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Orphanet Journal of Rare Diseases Jun 2021Dercum's disease (DD) and Roch-Leri mesosomatic lipomatosis (LMS) are rare and poorly characterized diseases. The clinical presentation combines multiple lipomas,...
CONTEXT
Dercum's disease (DD) and Roch-Leri mesosomatic lipomatosis (LMS) are rare and poorly characterized diseases. The clinical presentation combines multiple lipomas, painful in DD in contrast with LMS, without lipoatrophy.
OBJECTIVE
To identify any specific metabolic and immune phenotype of DD and LMS.
DESIGN AND PATIENTS
This monocentric retrospective study included 46 patients: 9 DD, 11 LMS, 18 lean and 8 obese controls. Metabolic and immunohematological characteristics of each group were compared.
RESULTS
The median age of the patients was similar in the 3 groups (31 years). The number of women, and of basophils, and CD3, CD4 and CD8 T lymphocytes was significantly higher in the DD versus the LMS group, without any difference of the metabolic parameters. Weight, BMI, blood pressure, gamma-GT, leptin, fasting insulin and C-peptide levels, fat mass percentage, and intra/total abdominal fat ratio were significantly higher in each lipomatosis group compared with the lean group. Compared with the lean group, the DD group had significantly higher fasting blood glucose, LDL-cholesterol, platelets, leukocytes, basophils, and a lower NK cell count, whereas the LMS group had a significantly lower rate of CD3, CD4, and CD8 lymphocytes. Compared with the obese controls, basophils remained higher in DD and T lymphocytes subpopulations lower in LMS groups.
CONCLUSION
DD and LMS show a common background of obesity and metabolic phenotype, but a distinct immunohematological profile characterized by a higher number of basophils in DD patients, an inflammatory profile that could contribute to pain. T lymphocyte depletion was present in LMS. These findings could offer specific therapeutic opportunities, especially for painful DD.
Topics: Adiposis Dolorosa; Adult; Female; Humans; Lipomatosis; Obesity; Phenotype; Retrospective Studies
PubMed: 34187516
DOI: 10.1186/s13023-021-01920-3 -
Dermatologic Surgery : Official... Aug 2021
Topics: Adiposis Dolorosa; Carbazoles; Drugs, Investigational; Female; Humans; Injections, Subcutaneous; Male; Middle Aged; Pain; Pain Measurement; Subcutaneous Fat; Treatment Outcome
PubMed: 33867475
DOI: 10.1097/DSS.0000000000002989 -
Case Reports in Dermatological Medicine 2020Dercum's disease, or adiposis dolorosa, is a rare disorder which consists of multiple, painful lipomas within the subcutaneous tissue and has a distribution mainly in...
Dercum's disease, or adiposis dolorosa, is a rare disorder which consists of multiple, painful lipomas within the subcutaneous tissue and has a distribution mainly in the abdomen and extremities. Dercum's disease can be defined as in combination with chronic painful adipose tissue. Although the etiology of Dercum's disease is not clear, it is thought to be a combination of a neurological and endocrine disorder. Treatment for this disease is centered at managing pain. Although there is no standard of care for managing pain, there are different pain management regimes that are promising.
PubMed: 33224536
DOI: 10.1155/2020/6129706 -
Dermatologic Therapy Nov 2020
Topics: Adiposis Dolorosa; Humans; Pain; Surgical Wound
PubMed: 32495450
DOI: 10.1111/dth.13751 -
Cureus Mar 2020Adiposis dolorosa or Dercum's disease is a rare lipomatous disorder characterized by painful lipomas. In this article, we report a case of rather large exophytic...
Adiposis dolorosa or Dercum's disease is a rare lipomatous disorder characterized by painful lipomas. In this article, we report a case of rather large exophytic adiposis dolorosa causing difficulties with ambulation, and our surgical management of the disorder. To our knowledge, this is the first reported case of a large exophytic adiposis dolorosa of the upper medial thigh causing problems with mobility. This is also the first reported case of the use of a delayed split-thickness skin graft (STSG) after interval use of wound vacuum-assisted closure (VAC) following dermolipectomy. A 77-year-old female presented with a chronic mass on the medial aspect of her right thigh for over 40-50 years. She had noticed a recent rapid increase in size, causing some discomfort and interference with mobility and activities of daily living. The patient underwent an MRI with finding consistent with adiposis dolorosa. She underwent dermolipectomy and reconstruction of the resulting defect with a combination of partial primary closure, wound VAC, and delayed closure using STSG. Dermolipectomy with interval application of a wound VAC combined with delayed reconstruction with STSG is a feasible option for patients with large lesions of the extremity that causes difficulty with mobility and activities of daily living.
PubMed: 32300502
DOI: 10.7759/cureus.7282 -
Skeletal Radiology May 2020We report a case of a 70-year-old woman with a BMI of 58 who developed cellulitis refractory to treatment, within an area of massive localized lymphedema. Biopsy showed...
We report a case of a 70-year-old woman with a BMI of 58 who developed cellulitis refractory to treatment, within an area of massive localized lymphedema. Biopsy showed angiosarcoma. MRI showed multiple lobulated, low T1, high T2 masses within a background of prominent soft tissue septal stranding, dilated lymphatic channels, and skin thickening. CT also showed the mass well, within the background lymphedema. Massive localized lymphedema is increasing in prevalence due to the worsening obesity epidemic. Radiologists should be aware that the presence of a nodule within an area of massive localized lymphedema is suspicious for sarcoma.
Topics: Aged; Biopsy; Diagnosis, Differential; Female; Hemangiosarcoma; Humans; Lymphedema; Magnetic Resonance Imaging; Soft Tissue Neoplasms; Thigh; Tomography, X-Ray Computed
PubMed: 31950200
DOI: 10.1007/s00256-020-03373-4 -
Federal Practitioner : For the Health... Nov 2019While current approaches to treatment focus on surgery, opiates, and other medications, health care providers may also consider ketamine infusion, electrostimulation,...
While current approaches to treatment focus on surgery, opiates, and other medications, health care providers may also consider ketamine infusion, electrostimulation, and perineural injections.
PubMed: 31892777
DOI: No ID Found -
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 -
Dermatologic Surgery : Official... Dec 2019
Topics: Adiposis Dolorosa; Deoxycholic Acid; Humans; Injections, Intralesional; Male; Middle Aged; Off-Label Use; Treatment Outcome
PubMed: 30789509
DOI: 10.1097/DSS.0000000000001800