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1.
BMJ Case Rep ; 16(8)2023 Aug 10.
Artículo en Inglés | MEDLINE | ID: mdl-37562864

RESUMEN

A woman in her 50s presented to her general practitioner (GP) with an 8-month history of multiple painful subcutaneous nodules of various sizes in her trunk and limbs. Her previous GP made the clinical diagnosis of lipoma when they presented as non-painful nodules 2 years ago. Initial ultrasound demonstrated multiple lipomata with the largest size in the left upper thighs displaying features of subtle internal vascularity, entirely contained within the subcutaneous layer. MRI scan of thigh lesions showed multiple angiolipomas consistent with the entity of Dercum's disease. Subsequent biopsy histology confirmed the image diagnosis of angiolipoma. She was referred to a musculoskeletal oncological surgeon for evaluation and reassurance regarding optimising medical management of her associated obesity. Her angiolipoma and obesity are well managed by her GP. This case highlights the diagnostic workup of a rare variant of lipoma, Dercum's disease. The differential diagnosis of multiple painful lipomas was reviewed and discussed, consequently leading to the discussion of managing the associated obesity in this case.


Asunto(s)
Adiposis Dolorosa , Angiolipoma , Lipoma , Neoplasias Cutáneas , Femenino , Humanos , Adiposis Dolorosa/diagnóstico , Lipoma/diagnóstico , Lipoma/diagnóstico por imagen , Obesidad , Dolor/etiología , Neoplasias Cutáneas/diagnóstico
2.
Clin Nucl Med ; 48(5): 451-452, 2023 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-36754360

RESUMEN

ABSTRACT: A 64-year-old man with recently diagnosed prostate adenocarcinoma and Gleason score 4 + 3 = 7 with a mildly elevated prostate-specific antigen of 5.17 µg/L was referred for 18 F-DCFPyL PET/CT for primary staging. The scan demonstrated incidental innumerable moderately avid subcutaneous nodules due to adiposis dolorosa (Dercum's disease), which is a rare adipose tissue disease.


Asunto(s)
Adiposis Dolorosa , Masculino , Humanos , Persona de Mediana Edad , Adiposis Dolorosa/diagnóstico , Tomografía Computarizada por Tomografía de Emisión de Positrones
3.
Yale J Biol Med ; 94(4): 603-608, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-34970098

RESUMEN

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.


Asunto(s)
Adiposis Dolorosa , Tejido Adiposo , Adiposis Dolorosa/diagnóstico , Diagnóstico Diferencial , Humanos , Obesidad , Dolor
4.
Georgian Med News ; (316-317): 7-9, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34511435

RESUMEN

Both lipedema and juxta-articular adiposis dolorsa are painful disorders of subcutaneous adipose tissue. We investigated 297 female patients with lipedema treated at our department for the presence of juxta-articular adiposis dolorsa. Occurrence of both disorders was identified in 4.4% of lipedema patients. The common presence of both disorders was observed only in more advanced lipedema (grade II and III). Juxta-articular adiposis dolorosa of the knees is seen exclusively on the inner knees, and it presents neither bruising nor creases or hypothermia. Choices of surgical treatment are either microcannula liposuction or dermolipectomy. Recurrences have not been observed.


Asunto(s)
Adiposis Dolorosa , Lipedema , Adiposis Dolorosa/complicaciones , Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/epidemiología , Femenino , Humanos , Lipedema/diagnóstico , Lipedema/epidemiología , Lipedema/cirugía
6.
Lymphat Res Biol ; 17(6): 671-679, 2019 12.
Artículo en Inglés | MEDLINE | ID: mdl-31408408

RESUMEN

Background: 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. Methods and Results: Women (cumulative n = 156) with lipedema (n = 110), DD (n = 25), or without an adipose disease matched for age and body mass index to early stage lipedema patients (i.e., controls n = 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 p < 0.05). Higher extracellular water was indicated (i) in the legs of patients with higher compared with lower stages of lipedema (p = 0.03), (ii) in the leg-to-arm impedance ratio in patients with lipedema compared with patients with DD (p ≤ 0.001), and (iii) in the leg-to-arm impedance ratio in patients with stage 1 lipedema compared with controls (p ≤ 0.01). Conclusion: 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.


Asunto(s)
Adiposis Dolorosa/diagnóstico , Impedancia Eléctrica , Lipedema/diagnóstico , Adiposis Dolorosa/etiología , Adulto , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Lipedema/etiología , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Evaluación de Síntomas , Adulto Joven
8.
Ugeskr Laeger ; 181(22)2019 May 27.
Artículo en Danés | MEDLINE | ID: mdl-31140409

RESUMEN

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.


Asunto(s)
Adiposis Dolorosa , Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/terapia , Femenino , Humanos , Obesidad , Dolor , Manejo del Dolor , Enfermedades Raras
10.
Br J Dermatol ; 180(5): 993-1000, 2019 05.
Artículo en Inglés | MEDLINE | ID: mdl-30422315

RESUMEN

BACKGROUND: Presentations of suspected lower-limb cellulitis are commonly misdiagnoses, resulting in avoidable antibiotic prescribing or hospital admissions. Understanding the challenges posed in diagnosing cellulitis may help enhance future care. OBJECTIVES: To examine and map out the challenges and facilitators identified by patients and health professionals in diagnosing lower-limb cellulitis. METHODS: A scoping systematic review was performed in MEDLINE and Embase in October 2017. Thematic analysis was used to identify key themes. Quantitative data were summarized by narrative synthesis. RESULTS: Three themes were explored: (i) clinical case reports of misdiagnosis, (ii) service development and (iii) diagnostic aids. Forty-seven different pathologies were misdiagnosed, including seven malignancies. Two different services have been piloted to reduce the misdiagnosis rates of lower-limb cellulitis and save costs. Four studies have looked at biochemical markers, imaging and a scoring tool to aid diagnosis. CONCLUSIONS: This review highlights the range of alternative pathologies that can be misdiagnosed as cellulitis, and emerging services and diagnostic aids developed to minimize misdiagnosis. Future work should focus on gaining a greater qualitative understanding of the diagnostic challenges from the perspective of patients and clinicians.


Asunto(s)
Celulitis (Flemón)/diagnóstico , Errores Diagnósticos/prevención & control , Adiposis Dolorosa/diagnóstico , Celulitis (Flemón)/sangre , Celulitis (Flemón)/patología , Pie Diabético/diagnóstico , Diagnóstico Diferencial , Gota/diagnóstico , Humanos , Pierna , Piel/patología
11.
J Dtsch Dermatol Ges ; 16(3): 313-327, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29431910

RESUMEN

Lipomatoses are benign proliferations of adipose tissue, often with typical distribution patterns, which usually occur without clear causes. In contrast to circumscribed lipomas, they develop diffusely and symmetrically and are not surrounded by a fibrous pseudocapsule. The most common form is benign symmetric lipomatosis (BSL; Launois-Bensaude syndrome), of which four types are distinguished based on the distribution pattern of hyperplastic adipose tissue. The etiology and pathogenesis of the disease are still largely unknown, although some forms appear to have a hereditary basis or to be associated with increased alcohol consumption. In some cases, mitochondrial dysfunctions have been detected. Lipomatoses may be solitary, but may also be associated with other symptoms or illnesses (comorbidity). Effective drug therapies are not known, but surgical procedures can alleviate the symptoms. Lipomatosis dolorosa, which can be distinguished from BSL, occurs predominantly in middle-aged women and is characterized by proliferation of adipose tissue and severe pain in the affected areas. Various differential diagnoses and concomitant illnesses need to be considered, in particular depression. There are no evidence-based recommendations for therapy. Repetitive lidocaine infusions can achieve temporary improvement. Recurrence after surgical intervention is not uncommon, but long-term success is also possible.


Asunto(s)
Lipomatosis/diagnóstico , Tejido Adiposo/cirugía , Adiposis Dolorosa/clasificación , Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/etiología , Adiposis Dolorosa/terapia , Diagnóstico Diferencial , Femenino , Humanos , Lidocaína/administración & dosificación , Lipectomía , Lipomatosis/clasificación , Lipomatosis/etiología , Lipomatosis/cirugía , Masculino , Persona de Mediana Edad
14.
Int J Obes (Lond) ; 41(2): 240-245, 2017 02.
Artículo en Inglés | MEDLINE | ID: mdl-27857136

RESUMEN

BACKGROUND: People with lipedema or Dercum's disease (DD) can have a similar distribution of excess painful nodular subcutaneous adipose tissue (SAT), making them difficult to differentiate. METHODS: Case series of 94 patients with DD, 160 with lipedema and 18 with both diagnoses (Lip+DD) from a single clinic in an academic medical center to improve identification and differentiation of these disorders by comparison of clinical findings, prevalence of type 2 diabetes (DM2), hypermobility by the Beighton score and assessment of a marker of inflammation, Total complement activity (CH50). RESULTS: Differences between groups were by Student's t-test with α of 0.05. The Lipedema Group had significantly greater weight, body mass index (BMI), gynoid distributed nodular SAT and fibrotic and heavy tissue than the DD Group. Hypermobility was significantly higher in the Lipedema (58±0.5%) than DD Group (23±0.4%; P<0.0001). DM2 was significantly greater in the DD (16±0.2%; P=0.0007) than the Lipedema Group (6±0.2%). Average pain by an analog scale was significantly higher in the DD (6±2.5%) than the Lipedema Group (4±2.1%; P<0.0001). Fatigue and swelling were common in both groups. Easy bruising was more common in the Lipedema Group, whereas abdominal pain, shortness of breath, fibromyalgia, migraines and lipomas were more prevalent in the DD Group. The percentage of patients with elevated CH50 was significantly positive in both groups. CONCLUSIONS: The significantly lower prevalence of DM2 in people with lipedema compared with DD may be due to the greater amount of gynoid fat known to be protective against metabolic disorders. The high percentage of hypermobility in lipedema patients indicates that it may be a comorbid condition. The location of fat, high average daily pain, presence of lipomas and comorbid painful disorders in DD patients may help differentiate from lipedema.


Asunto(s)
Adiposis Dolorosa/diagnóstico , Lipedema/diagnóstico , Adiposis Dolorosa/epidemiología , Adiposis Dolorosa/patología , Diabetes Mellitus Tipo 2/epidemiología , Diagnóstico Diferencial , Femenino , Humanos , Lipedema/epidemiología , Lipedema/patología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Dolor/etiología , Dolor/patología , Dimensión del Dolor , Guías de Práctica Clínica como Asunto , Grasa Subcutánea/diagnóstico por imagen , Grasa Subcutánea/patología
15.
Wien Med Wochenschr ; 165(17-18): 374-7, 2015 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-26289595

RESUMEN

Juxta-articular adiposis dolorosa is a rare subtype of Dercum's disease. It manifests mainly on the medial parts of the knees. Pain and impaired mobility are common symptoms. We report on four females (aged between 52 and 83 years) who suffered from juxta-articular adiposis dolorosa for more than 10 years. These patients were successfully treated by dermolipectomy resulting in dramatically improved pain and mobility. Adverse effects and complications were minor with a lymph fistula in a single patient which was treated by surgery.


Asunto(s)
Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/cirugía , Lipectomía/métodos , Anciano , Anciano de 80 o más Años , Fístula Cutánea/etiología , Fístula Cutánea/cirugía , Femenino , Fístula/etiología , Fístula/cirugía , Humanos , Enfermedades Linfáticas/etiología , Enfermedades Linfáticas/cirugía , Persona de Mediana Edad , Limitación de la Movilidad , Dimensión del Dolor , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Reoperación
16.
Skeletal Radiol ; 44(6): 839-47, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25672948

RESUMEN

PURPOSE: Adiposis dolorosa (Dercum's disease) is a condition of benign, painful subcutaneous lipomatous lesions associated with weakness, endocrine and lipid abnormalities, and mental disturbances. There is little information documenting the cross-sectional imaging findings that differentiate it from lipomatous and neoplastic soft tissue masses, or massive localized lymphedema. The purpose of this study was to provide a radiological case series of adiposis dolorosa. METHODS: A 10-year retrospective review of the picture archiving and communications system was performed. Two musculoskeletal radiologists reviewed images to confirm and document imaging features, location, size, and patient demographics. Medical records were reviewed to characterize patients into three groups: one group met at least three of the four criteria of Dercum's syndrome, the second group met less than three criteria, and the third group had clinical diagnosis of cellulitis of the lower extremity. RESULTS: Seventeen cases (25 masses) of adiposis dolorosa were found, nine cases of which met at least three criteria of Dercum's syndrome. All cases in the first two groups demonstrated skin thickening and lymphedema of subcutaneous fat, which was fluid attenuation on CT and low or intermediate T1-weighted and high STIR/T2-weighted MR signal. Two cases with pathology showed mild fatty infiltration with fibrous septa, and the third case showed massive localized lymphedema. The third group of ten cellulitis patients demonstrated non-mass-like subcutaneous edema with similar CT attenuation and MR signal characteristics to the first two groups, but differed by the presence of post-contrast enhancement and non-mass-like appearance in 90%. CONCLUSION: Imaging findings of adiposis dolorosa and massive localized lymphedema overlap, as do the symptoms and pathological features. Due to the mass-like engorgement of the soft tissues and pain, patients will often undergo imaging to exclude neoplasm or infection. Knowledge of these conditions and the characteristic imaging findings is important to prevent unnecessary biopsy and misdiagnosis.


Asunto(s)
Adiposis Dolorosa/diagnóstico , Linfedema/diagnóstico , Imagen por Resonancia Magnética/métodos , Tomografía Computarizada por Rayos X/métodos , Adulto , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Estudios Retrospectivos
17.
Clin Radiol ; 68(10): 1047-53, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23809264

RESUMEN

AIM: To describe ultrasound and magnetic resonance imaging (MRI) features of adiposis dolorosa, Dercum's disease, and to evaluate the MRI features prospectively against a large number of MRI examinations. MATERIALS AND METHODS: Institutional review board approval for this study was obtained. The imaging features at MRI and ultrasound of 13 cases of adiposis dolorosa (nine female, four male; age range 32-72 years) were reviewed. MRI findings typical for adiposis dolorosa were proposed and prospectively evaluated on 6247 MRI examinations performed over a period of 8 months. RESULTS: Adiposis dolorosa demonstrates multiple, oblong, fatty lesions in the superficial subcutaneous fatty tissue. They are mostly <2 cm in long axis diameter. They demonstrate nodular ("blush-like") increased fluid signal at unenhanced MRI and are markedly hyperechoic at ultrasound. There is no contrast medium enhancement at MRI and no increased Doppler signal at ultrasound. Most lesions were clinically asymptomatic, some were painful/tender. There was no imaging evidence of oedema or inflammation. During prospective validation of these MRI features on 6247 MRI examinations, two cases with typical imaging features were encountered; both were diagnosed as adiposis dolorosa on clinical review. All cases of adiposis dolorosa showed these imaging findings. This results in a very low likelihood that a nodular, blush-like appearance of subcutaneous fat on MRI is not due to adiposis dolorosa. DISCUSSION: Adiposis dolorosa, Dercum's disease, should be suggested in the presence of multiple (many) small, oblong, fatty lesions in the subcutaneous fatty tissue in adult patients if they are hyperechoic on ultrasound imaging or blush-like at unenhanced MRI; typically a small number of these lesions are tender/painful. Imaging does not demonstrate inflammation or oedema in relation to these lesions. These MRI features should suggest the diagnosis and are likely to be pathognomonic. The radiologist is often the first to suggest the diagnosis based on the imaging features.


Asunto(s)
Adiposis Dolorosa/diagnóstico por imagen , Adiposis Dolorosa/diagnóstico , Imagen por Resonancia Magnética/métodos , Adulto , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Ultrasonografía
18.
J Plast Surg Hand Surg ; 46(6): 421-6, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23088636

RESUMEN

Body composition measurements are used to evaluate surgical treatment, such as bariatric surgery in overweight patients. Nowadays, there are many different methods to measure body composition available. However, none of them has been validated for use in patients after operation. The aim of the present study was to compare the amount of surgically removed fat with two different methods, bioelectrical impedance analysis (BIA) and total body potassium (TBK). Amount of removed fat during liposuction was measured 1, 2, 3, 4, 14 days, and 1 year postoperatively in 27 obese patients after liposuction. The results were compared with actual removed fat during the operation. The median fat mass removed was 4020 grams (range 1954-9655). Postoperatively there was a varying reduction in fat mass, as measured with BIA and with TBK. There was a clear difference between the range of amounts removed measured with BIA and the range of amount removed measured with TBK. This study indicates that BIA, but not TBK, is an appropriate method to measure fat mass in obese subjects in a surgical setting. BIA seems to underestimate the amount of fat removed, whereas TBK seems to overestimate it.


Asunto(s)
Tejido Adiposo/anatomía & histología , Adiposis Dolorosa/cirugía , Lipectomía/métodos , Obesidad/cirugía , Absorciometría de Fotón , Tejido Adiposo/diagnóstico por imagen , Adiposis Dolorosa/diagnóstico , Adulto , Anciano , Composición Corporal , Índice de Masa Corporal , Estudios de Cohortes , Impedancia Eléctrica , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Obesidad/diagnóstico , Tamaño de los Órganos , Medición de Riesgo , Estadísticas no Paramétricas , Suecia , Resultado del Tratamiento , Adulto Joven
19.
Orphanet J Rare Dis ; 7: 23, 2012 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-22546240

RESUMEN

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.


Asunto(s)
Adiposis Dolorosa , Tejido Adiposo/patología , Adiposis Dolorosa/clasificación , Adiposis Dolorosa/diagnóstico , Adiposis Dolorosa/fisiopatología , Adiposis Dolorosa/terapia , Enfermedad Crónica , Femenino , Humanos , Masculino , Obesidad , Dolor/diagnóstico , Dolor/fisiopatología , Enfermedades Raras , Índice de Severidad de la Enfermedad
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