Diagnosi differenziale DD e patologie RAD

RISORSE INFORMATIVE

2017 Feb;41(2):240-245.
 doi: 10.1038/ijo.2016.205. Epub 2016 Nov 18.

Differentiating lipedema and Dercum’s disease

Affiliations 

Abstract
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.

Hansson et al. Orphanet Journal of Rare Diseases 2012, 7:23http://www.ojrd.com/content/7/1/23

Review of Dercums disease and proposal of diagnostic criteria, diagnostic methods, classification and management. 

Emma Hansson, Henry Svensson, Håkan Brorson

Reumatologia. 2019; 57(5): 281–287.
Published online 2019 Oct 31. doi: 10.5114/reum.2019.89521
PMCID: PMC6911249
PMID: 31844341


Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management


Eugeniusz J. Kucharz,corresponding author Magdalena Kopeć-Mędrek, Justyna Kramza, Monika Chrzanowska, and Przemysław Kotyla

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.

Keywords: Dercum’s disease, adiposis dolorosa, subcutaneous fat masses

Adiposis Dolorosa

In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan.
.

Excerpt

Adiposis dolorosa is a rare disorder of multiple painful subcutaneous growths of adipose tissue. It is also known as Dercum disease, Ander syndrome, morbus Dercum, adipose tissue rheumatism, adiposalgia, or lipomatosis dolorosa. This disease was first discovered in the late 1800s by American neurologist Francis Xavier Dercum. It is classified into 4 types which include generalized diffuse, generalized nodular, localized nodular, and juxta-articular forms. The generalized diffuse type presents with widespread painful adipose tissue with no apparent lipomas. The generalized nodular type presents with widespread, painful adipose tissue that is more painful in the vicinity of lipomas. In the localized nodular type, the pain is restricted to areas within and around lipomas. Lastly, the juxta-articular type presents as painful solitary adipose tissue near large joints. The diagnosis of adiposis dolorosa is made clinically and is a diagnosis of exclusion. The proposed criteria for the diagnosis includes chronic pain of the subcutaneous tissue for over 3 months in overweight patients or patients with obesity, though the criteria still need to be validated. The pain is often disabling and resistant to treatment. The disease is associated with weakness and psychiatric symptoms such as depression. Other associated symptoms include fatty deposits, easy bruising, sleep disturbances, impaired memory, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, and joint pain.

 . 2020; 19: e00682.

Published online 2019 Dec 19. doi: 10.1016/j.idcr.2019.e00682
PMCID: PMC6940691
PMID: 31908946

Infections preceding the development of Dercum disease

Karen Beltraninvestigation, Rita Wadeeainvestigation, and Karen L. Herbstinvestigation

Abstract
Dercum Disease (DD) is a rare inflammatory disease of painful subcutaneous fat masses with known alterations in lymphatic vessels. DD masses vary from pearl to walnut-size or larger and occur anywhere in body fat. Signs and symptoms of DD are similar to fibromyalgia. While the etiology of DD is unknown, metabolic, autoimmune, or autosomal genetic transmission has been proposed. This series presents 7 cases where DD followed an infection either histoplasmosis, coccidioidomycosis or Lyme disease. Known changes in fat by infectious agents are reviewed. Early diagnosis and treatment of infections may prevent further damage to fat.

Keywords: Histoplasmosis, Toxoplasmosis, Lyme disease, Coccidioidomycosis, Dercum disease

Abstract
Objective: To describe signs and symptoms of adiposis dolorosa (AD) in a large cohort of individuals with this disorder to increase recognition, diagnosis, and research of this syndrome. Design: Survey. Subjects: One hundred ten men and women with AD. Measurements: The survey included questions on demographics, obesity, signs and symptoms in soft tissues and body systems, susceptibility to infection, diet, treatments and lifestyles, medical and family history, habits, and exercise. Results: Subjects were white, non-Hispanic except for 3 Hispanics and 2 African Americans, and mostly women (82.7%) of northern European ancestry living in the United States (87.3%), average age 48.5 ± 10 years. The majority of respondents were obese, body-mass index of 34.7 ± 8.7 kg/m2 and had AD for 14.5 ± 11.9 years. Onset of growths began primarily in the arm or leg at a mean age of 34.5 ± 12.8 years (range, 7-67 years). The majority reported pain, fatigue, weight gain (25.7 ± 15 kg), multiple growths, and weakness at onset. The median number of palpable growths was 77.3 ± 84.5 in subcutaneous fat from scalp to dorsal foot. Growths ranged from firm and pea-sized to large, fixed, and deep. Pathology descriptions of growths included lipoma, fibrolipoma, or angiolipomas. Autoimmune disease and fibromyalgia were prominent (38.2%), as was diabetes (16.4%). Almost all respondents reported exercise-associated pain in growths, and 100% saw no decrease in growths with weight loss. Over 75% had liposuction and over half reported regrowth. Almost half reported family members with growths. Conclusion: Our survey results suggest AD is a painful syndrome involving growths in all subcutaneous fat. It can be inherited or sporadic, beginning in the third decade of life, but can occur in children and is accompanied by obesity, fatigue, joint and muscle aches, and other signs and symptoms affecting multiple organ systems. Exercise induces pain and has no effect on fatty growth size. The etiology of this syndrome remains unknown.

Dercum’s Disease (Adiposis Dolorosa) in Orphanet

Dercum’s Disease (Adiposis Dolorosa) in Dr. Herbst’s Website

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