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Annals of Clinical and Translational... Mar 2014Francis Xavier Dercum (1856-1931) is known primarily for his prominence in the field of neurology and for his identification of "Adiposis Dolorosa", known as Dercum's...
Francis Xavier Dercum (1856-1931) is known primarily for his prominence in the field of neurology and for his identification of "Adiposis Dolorosa", known as Dercum's disease. His brilliance, however, extends well beyond neurology. Born and raised in Philadelphia to parents of American and European descent, Dercum's natural curiosity oriented his interests toward medicine and philosophy. His scholarship flourished, and much recognition came his way. He died in Benjamin Franklin's Chair, closing what would be his last session as President of the American Philosophical Society. From anatomy, histology, pathology, and neurology to teaching, writing, and philosophy, Dercum's life was that of a man for all seasons.
PubMed: 25356401
DOI: 10.1002/acn3.36 -
Lymphatic Research and Biology Dec 2019Lipedema and Dercum's disease (DD) are incompletely characterized adipose tissue diseases, and objective measures of disease profiles are needed to aid in differential...
Lipedema and Dercum's disease (DD) are incompletely characterized adipose tissue diseases, and objective measures of disease profiles are needed to aid in differential diagnosis. We hypothesized that fluid properties, quantified as tissue water bioimpedance in the upper and lower extremities, differ regionally between these conditions. Women (cumulative = 156) with lipedema ( = 110), DD ( = 25), or without an adipose disease matched for age and body mass index to early stage lipedema patients (i.e., controls = 21) were enrolled. Bioimpedance spectroscopy (BIS) was applied to measure impedance values in the arms and legs, indicative of extracellular water levels. Impedance values were recorded for each limb, as well as the leg-to-arm impedance ratio. Regression models were applied to evaluate hypothesized relationships between impedance and clinical indicators of disease (significance criteria: two-sided < 0.05). Higher extracellular water was indicated (i) in the legs of patients with higher compared with lower stages of lipedema ( = 0.03), (ii) in the leg-to-arm impedance ratio in patients with lipedema compared with patients with DD ( ≤ 0.001), and (iii) in the leg-to-arm impedance ratio in patients with stage 1 lipedema compared with controls ( ≤ 0.01). BIS is a noninvasive portable modality to assess tissue water, and this device is available in both specialized and nonspecialized centers. These findings support that regional bioimpedance measures may help to distinguish lipedema from DD, as well as to identify early stages of lipedema.
Topics: Adiposis Dolorosa; Adult; Aged; Diagnosis, Differential; Electric Impedance; Female; Humans; Lipedema; Middle Aged; Severity of Illness Index; Symptom Assessment; Young Adult
PubMed: 31408408
DOI: 10.1089/lrb.2019.0011 -
The Yale Journal of Biology and Medicine Dec 2021Dercum's disease (DD), also described as adiposis dolorosa, is a poorly understood and rare adipose tissue disorder involving obesity and painful adipose tissue masses.... (Review)
Review
Dercum's disease (DD), also described as adiposis dolorosa, is a poorly understood and rare adipose tissue disorder involving obesity and painful adipose tissue masses. Patients may have associated bruising and constitutional symptoms such as fatigue, difficulty concentrating, and sleep disturbance. DD was initially described in 1888 by Francis Xavier Dercum, and was classified into four subtypes, including generalized diffuse, generalized nodular, localized nodular, and juxta-articular subtypes. While this disease has been described for more than 130 years, its etiology and treatment remain elusive. We describe a case of a patient with DD who presented to Ochsner Medical Center, New Orleans, LA, for evaluation of treatment options. We review current knowledge on this rare disease and data on modern treatment methods.
Topics: Adipose Tissue; Adiposis Dolorosa; Diagnosis, Differential; Humans; Obesity; Pain
PubMed: 34970098
DOI: No ID Found -
Journal of Neurology Apr 2017
Topics: Adiposis Dolorosa; Brain; Family Health; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Nervous System Diseases
PubMed: 28194532
DOI: 10.1007/s00415-017-8415-1 -
Giornale Italiano Di Dermatologia E... Dec 2017
Topics: Adiposis Dolorosa; Adult; Diagnosis, Differential; Familial Multiple Lipomatosis; Female; Humans; Lipomatosis, Multiple Symmetrical; Male; Middle Aged; Skin Diseases
PubMed: 29050453
DOI: 10.23736/S0392-0488.16.05410-9 -
Orphanet Journal of Rare Diseases Apr 2012DEFINITION AND CLINICAL PICTURE: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose... (Review)
Review
UNLABELLED
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.
Topics: Adipose Tissue; Adiposis Dolorosa; Chronic Disease; Female; Humans; Male; Obesity; Pain; Rare Diseases; Severity of Illness Index
PubMed: 22546240
DOI: 10.1186/1750-1172-7-23 -
Medicine Dec 2021Dercum's disease (DD), or adiposis dolorosa, is a rare condition of unknown etiology characterized by growth of painful subcutaneous adipose tissue. No specific...
Dercum's disease (DD), or adiposis dolorosa, is a rare condition of unknown etiology characterized by growth of painful subcutaneous adipose tissue. No specific treatment exists. Pain is often invalidating and resistant to analgesic drugs. We tested the efficacy of Frequency Rhythmic Electrical Modulation System (FREMS) therapy on pain relief. Subcutaneous biopsies were performed for genetic analysis.Nine DD patients were enrolled. Five cycles of FREMS at 3-month intervals during 1 year were administered. Visual analogue scale (VAS), Bartel Index Questionnaire and Short Form 36 questionnaire were used to measure pain and general health status at baseline, 6 and 12 months. Dual-energy X-ray absorptiometry (DEXA) quantified fat mass. Next-Generation Sequencing (NGS) was performed on adipose tissue biopsies and peripheral blood sample to search for somatic variants and specific protein pathway mutation.Seven patients were included in the final analysis. FREMS induced a reduction in VAS score (from 92 to 52.5, P = .0597) and a significant improvement in SF-36 domains (Physical functioning, Role limitation due to physical health, Body pain, Vitality, Social functioning, P < .05). No modification in anthropometrics and DEXA values was observed. The analysis of the mitochondrial Displacement loop (D-loop) region confirmed the clonality of all lipomatous lesions. The presence of the mitochondrially encoded tRNA-Lysine (MT-TK) m.8344A>G variant, occasionally identified in patients with multiple symmetric lipomatosis, was excluded in all subjects. On the other hand, we observed variants in genes belonging to signaling pathways involved in cell cycle and proliferation (Phosphoinositide 3-kinase/AKT/mTOR, MAPK/ERK, and Hippo).FREMS can be a useful tool to alleviate pain and improve overall quality of life in patients with DD. Genetic analysis highlighted the molecular heterogeneity of lipomas.
Topics: Adiposis Dolorosa; Adult; Female; High-Throughput Nucleotide Sequencing; Humans; Lipoma; Male; Middle Aged; Pain; Pain Management; Pilot Projects; Quality of Life; Transcutaneous Electric Nerve Stimulation
PubMed: 34941153
DOI: 10.1097/MD.0000000000028360 -
American Practitioner and Digest of... Apr 1951
Topics: Adiposis Dolorosa; Humans
PubMed: 14819532
DOI: No ID Found -
Nordisk Medicin Mar 1958
Topics: Adiposis Dolorosa; Humans
PubMed: 13517644
DOI: No ID Found -
Cureus Nov 2023Dercum's disease is a rare and poorly understood condition characterized by painful subcutaneous adipose tissue growth that can occur anywhere beneath the skin surface....
Dercum's disease is a rare and poorly understood condition characterized by painful subcutaneous adipose tissue growth that can occur anywhere beneath the skin surface. We present the case of a 27-year-old man with no significant medical history who had been experiencing painful subcutaneous nodules for two years. Skin biopsy revealed the proliferation of mature adipocytes that were surrounded by fibrous septa. There are currently no treatments approved by the US Food and Drug Administration for Dercum's disease, and the effectiveness of treatments that have been attempted is variable.
PubMed: 38084160
DOI: 10.7759/cureus.48615