trunk, and upper arms and usually in a multifocal distribution. We are reporting an unusual presentation of Dercum's disease, presenting as a single painful, erythematous lesion around the left hip in a 71-year old postmenopausal woman. This report emphasizes the unusual presentation of adiposa dolorosa with a new modality for therapy. A summary of the major clinical associations, diagnostic challenges, and treatment modalities are also included in this manuscript.

Treatment of pain in Dercum's disease with Lidoderm (lidocaine 5% patch): a case report.

INTRODUCTION: Dercum's disease is a rare disorder characterized by multiple painful subcutaneous lipomas on the trunk and extremities. It most commonly occurs in obese, postmenopausal women. The pain associated with this condition is postulated to arise from enlarging lipomas producing pressure on peripheral nerves, thereby initiating pain and sometimes paresthesias. Treatment has been challenging due to the rarity of this condition. CASE: A patient with Dercum's disease successfully treated with transdermal lidocaine 5% patches. The patient's pain was initially rated as an 8/10. At follow-up examination after 1 month, the patient rated her pain as 3/10--a >60% reduction in pain; this pain reduction persisted at subsequent 1-month follow-up intervals. CONCLUSION: Current therapeutic options in the treatment of Dercum's disease have proven either ineffective or cumbersome. The use of transdermal lidocaine is a safe and non-invasive treatment modality that has been efficacious in alternate forms. The use of this medication might prove preferable to more invasive or risky treatment and warrants further investigation.

Obesity and the skin: skin physiology and skin manifestations of obesity.

UNLABELLED: Obesity is widely recognized as an epidemic in the Western world; however, the impact of obesity on the skin has received minimal attention. The purpose of this article is to highlight the association between obesity and dermatologic conditions. We review the impact of obesity on the skin, including skin physiology, skin manifestations of obesity, and dermatologic diseases aggravated by obesity. Obesity is responsible for changes in skin barrier function, sebaceous glands and sebum production, sweat glands, lymphatics, collagen structure and function, wound healing, microcirculation and macrocirculation, and subcutaneous fat. Moreover, obesity is implicated in a wide spectrum of dermatologic diseases, including acanthosis nigricans, acrochordons, keratosis pilaris, hyperandrogenism and hirsutism, striae distensae, adiposis dolorosa, and fat redistribution, lymphedema, chronic venous insufficiency, plantar hyperkeratosis, cellulitis, skin infections, hidradenitis suppurativa, psoriasis, insulin resistance syndrome, and tophaceous gout. We review the clinical features, evidence for association with obesity, and management of these various dermatoses and highlight the profound impact of obesity in clinical dermatology. LEARNING OBJECTIVE: After completing this learning activity, participants should be aware of obesity-associated changes in skin physiology, skin manifestations of obesity, and dermatologic diseases aggravated by obesity, and be able to formulate a pathophysiology-based treatment strategy for obesity-associated dermatoses.

Liposuction in Dercum's disease: impact on haemostatic factors associated with cardiovascular disease and insulin sensitivity.

OBJECTIVE: To study the impact of adipose tissue removal by liposuction on factors associated with increased risk of cardiovascular atherosclerotic disease within the coagulation and fibrinolytic system and glucose metabolism. DESIGN, SETTING AND SUBJECTS: Liposuction was performed in 53 patients with Dercum's disease. The levels of fibrinogen, von Willebrand factor antigen (VWF:Ag) and plasminogen activator inhibitor type 1 activity (PAI-1) were measured preoperatively, and 2 weeks, 4 weeks and 3 months postoperatively. In a subsample of 10 patients, insulin sensitivity was determined before and 2-4 weeks after surgery using the 2-h euglycaemic hyperinsulinaemic clamp technique. The study was performed as a single-centre study. MAIN OUTCOME MEASURE: Fibrinogen, PAI-1 and VWF:Ag levels, and glucose uptake before and after removal of adipose tissue. RESULTS: Weight reduction was sustained throughout the follow-up period with a mean decrease from 90.7 to 86.6 kg (P < 0.0001). There was a slight increase in levels of coagulation factors 2 and 4 weeks postoperatively, probably in reaction to the surgical trauma. After 3 months the values had returned to preoperative levels except for PAI-1, which still showed a slight increase (P < 0.05). In the subsample of 10 patients, glucose uptake was improved (P < 0.05) from a short-term perspective after surgery. CONCLUSION: Surgical removal of adipose tissue, without change in lifestyle, does not seem to improve the levels of coagulation and fibrinolytic factors associated with cardiovascular atherosclerotic disease, whereas glucose takeup may be facilitated and insulin sensitivity increases from a short-term perspective.

Long-standing pain relief of adiposis dolorosa (Dercum's disease) after intravenous infusion of lidocaine.

A 60-year-old woman with adiposis dolorosa for 20 years was treated with repeated intravenous infusions of lidocaine. Partial relief of pain in the legs was obtained after 1.3 gm. A dose of 5.2 gm lidocaine given for 4 days was needed for complete pain relief. The effect lasted for 3 weeks, and then the pain gradually returned. The patient has now been given two additional treatments with complete pain relief for 2 months. The mechanism of the effect remains unknown.

Dercum's disease (adiposis dolorosa). A case report and review of the literature.

Dercum's disease consists of multiple, painful lipomata and occurs in obese, postmenopausal women. A 52-year-old obese woman complained of multiple painful "lumps" in the extremities. The diagnosis of Dercum's disease (adiposis dolorosa) was established by excisional biopsy of four tumors. There is no effective treatment for this condition. Surgical excision, in this case, relieved the patient's symptoms locally, but after one year the painful lipomata had appeared at other sites. Case reports of this condition were not found in a review of the orthopedic literature.

The regulation of subcutaneous blood flow in patient with Dercum's disease.

Dercum's disease or adiposis dolorosa is a poorly understood disorder with painful fatty deposits in the skin localized to the lower extremities. The etiology is unknown. In such a patient the mechanisms of local regulation of blood flow in subcutaneous tissue was investigated by the local 133Xenon washout technique. The patient was reinvestigated one week after treatment with intravenous lidocaine. The local vasoconstrictor response to increase in venous transmural pressure was not present in this patient, but reappeared after lidocaine treatment. Autoregulation of blood flow in subcutaneous tissue was present before as well as after lidocaine treatment. It seems likely that a pain elicited increase in sympathetic activity in the vasoconstrictor fibres abolished the normal vasoconstrictor response to increase in venous transmural pressure. The mechanism of pain relief after intravenous lidocaine infusion is uncertain, but central as well as peripheral mechanisms may be considered.

A case of adiposis dolorosa: lipid metabolism and hormone secretion.

The present report describes a 53-year-old non-obese man with adiposis dolorosa whose pain was dramatically relieved by the intravenous injection of lidocaine. The patient showed a paradoxical response of growth hormone to thyrotropin-releasing hormone. In addition, in-vitro studies on adipose tissue metabolism revealed the reduced glucose conversion to neutral glycerides in painful adipose tissue. These abnormalities may be related in some ways to the pathogenesis of this disorder.