PMID- 22546240
OWN - NLM
STAT- MEDLINE
DCOM- 20130214
LR  - 20231105
IS  - 1750-1172 (Electronic)
IS  - 1750-1172 (Linking)
VI  - 7
DP  - 2012 Apr 30
TI  - Review of Dercum's disease and proposal of diagnostic criteria, diagnostic 
      methods, classification and management.
PG  - 23
LID - 10.1186/1750-1172-7-23 [doi]
AB  - 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 alpha-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.
FAU - Hansson, Emma
AU  - Hansson E
AD  - Department of Clinical Sciences in Malmo, Lund University, Plastic and 
      Reconstructive Surgery, Skane University Hospital, Malmo, Sweden. 
      emma.hansson@med.lu.se
FAU - Svensson, Henry
AU  - Svensson H
FAU - Brorson, Hakan
AU  - Brorson H
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20120430
PL  - England
TA  - Orphanet J Rare Dis
JT  - Orphanet journal of rare diseases
JID - 101266602
SB  - IM
MH  - Adipose Tissue/pathology
MH  - *Adiposis Dolorosa/classification/diagnosis/physiopathology/therapy
MH  - Chronic Disease
MH  - Female
MH  - Humans
MH  - Male
MH  - Obesity
MH  - Pain/diagnosis/physiopathology
MH  - Rare Diseases
MH  - Severity of Illness Index
PMC - PMC3444313
EDAT- 2012/05/02 06:00
MHDA- 2013/02/15 06:00
PMCR- 2012/04/30
CRDT- 2012/05/02 06:00
PHST- 2011/09/30 00:00 [received]
PHST- 2012/04/06 00:00 [accepted]
PHST- 2012/05/02 06:00 [entrez]
PHST- 2012/05/02 06:00 [pubmed]
PHST- 2013/02/15 06:00 [medline]
PHST- 2012/04/30 00:00 [pmc-release]
AID - 1750-1172-7-23 [pii]
AID - 10.1186/1750-1172-7-23 [doi]
PST - epublish
SO  - Orphanet J Rare Dis. 2012 Apr 30;7:23. doi: 10.1186/1750-1172-7-23.