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Obesity (Silver Spring, Md.) Oct 2014Investigational, near-infrared fluorescence (NIRF) lymphatic imaging was used to assess lymphatic architecture and contractile function in participants diagnosed with... (Clinical Trial)
Clinical Trial
OBJECTIVE
Investigational, near-infrared fluorescence (NIRF) lymphatic imaging was used to assess lymphatic architecture and contractile function in participants diagnosed with Dercum's disease, a rare, poorly understood disorder characterized by painful lipomas in subcutaneous adipose tissues.
METHODS
After informed consent and as part of an FDA-approved feasibility study to evaluate lymphatics in diseases in which their contribution has been implicated, three women diagnosed with Dercum's disease and four control subjects were imaged. Each participant received multiple intradermal and subcutaneous injections of indocyanine green (ICG, total dose ≤400 µg) in arms, legs, and/or trunk. Immediately after injection, ICG was taken up by the lymphatics and NIRF imaging was conducted.
RESULTS
The lymphatics in the participants with Dercum's disease were intact and dilated, yet sluggishly propelled lymph when compared to control lymphatics. Palpation of regions containing fluorescent lymphatic pathways revealed tender, fibrotic, tubular structures within the subcutaneous adipose tissue that were associated with painful nodules, and, in some cases, masses of fluorescent tissue indicating that some lipomas may represent tertiary lymphoid tissues.
CONCLUSIONS
These data support the hypothesis that Dercum's disease may be a lymphovascular disorder and suggest a possible association between abnormal adipose tissue deposition and abnormal lymphatic structure and function.
Topics: Adiposis Dolorosa; Female; Humans; Indocyanine Green; Infrared Rays; Lymphatic Diseases; Lymphatic System; Middle Aged; Optical Imaging; Pain; Phenotype; Subcutaneous Fat
PubMed: 25044620
DOI: 10.1002/oby.20836 -
Surgical Neurology International 2023Dercum's Disease (DD) is a rare chronic pain syndrome in which patients experience extreme burning pain associated with subcutaneous lipomatous tissue deposits. These...
BACKGROUND
Dercum's Disease (DD) is a rare chronic pain syndrome in which patients experience extreme burning pain associated with subcutaneous lipomatous tissue deposits. These patients may also present with; weakness, psychiatric symptoms, metabolic derangements, sleep disturbance, impaired memory, and easy bruising. Common risk factors for DD include: obesity, Caucasian race, and female sex. The etiology of DD remains under debate while it has proven highly resistant to treatment (i.e., requiring high doses of opioids for adequate pain management).
CASE DESCRIPTION
A 48-year-old female with DD and a prior spinal cord stimulator (SCS) placed for chronic back pain, presented with recurrent back pain, and increased falling. Surgery to replace her SCS resulted in improvement in her back pain and a decreased incidence of falls. Furthermore, she noticed significant improvement in the burning pain attributed to her subcutaneous nodules; this most markedly occurred at and below the level of stimulator placement.
CONCLUSION
A 48-year-old female with the extremely rare condition, DD experienced dramatic reduction in her pain following the successful revision of her SCS.
PubMed: 37025543
DOI: 10.25259/SNI_1148_2022 -
The Journal of Headache and Pain Dec 2010A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back....
A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back. Six weeks prior to the presentation to our service, she developed severe occipital headache refractory to drug treatment. She improved after bilateral greater occipital nerve blocks. She was subjected to bilateral greater occipital chemical neurolysis which has given her complete pain relief.
Topics: Adiposis Dolorosa; Diagnosis, Differential; Female; Headache Disorders; Humans; Middle Aged; Neuralgia; Scalp; Spinal Nerves
PubMed: 20811764
DOI: 10.1007/s10194-010-0253-9 -
American Journal of Human Genetics Jun 1963
PubMed: 17948485
DOI: No ID Found -
Proceedings of the Royal Society of... 1910
PubMed: 19974159
DOI: No ID Found -
Cureus Aug 2022Here, we report a case of a 70-year-old female who presented with a slowly enlarging tender nodule on the right forearm for several months. Physical examination showed a...
Here, we report a case of a 70-year-old female who presented with a slowly enlarging tender nodule on the right forearm for several months. Physical examination showed a faintly blue-tinged freely mobile subcutaneous nodule. Excision was complicated by greater than expected bleeding and revealed an unexpected intravenous mass. Histopathology demonstrated capillary lobules separated by fibrous septae within a vein, consistent with intravenous lobular capillary hemangioma (IVLCH). IVLCH is a rare benign capillary proliferation of unclear etiology. Excision is typically curative and relieves any pain and discomfort the patient might be experiencing. With the addition of IVLCH, we respectfully propose a new acronym for the differential diagnosis of cutaneous tender tumors: intravenous lobular capillary hemangioma, foreign body (reaction), hidradenoma, osteoma cutis, glomus tumor, scar, fibromyxoma, leiomyosarcoma, eccrine angiomatous hamartoma, Dercum's disease (adiposis dolorosa), piezogenic pedal papule, eccrine spiradenoma, neurilemmoma (schwannoma), calcinosis cutis, angioendotheliomatosis, leiomyoma, metastases, angiolipoma, neuroma, dermatofibroma, granular cell tumor, endometriosis, thrombus, blue rubber bleb nevus, angioma, chondrodermatitis nodularis helicis, and keloid ("IF HOGS FLED PEN, CALM AND GET BACK"). Future additions to the cutaneous tender tumor differential diagnosis may require creative additions and rearrangements to this acronym. However, continual updates will allow it to serve both clinicians and pathologists alike as a comprehensive representation of etiologies to consider for cutaneous tender tumors.
PubMed: 36120217
DOI: 10.7759/cureus.28030 -
Proceedings of the Royal Society of... 1924
PubMed: 19983505
DOI: No ID Found -
Annals of the Rheumatic Diseases Oct 1979Juxta-articular adiposis dolorosa may be defined as the localised accumulation of painful fat near joints, most commonly the knee. It exists as a separate entity that...
Juxta-articular adiposis dolorosa may be defined as the localised accumulation of painful fat near joints, most commonly the knee. It exists as a separate entity that must be distinguished from other causes of knee pain. We present 2 patients with this condition and review the literature. Various approaches to therapy are discussed. A classification of painful fat near joints is proposed.
Topics: Adiposis Dolorosa; Female; Humans; Joint Diseases; Knee Joint; Middle Aged
PubMed: 518149
DOI: 10.1136/ard.38.5.479 -
JAAD Case Reports Sep 2021
PubMed: 34409145
DOI: 10.1016/j.jdcr.2021.07.009 -
Journal of Internal Medicine Mar 1998To study the impact of adipose tissue removal by liposuction on factors associated with increased risk of cardiovascular atherosclerotic disease within the coagulation...
OBJECTIVE
To study the impact of adipose tissue removal by liposuction on factors associated with increased risk of cardiovascular atherosclerotic disease within the coagulation and fibrinolytic system and glucose metabolism.
DESIGN, SETTING AND SUBJECTS
Liposuction was performed in 53 patients with Dercum's disease. The levels of fibrinogen, von Willebrand factor antigen (VWF:Ag) and plasminogen activator inhibitor type 1 activity (PAI-1) were measured preoperatively, and 2 weeks, 4 weeks and 3 months postoperatively. In a subsample of 10 patients, insulin sensitivity was determined before and 2-4 weeks after surgery using the 2-h euglycaemic hyperinsulinaemic clamp technique. The study was performed as a single-centre study.
MAIN OUTCOME MEASURE
Fibrinogen, PAI-1 and VWF:Ag levels, and glucose uptake before and after removal of adipose tissue.
RESULTS
Weight reduction was sustained throughout the follow-up period with a mean decrease from 90.7 to 86.6 kg (P < 0.0001). There was a slight increase in levels of coagulation factors 2 and 4 weeks postoperatively, probably in reaction to the surgical trauma. After 3 months the values had returned to preoperative levels except for PAI-1, which still showed a slight increase (P < 0.05). In the subsample of 10 patients, glucose uptake was improved (P < 0.05) from a short-term perspective after surgery.
CONCLUSION
Surgical removal of adipose tissue, without change in lifestyle, does not seem to improve the levels of coagulation and fibrinolytic factors associated with cardiovascular atherosclerotic disease, whereas glucose takeup may be facilitated and insulin sensitivity increases from a short-term perspective.
Topics: Adiposis Dolorosa; Adult; Aged; Arteriosclerosis; Female; Fibrinolysis; Glucose; Hemostasis; Humans; Insulin; Lipectomy; Male; Middle Aged; Risk Factors
PubMed: 9627156
DOI: 10.1046/j.1365-2796.1998.00264.x