Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management

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.

Trauma-induced adiposis dolorosa (Dercum’s disease)

“…The aetiology of adiposis dolorosa is unknown, though multiple theories have been purported, including inflammation, adipose tissue dysfunction and trauma 1. On histopathology, the fat deposition in adiposis dolorosa is notable for mature adult fatty tissue and sometimes, a number of blood vessels suggesting angiolipoma.…”

“…Adiposis dolorosa (Dercum’s disease) is a rare, sporadic disease characterised by generalised obesity with pronounced pain in the adipose tissue 1. Neurologist Francis Dercum first described the condition in 1888,2 characterising it as painful adipose deposits associated with obesity and psychiatric disturbances.…”

“…Neurologist Francis Dercum first described the condition in 1888,2 characterising it as painful adipose deposits associated with obesity and psychiatric disturbances. Adiposis dolorosa should be diagnosed through physical examination and a comprehensive exclusion of alternative diagnoses, especially as varied unusual signs have been described 1. A high index of suspicion is needed for appropriate diagnosis as the symptomatology overlaps with numerous common and uncommon diseases.…”

The Many Biological Functions of the Alternative Cellular Energy (ACE) Pathway

“…Thus, there is an intrinsic capacity of the immune system to cause collateral damage to the body and to aid in the dissemination of certain pathogens. Except for Morgellon's disease and, as will be discussed later, Dercum's and related diseases [38], no self-harm has been attributed to the ACE pathway.…”

“…This is a relatively rare illness characterized by the formation of multiple painful lipomas [38]. While genetic factors can play a role, several patients with this illness have had strikingly positive cultures for stealth adapted viruses.…”