Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management
Identifieur interne : 004D29 ( Ncbi/Checkpoint ); précédent : 004D28; suivant : 004D30Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management
Auteurs : Emma Hansson [Suède] ; Henry Svensson [Suède] ; H Kan Brorson [Suède]Source :
- Orphanet Journal of Rare Diseases [ 1750-1172 ] ; 2012.
Abstract
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.
We suggest that Dercum’s disease is classified into: I.
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.
Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.
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 diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours.
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.
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.
The pain in Dercum’s disease seems to be relatively constant over time.
Url:
DOI: 10.1186/1750-1172-7-23
PubMed: 22546240
PubMed Central: 3444313
Affiliations:
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University Hospital, SE-205 02, Malmö</wicri:regionArea><wicri:noRegion>Malmö</wicri:noRegion></affiliation></author><author><name sortKey="Svensson, Henry" sort="Svensson, Henry" uniqKey="Svensson H" first="Henry" last="Svensson">Henry Svensson</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö, Sweden</nlm:aff><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö</wicri:regionArea><wicri:noRegion>Malmö</wicri:noRegion></affiliation></author><author><name sortKey="Brorson, H Kan" sort="Brorson, H Kan" uniqKey="Brorson H" first="H Kan" last="Brorson">H Kan Brorson</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University 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wicri:level="1"><nlm:aff id="I2">Plastic and Reconstructive Surgery, Skåne University Hospital, SE-205 02, Malmö, Sweden</nlm:aff><country xml:lang="fr">Suède</country><wicri:regionArea>Plastic and Reconstructive Surgery, Skåne University Hospital, SE-205 02, Malmö</wicri:regionArea><wicri:noRegion>Malmö</wicri:noRegion></affiliation></author><author><name sortKey="Svensson, Henry" sort="Svensson, Henry" uniqKey="Svensson H" first="Henry" last="Svensson">Henry Svensson</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö, Sweden</nlm:aff><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö</wicri:regionArea><wicri:noRegion>Malmö</wicri:noRegion></affiliation></author><author><name sortKey="Brorson, H Kan" sort="Brorson, H Kan" uniqKey="Brorson H" first="H Kan" last="Brorson">H Kan Brorson</name><affiliation wicri:level="1"><nlm:aff id="I1">Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö, Sweden</nlm:aff><country xml:lang="fr">Suède</country><wicri:regionArea>Department of Clinical Sciences in Malmö, Lund University, Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö</wicri:regionArea><wicri:noRegion>Malmö</wicri:noRegion></affiliation></author></analytic><series><title level="j">Orphanet Journal of Rare Diseases</title><idno type="eISSN">1750-1172</idno><imprint><date when="2012">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec><title>Definition and clinical picture</title><p>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.</p></sec><sec><title>Classification</title><p>We suggest that Dercum’s disease is classified into: I. <italic>Generalised diffuse form</italic> A form with diffusely widespread painful adipose tissue without clear lipomas, II. <italic>Generalised nodular form</italic> - a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. <italic>Localised nodular form</italic> - a form with pain in and around multiple lipomas IV. <italic>Juxtaarticular form</italic> - a form with solitary deposits of excess fat for example at the medial aspect of the knee.</p></sec><sec><title>Epidemiology</title><p>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.</p></sec><sec><title>Aetiology</title><p>Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma.</p></sec><sec><title>Diagnosis and diagnostic methods</title><p>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.</p></sec><sec><title>Differential diagnosis</title><p>Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours.</p></sec><sec><title>Genetic counselling</title><p>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.</p></sec><sec><title>Management and treatment</title><p>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.</p></sec><sec><title>Prognosis</title><p>The pain in Dercum’s disease seems to be relatively constant over time.</p></sec></div></front><back><div1 type="bibliography"><listBibl><biblStruct></biblStruct><biblStruct></biblStruct><biblStruct><analytic><author><name sortKey="Dercum, Fx" uniqKey="Dercum F">FX Dercum</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Brorson, H" uniqKey="Brorson H">H Brorson</name></author><author><name sortKey="Fagher, B" uniqKey="Fagher B">B Fagher</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lange, U" uniqKey="Lange U">U Lange</name></author><author><name sortKey="Oelzner, P" uniqKey="Oelzner P">P Oelzner</name></author><author><name sortKey="Uhlemann, C" uniqKey="Uhlemann C">C 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Mease</name></author></analytic></biblStruct></listBibl></div1></back></TEI><affiliations><list><country><li>Suède</li></country></list><tree><country name="Suède"><noRegion><name sortKey="Hansson, Emma" sort="Hansson, Emma" uniqKey="Hansson E" first="Emma" last="Hansson">Emma Hansson</name></noRegion><name sortKey="Brorson, H Kan" sort="Brorson, H Kan" uniqKey="Brorson H" first="H Kan" last="Brorson">H Kan Brorson</name><name sortKey="Hansson, Emma" sort="Hansson, Emma" uniqKey="Hansson E" first="Emma" last="Hansson">Emma Hansson</name><name sortKey="Svensson, Henry" sort="Svensson, Henry" uniqKey="Svensson H" first="Henry" last="Svensson">Henry Svensson</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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