Surgical Amelioration of Dercum's Disease: A Report and Review

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.

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“…Lipectomy has been tried in a few cases[23,25,43,50,61,67,90,91]. The treatment was effective, but the pain returned in all cases but one.…”

Section: Management Including Treatmentmentioning

confidence: 99%

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

Hansson

Svensson

Brorson

2012

Orphanet J Rare Dis

105

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“…Perivascular cells [203] , giant cells [204] , and granulomas suggestive of a foreign body reaction are apparent in some areas [205] . The histology of DD SAT can also appear normal [194,[206][207][208][209] .…”

Section: Diagnosismentioning

confidence: 99%

Rare adipose disorders (RADs) masquerading as obesity

2012

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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidencebased data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.

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“…Surgical therapies in the literature include lipoma resection and liposuction. Surgery has demonstrated only partial relief of the pain, with the advent of new painful lesions at nearby locations [5,13].…”

Section: Discussionmentioning

confidence: 99%

“…Most cases, however, tend to occur sporadically. Although the exact patho-physiology is unknown, it is postulated that the etiology of the disease is related to abnormal blood flow or circulatory dysfunction [5]. Treatment has been challenging due to the rarity of this condition.…”

Section: Introductionmentioning

confidence: 99%