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Adiposis Dolorosa

Dercum's Disease

Adiposis dolorosa is a disorder of unknown etiology found in obese individuals. The condition is defined by the presence of numerous painful lipomas, along with a range of other symptoms. There is usually no familial pattern of inheritance.


Presentation

Adiposis dolorosa (AD) is a disease that usually affects women, but has also been observed in men [1] [2]. It rarely occurs in children. It is frequently reported in peri-menopausal women, although 35-50 years is the age range often stated in literature [3]. AD presents with generalized obesity, as well as numerous, painful lipomas which vary in size, primarily affecting the torso and extremities, although there is no involvement of the hands. AD does not affect the face.

Other commonly associated symptoms are fatigue, weakness, chronic pain, thinning pubic hair, and various gastrointestinal problems. Pain is progressive, increases with body mass index (BMI), and may be severe enough to limit mobility and disrupt daily activity [1] [2]. It may also be experienced as tenderness on palpation or as transient episodes, and is affected by changes in weather and temperature [4].

Patients may present with easy bruising, angiolipomas, menstrual changes, bleeding diatheses, migraines, joint pain or stiffness, and myalgias. More general symptoms such as non-pitting edema, dyspnea, tachycardia and fatigue have been reported [2]. Memory impairment, difficulties in thought processes, sleep derangements, depression, anxiety, and other psychiatric disorders can occur.

AD may be classified into Type I, with widespread distribution of painful fat tissue without recognizable lipomas (generalized diffuse form); Type II, with pain in both diffuse adipose tissue and in localized lipomas (generalized nodular form); Type III, with pain in numerous lipomas (localized nodular form); and type IV, with singular fat accumulations near the joints (juxta-articular form) [5]. There are cases of a familial form of AD, with an autosomal dominant pattern of inheritance [5]. The particular gene mutation has not yet been discovered.

Proposed precipitating factors are prolonged high dose steroid use, trauma, endocrine or adipose tissue abnormalities, as well as autoimmune conditions [5] [6].

Pain
  • 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.[ncbi.nlm.nih.gov]
  • Pain usually increases when a lipoma is bumped or pressed. In some cases, just clothing touching the skin is enough to cause pain.[web.archive.org]
  • pain in Dercum's disease.[en.wikipedia.org]
Fatigue
  • Other commonly associated symptoms are fatigue, weakness, chronic pain, thinning pubic hair, and various gastrointestinal problems.[symptoma.com]
  • 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.[doi.org]
  • It is might be associated with obesity, emotional features or fatigue. Females are more likely to develop this disease than men. Tumors are painful and present in multiples on extremities. It occurs in the age of 35-50 years.[curerator.com]
  • Her systemic symptoms included generalized weakness, fatigue, memory impairment, and arthralgias. Although some of the lesions were tender, most were only appreciated with palpation.[ncbi.nlm.nih.gov]
  • Fibromyalgia syndrome is a common and chronic disorder characterised by widespread muscle pain, fatigue, and multiple tender points.[books.google.de]
Weight Gain
  • 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.[doi.org]
  • In some cases, affected individuals may also experience weight gain, depression, lethargy, and/or confusion. The exact cause of Dercum’s disease is unknown.[diseasesdic.com]
  • gain in most Vascular involvement; angiolipomas Multiple cherry angiomas and petechiae Easy bruising Flushing Heavy or prolonged menstrual bleeding Hematuria of unknown cause Non-pitting edema in subcutaneous fat GERD; gastro-esophageal reflux disease[web.archive.org]
  • Significant weight gain is a common occurrence for most individuals affected by Dercum’s disease.[rarediseases.org]
  • The patient experienced recurrent weight gain and lipoma pain with discontinuation of these medications. [38] Desai et al reported on treatment with a lidocaine (5%) patch, [39] and Lange et al reported on successful therapy with pregabalin with manual[emedicine.medscape.com]
Weight Loss
  • Weight loss does not result in pain relief or resolution of the lipomas. Surgical options include liposuction or excision. Recurrence of the lipomas after surgical removal, either at the same site or at other locations, is common.[orpha.net]
  • Adiposis dolorosa (AD) is a rare disorder of painful nodular subcutaneous fat accompanied by fatigue, difficulty with weight loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia.[ncbi.nlm.nih.gov]
  • loss, inflammation, increased fluid in adipose tissue (lipedema and lymphedema), and hyperalgesia.[dx.doi.org]
Fever
  • Some patients report feeling hot; in rare cases adiposis dolorosa is associated with low-grade fever. Morning stiffness may occur.[dermatologyadvisor.com]
  • Still, other symptoms may present themselves, such as a long-term, low-grade fever, minor headaches, and random, otherwise unexplained swelling or bruising of body parts. Adiposis dolorosa is a rare disease, therefore detailed research is lacking.[wisegeek.com]
  • No satisfactory treatment is available for the prevention or management of the chronic syndrome, but the use of corticosteroids in the early stages of EMS frequently results in improvement of myalgias, tissue swelling, fever, and respiratory symptoms[dercum.tripod.com]
  • It predominantly affects middle age men of mediterranean origin A systemic disease, often associated with arthritis, fever, and weight loss. The cutaneous lesions consist of histiocytes, which are usually confined to the dermis.[icd9data.com]
Early Satiety
  • […] angiomas and petechiae Easy bruising Flushing Heavy or prolonged menstrual bleeding Hematuria of unknown cause Non-pitting edema in subcutaneous fat GERD; gastro-esophageal reflux disease Irritable bowel syndrome, bloating, and abdominal pain Migranes Early[web.archive.org]
Thrombosis
  • This is a case study suggesting lipedema with associated morbid obesity as a possible risk factor for arterial thrombosis. There are no similar cases reported in literature, and further research may throw some light on this rare entity.[dercumsdiseaseresearch.wordpress.com]
  • No symptomatic postoperative deep venous thrombosis was seen. The aspirate was collected in 2,000 mL plastic containers graded to an accuracy of 20 mL.[doi.org]
Palpitations
  • , bloating, and abdominal pain Migranes Early satiety Joint pain and/or stiffness; increased in areas of fat deposits Muscle pain and stiffness; especially upon wakening or the day after physical activity Shortness of breath Tachycardia; rapid heart palpitations[web.archive.org]
Subcutaneous Nodule
  • A 41-year-old woman with painful subcutaneous nodules. N Engl J Med. 2006;355:714-722. Wortham NC, Tomlinson IP. Dercum’s disease. Skinmed. 2005;4:157-62. Steiner J, Schiltz K, Heidenreich F, Weissborn K.[rarediseases.org]
  • A 41-year-old woman with painful subcutaneous nodules”. N Engl J Med. vol. 17;355. 2006. pp. 714-22. (A well-written differential diagnosis of adiposis dolorosa and its management.) Copyright 2017, 2013 Decision Support in Medicine, LLC.[dermatologyadvisor.com]
  • A 41-year-old woman with painful subcutaneous nodules". N Engl J Med. vol. 17;355. 2006. pp. 714-22. Copyright 2017, 2012 Decision Support in Medicine, LLC. All rights reserved.[clinicaladvisor.com]
  • Surgical removal or liposuction may help, but lipomas may recur. 0012378 Obesity Having too much body fat 0001513 Psychosis 0000709 Subcutaneous nodule Firm lump under the skin Growth of abnormal tissue under the skin [5] [6] [4] Because the condition[rarediseases.info.nih.gov]
Compulsive Disorder
  • Post-traumatic stress disorder (PTSD), obsessive-compulsive disorder (OCD), and generalised anxiety disorder (GAD) have been expressly described in a community sample of patients with fibromyalgia [ 16 ].[ncbi.nlm.nih.gov]
Psychiatric Manifestation
  • Furthermore, in 1930, Gram presented 69 cases of Dercum’s disease and he did not note explicit psychiatric manifestations in any them [ 11 ].[ncbi.nlm.nih.gov]
Headache
  • 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.[ncbi.nlm.nih.gov]
  • (e.g., tension headaches, classic migraine, neck headaches) Diagnostic Modalities Laboratory tests are very nonspecific.[academic.oup.com]
  • Some patients may have headaches or difficulty sleeping. Diagnosis Dercum's disease is very rare and difficult to diagnose, and there are no tests that can be used to conclusively diagnose the condition.[web.archive.org]
  • […] dementia confusion memory issues depression the difficulty with concentration rapid heart beat bruised easily obesity shortness of breath painful fatty lipomas stiffness trouble with sleeping diabetes bloating joint aches emotional instability constipation headache[curerator.com]
Babinski Sign
  • Neurologically, a positive Babinski’s sign was found on the left side and sensation errors for vibration were noted at and below the iliac crest. During surgery, an adipose tissue tumour was found connected with the peritoneum.[ncbi.nlm.nih.gov]
Stroke
  • To date, there has been no recorded association with an increased risk of VTE but there have been reports of stroke-like events. It is unclear if these are caused by the condition itself or are co-incidental.[dercumsdiseaseresearch.wordpress.com]
  • In 1999, Tom began having difficulty walking, his handwriting changed and a series of small strokes exacerbated the onset of dementia and erased any recollection of regular falls and missteps that left bruises and broken bones.[globalgenes.org]

Workup

The confirmation of adiposis dolorosa is based on certain diagnostic criteria, with minimum requirements of being obese or overweight, and experiencing chronic pain in fat tissue for 3 or more months [5]. Physical exam is adequate for making a diagnosis. Dissection of resected masses can further solidify the diagnosis [7]. Reaching the correct diagnosis may be challenging due to varying presentations.

Imaging techniques, including ultrasound and magnetic resonance imaging (MRI), can be used non-invasively to ensure that the masses are lipomas [8] [9] [10]. Laboratory tests may be carried out to exclude any suspected endocrine disorders such as thyroid or adrenal disease. High erythrocyte sedimentation rate (ESR), alpha-1 antitrypsin, haptoglobin, and complement levels may be noted [11].

Treatment

  • Management and treatment Treatment is symptomatic. Pain may be relieved by intravenous administration of lidocaine, local corticosteroid (prednisone) injections and combinations of mexiletine and amitriptyline, or infliximab and methotrexate.[orpha.net]
  • Ultrasound and magnetic resonance imaging can play a role in diagnosis. [9] Treatment [ edit ] Common treatments for Dercum's disease is directed towards treating the individual symptoms.[en.wikipedia.org]
  • Treatment consisted in 4 cycles of 30 minutes FREMS sessions over a 6-month period.[ncbi.nlm.nih.gov]

Prognosis

  • Prognosis The disease course is chronic and progressive. The documents contained in this web site are presented for information purposes only.[orpha.net]
  • Prognosis - Adiposis dolorosa Not supplied. Treatment - Adiposis dolorosa Resources - Adiposis dolorosa[checkorphan.org]
  • Adiposis Dolorosa Prognosis and Diagnosis The prognosis of Dercum’s disease is that it’s a chronic condition that develops gradually.[myphiladelphiadisabilitylawyer.com]

Etiology

  • Dercum's disease mainly occurs in adults and more women are affected than men. (1) Etiology T he specific etiology of Dercum's Disease is unknown.[web.archive.org]
  • The etiology is uncertain, but is probably multifactorial. Numerous treatments to relieve the pain have generally been unsuccessful. A patient with adiposis dolorosa was treated with intravenous infusions of lidocaine over a two-year period.[ncbi.nlm.nih.gov]
  • Etiology The aetiology remains unknown but several hypotheses have been proposed including an autoimmune mechanism, alterations in fatty acid or carbohydrate metabolism and endocrine anomalies.[orpha.net]

Epidemiology

  • Summary Epidemiology Prevalence is unknown. The disease usually occurs in postmenopausal women between 45 and 60 years of age but a few cases have been reported in males.[orpha.net]
  • Definition / general Tender accumulations of subcutaneous fat / lipomas, often in pelvis and lower extremities of postmenopausal women Terminology Also called Dercum disease Epidemiology Associated with women, obesity, hypercholesterolemia Clinical features[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • Understanding the pathophysiology and metabolism of lipomatosis in AD may contribute to a better understanding of metabolism in non-lipomatosis obesity.[ncbi.nlm.nih.gov]
  • Although many cases of adiposis dolorosa have been described in the literature, 2, 3 4 5 little has been added in clinical description, pathophysiologic interpretation or etiologic definition since Dercum's original publication.[nejm.org]
  • In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may[skincareguide.ca]

Prevention

  • Prevention High quality scientific evidence regarding prevention of Dercum's disease is currently lacking.[web.archive.org]
  • Knowledge of these conditions and the characteristic imaging findings is important to prevent unnecessary biopsy and misdiagnosis.[ncbi.nlm.nih.gov]
  • Thus, early recognition of lipedema by its clinical manifestations is necessary to prevent complications such as ischemia. Full Text : Mutnal S, Rangappa P, Jacob I, Chaitanya K, Rao K.[dercumsdiseaseresearch.wordpress.com]

References

Article

  1. Learning About Dercum Disease. National Human Genome Research Institute. http://www.genome.gov/17516629. Updated June 27, 2012. Accessed March 6, 2018.
  2. Dercum's Disease. Fat Disorders Research Society. http://www.fatdisorders.org/fat-disorders/dercums-description. Accessed March 6, 2018.
  3. Campen R, Mankin H, Louis DN, Hirano M, Maccollin M. Familial occurrence of adiposis dolorosa. J Am Acad Dermatol. 2001;44(1):132-136.
  4. Brodovsky S, Westreich M, Leibowitz A, Schwartz Y. Adiposis dolorosa (Dercum's disease): 10-year follow-up. Ann Plast Surg. 1994;33(6):664-668.
  5. Hansson E, Svensson H, Brorson H. Review of Dercum's disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012;7:23.
  6. Adiposis dolorosa. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/adiposis-dolorosa. Reviewed July, 2012. Accessed March 6, 2018.
  7. Dercum's disease. National Organization for Rare Disorders. https://rarediseases.org/rare-diseases/dercums-disease/. Accessed March 6, 2018.
  8. Amine B, Leguilchard F, Benhamou CL. Dercum's disease (adiposis dolorosa): a new case-report. Joint Bone Spine. 2004;71(2):147-149.
  9. Tins BJ, Matthews C, Haddaway M, et al. Adiposis dolorosa (Dercum's disease): MRI and ultrasound appearances. Clin Radiol. 2013;68(10):1047-1053.
  10. Petscavage-Thomas JM, Walker EA, Bernard SA, Bennett J. Imaging findings of adiposis dolorosa vs. massive localized lymphedema. Skeletal Radiol. 2015;44(6):839-847.
  11. Greenbaum SS, Varga J. Corticosteroid-induced juxta-articular adiposis dolorosa. Arch Dermatol. 1991;127(2):231-233.

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Last updated: 2019-07-11 21:32