Dercum's disease (adiposis dolorosa)

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.

show abstract

“…[Reece et al 36] noted a patient who developed painful fatty swellings around the neck. Later, a tender lump was excised from the back.…”

Section: Definition and Clinical Descriptionmentioning

confidence: 99%

“…The resulting pain relief lasted from 10 hours [21] to 12 months [92]. In five of the cases, the lidocaine treatment was combined with mexiletine (Mexitil®), which is a class 1B anti-arrhythmic with similar pharmacological properties as lidocaine [27,36,42,102]. …”

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

View full text Add to dashboard Cite

show abstract

“…She had no dysmorphic features or apparent developmental delays, which made diseases such as Proteus syndrome or myoclonus, epilepsy, and ragged-red fiber (or MERRF) syn- drome unlikely. Dercum disease (also known as adiposis dolorosa) was also unlikely, given the fact that this condition usually involves diffuse or localized painful fat deposits throughout the body, although the head and neck are spared, which was not the case in this patient (12). There was no family history of similar problems, which made familial lipomatosis unlikely.…”

Section: Discussionmentioning

confidence: 88%

Case 143: Madelung Disease

Landis¹,

Etemad-Rezai²,

Shetty³

et al. 2009

Radiology

View full text Add to dashboard Cite

“…Therefore, the present Japanese female case is considered to be extremely rare (figure 1). Two clinical phenotypes are recognized in BSL [4]. Type 1 BSL affects primarily men and is characterized by fat tissue accumulation around the neck, nape of the neck, upper back, shoulders, and upper arms.…”

mentioning

confidence: 99%