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      Review of Dercum’s 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 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.

          Related collections

          Most cited references101

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          Overweight and obesity are associated with psychiatric disorders: results from the National Epidemiologic Survey on Alcohol and Related Conditions.

          This study evaluated associations between body mass index (BMI) and psychiatric disorders. Data from 41,654 respondents in the National Epidemiologic Survey on Alcohol and Related Conditions were analyzed. After controlling for demographics, the continuous variable of BMI was significantly associated with most mood, anxiety, and personality disorders. When persons were classified into BMI categories of underweight, normal weight, overweight, obese, and extremely obese, both obese categories had significantly increased odds of any mood, anxiety, and alcohol use disorder, as well as any personality disorder, with odds ratios (ORs) ranging from 1.21 to 2.08. Specific Diagnostic and Statistical Manual of Mental Disorders-revision IV mood and personality disorders associated with obesity included major depression, dysthmia, and manic episode (ORs, 1.45-2.70) and antisocial, avoidant, schizoid, paranoid, and obsessive-compulsive personality disorders (ORs, 1.31-2.55). Compared with normal weight individuals, being moderately overweight was significantly associated with anxiety and some substance use disorders, but not mood or personality disorders. Specific anxiety disorders that occurred at significantly higher rates among all categories of persons exceeding normal weight were generalized anxiety, panic without agoraphobia, and specific phobia (ORs, 1.23-2.60). Being underweight was significantly related to only a few disorders; it was positively related to specific phobia (OR, 1.31) and manic episode (OR, 1.83), and negatively associated with social phobia (OR, 0.60), panic disorder with agoraphobia (OR, 0.40), and avoidant personality disorder (OR, 0.59). These data provide a systematic and comprehensive assessment of the association between body weight and psychiatric conditions. Interventions addressing weight loss may benefit from integrating treatment for psychiatric disorders.
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            Multiple endocrine neoplasia type 1

            Multiple Endocrine Neoplasia type 1 (MEN1) is a rare autosomal dominant hereditary cancer syndrome presented mostly by tumours of the parathyroids, endocrine pancreas and anterior pituitary, and characterised by a very high penetrance and an equal sex distribution. It occurs in approximately one in 30,000 individuals. Two different forms, sporadic and familial, have been described. The sporadic form presents with two of the three principal MEN1-related endocrine tumours (parathyroid adenomas, entero-pancreatic tumours and pituitary tumours) within a single patient, while the familial form consists of a MEN1 case with at least one first degree relative showing one of the endocrine characterising tumours. Other endocrine and non-endocrine lesions, such as adrenal cortical tumours, carcinoids of the bronchi, gastrointestinal tract and thymus, lipomas, angiofibromas, collagenomas have been described. The responsible gene, MEN1, maps on chromosome 11q13 and encodes a 610 aminoacid nuclear protein, menin, with no sequence homology to other known human proteins. MEN1 syndrome is caused by inactivating mutations of the MEN1 tumour suppressor gene. This gene is probably involved in the regulation of several cell functions such as DNA replication and repair and transcriptional machinery. The combination of clinical and genetic investigations, together with the improving of molecular genetics knowledge of the syndrome, helps in the clinical management of patients. Treatment consists of surgery and/or drug therapy, often in association with radiotherapy or chemotherapy. Currently, DNA testing allows the early identification of germline mutations in asymptomatic gene carriers, to whom routine surveillance (regular biochemical and/or radiological screenings to detect the development of MEN1-associated tumours and lesions) is recommended.
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              Relationship between fibromyalgia and obesity in pain, function, mood, and sleep.

              Fibromyalgia syndrome (FMS) is a prevalent and disabling chronic pain disorder. Past research suggests that obesity is a common comorbidity and may be related to the severity of FMS. The main objective of the present study was to evaluate the relationships between FMS and obesity in the multiple FMS-related domains: hyperalgesia, symptoms, physical abilities, and sleep. A total of 215 FMS patients completed a set of self-report inventories to assess FMS-related symptoms and underwent the tender point (TP) examination, physical performance testing, and 7-day home sleep assessment. Forty-seven percent of our sample was obese and an additional 30% was overweight. Obesity was related significantly to greater pain sensitivity to TP palpation particularly in the lower body areas, reduced physical strength and lower-body flexibility, shorter sleep duration, and greater restlessness during sleep. The results confirmed that obesity is a prevalent comorbidity of FMS that may contribute to the severity of the problem. Potential mechanisms underlying the relationship are discussed. This report presents how obesity may be interrelated to fibromyalgia pain, disability, and sleep. We found that obesity is common in FMS. Approximately half of our patients were obese and an additional 30% were overweight. We also found that obesity in FMS was associated with greater pain sensitivity, poorer sleep quality, and reduced physical strength and flexibility. The results suggest that obesity may aggregate FMS and weight management may need to be incorporated into treatments. Copyright © 2010 American Pain Society. Published by Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central
                1750-1172
                2012
                30 April 2012
                : 7
                : 23
                Affiliations
                [1 ]Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö, Sweden
                [2 ]Plastic and Reconstructive Surgery, Skåne University Hospital, SE-205 02, Malmö, Sweden
                Article
                1750-1172-7-23
                10.1186/1750-1172-7-23
                3444313
                22546240
                420355a9-2903-4ceb-9d75-9b10fc04eba8
                Copyright ©2012 Hansson et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 September 2011
                : 6 April 2012
                Categories
                Review

                Infectious disease & Microbiology
                adiposalgia,chronic pain,diagnostic criteria,adiposis dolorosa,adipose tissue,dercum’s disease

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