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Cureus Sep 2021Adiposis dolorosa, also known as Dercum's disease, is a rare disorder characterized by debilitating painful lipomas throughout the body. The prevalence and etiology...
Adiposis dolorosa, also known as Dercum's disease, is a rare disorder characterized by debilitating painful lipomas throughout the body. The prevalence and etiology of Dercum's disease are unknown as mentioned in the National Organization of Rare Disorders. We present an interesting case of Dercum's disease in a 53-year-old female who initially presented with a six-week history of painful subcutaneous masses. Ultrasound findings were suggestive of lipomas, however, her symptoms were debilitating beyond that of benign lipomas. She then represented with a rapidly increasing number of soft tissue masses manifesting throughout her body, as well as significant diffuse pain concentrating around these lesions within a short period of time following her initial presentation. The patient underwent surgical excision of a select number of these masses, with histopathology consistent with lipomas. Most cases of Dercum's disease are sporadic, and no guidelines exist regarding the treatment of the disease. Due to the rarity of this condition, in conjunction with simple lipomas typically presenting as painless masses, many patients may be misdiagnosed and neglected due to being falsely labeled as pain seeking or having their symptoms attributed to psychological disorders. Management, therefore, is complex and currently consists of a multidisciplinary approach employing multimodal treatments, including pain control, surgical excision, and psychotherapy. Although this condition has been described in the literature for over 100 years, there have been minimal advancements towards alleviating the suffering of these patients. We aimed to unearth and bring to light the reality and the suffering experienced by patients with Decrum's disease.
PubMed: 34646696
DOI: 10.7759/cureus.17649 -
JAAD Case Reports Sep 2021
PubMed: 34409145
DOI: 10.1016/j.jdcr.2021.07.009 -
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 -
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 -
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 -
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 -
Reumatologia 2019Dercum's disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful subcutaneous adipose tissue deposits with various localization over... (Review)
Review
Dercum's disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful subcutaneous adipose tissue deposits with various localization over the body. The deposits occur histologically as lipomas and are associated with overweight or obesity and a variety of psychiatric disturbances (anxiety, depression, sleep disturbances). Classification of Dercum's disease is related to size and location of adipose nodules (generalized diffuse, generalized nodular, localized nodular and juxta-articular forms). Diagnosis in based on clinical presentation and exclusion of a number of other disorders associated with lipomas. There is no generally accepted management of the patients. Liposuction or lidocaine application has been reported successful in some cases. Other therapeutic methods have been reported but their effectiveness is based on anecdotal descriptions only, and were not confirmed in clinical trials.
PubMed: 31844341
DOI: 10.5114/reum.2019.89521 -
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 -
Ugeskrift For Laeger May 2019Dercum's disease is a rare disease, which mainly affects women and has an unknown prevalence and aetiology. The disease is characterised by generalised obesity and more...
Dercum's disease is a rare disease, which mainly affects women and has an unknown prevalence and aetiology. The disease is characterised by generalised obesity and more than three-month painful subcutaneous adipose tissue not responding to usual pain treatment. A suggested classi-fication of the disease includes four types: generalised diffuse, generalised nodular, localised nodular and juxta-articular. Diagnosis is one of exclusion, and treatment includes medical and surgical options with the aim of pain palliation and increased mobility and function.
Topics: Adiposis Dolorosa; Female; Humans; Obesity; Pain; Pain Management; Rare Diseases
PubMed: 31140409
DOI: No ID Found -
Anais Brasileiros de Dermatologia 2019
Topics: Adiposis Dolorosa; Aged; Female; Humans; Lipectomy; Lipoma; Lower Extremity
PubMed: 31090842
DOI: 10.1590/abd1806-4841.20198231