Review of Dercums disease and proposal of diagnostic criteria, diagnostic methods, classification and management

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Abstract

Definition and clinical picture. We propose the minimal definition of Dercums disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercums 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 Dercums 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. Dercums disease most commonly appears between the ages of 35 and 50years and is five to thirty times more common in women than in men. The prevalence of Dercums 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 Dercums disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercums 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 Dercums disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon -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 Dercums disease should be treated in multidisciplinary teams specialised in chronic pain. Prognosis. The pain in Dercums disease seems to be relatively constant over time. © 2012Hansson et al.; licensee BioMed Central Ltd.

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Hansson, E., Svensson, H., & Brorson, H. (2012). Review of Dercums disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet Journal of Rare Diseases. https://doi.org/10.1186/1750-1172-7-23

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