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

  The subjective pain sensation was evaluated with a visual analog scale and number of words chosen, and the objective pain sensation with the mechanical pressure pain threshold. [pubfacts.com]

  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. [pubmed.ncbi.nlm.nih.gov]

  Treatment of pain in Dercum's disease with Lidoderm (lidocaine 5% patch): a case report. Pain Med. Nov 2008;9(8):1224–6. [dermnetnz.org]

• Fatigue

  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]

  Other commonly associated symptoms are fatigue, weakness, chronic pain, thinning pubic hair, and various gastrointestinal problems. [symptoma.com]

  Fibromyalgia syndrome is a common and chronic disorder characterised by widespread muscle pain, fatigue, and multiple tender points. [books.google.de]

  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]

• Severe Pain

  Pain may vary from mild discomfort when a growth is pressed or touched to severe pain that is disproportionate to the physical findings. [autoimmuneregistry.org]

  Some people feel pain occasionally, but there are others who are constantly in severe pain. A decrease in mental function can also affect a person’s ability to complete job tasks. [kolblegal.com]

  Pain associated with Dercum’s disease can often be severe. Pain may be caused by these growths pressing on nearby nerves. Dercum’s disease mainly occurs in adults and more women are affected than men. [diseasesdic.com]

• 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]

  It may be associated with thyroid dysfunction and may present as persistent fatigue and weight gain despite biochemical euthyroidism. [endocrine-abstracts.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]

  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]

• 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]

  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. [doi.org]

  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]

• Muscle Weakness

  On physical examination she had full facies, oral candidiasis, a "buffalo hump," marked truncal obesity, and grade 3/5 proximal muscle weakness. Diffuse subcutaneous induration was present in all her extremities. [dercum.tripod.com]

  The signs and symptoms of DD include “chronic pain, fatigue, brain fog, insomnia, cardiac arrhythmia most often tachycardia (palpitations), gastrointestinal distress, often irritable bowel syndrome, muscle weakness, tremor or jerking of muscles (myoclonus [futuremedicine.com]

• Swelling of Hand

  There is history of paresthesias and swelling of hands. There is no history of morning stiffness, tiredness, headache, depression, loss of sleep and appetite or bruising tendency. [edoj.org.eg]

• Subcutaneous Nodule

  Figure 1: A swan neck deformity in fingers and multiple 1 to 2 cm subcutaneous nodules. Figure 2: Diagnosed with rheumatoid arthritis and a biopsy of a nodule in the left forearm was performed. [ijocs.org]

  Subnaik Affiliations University of Louisville, Lousville, KY Dercum’s disease (Adiposis dolorosa) is a rare disorder characterized by multiple, tender subcutaneous nodules on the trunk and extremities. [jpain.org]

  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. Copyright © 2017, 2012 Decision Support in Medicine, LLC. All rights reserved. [clinicaladvisor.com]

  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]

• Skin Disease

  On DermNet NZ: Lipoma Angiolipoma Metabolic syndrome and obesity Lipoedema Other websites: Adiposis Dolorosa – Medscape Drugs & Diseases Adiposis dolorosa – Genetics Home Reference Dercum's disease support Books about skin diseases: See the DermNet NZ [dermnetnz.org]

  […] action=getRecordDetail&idt=16868247 Other title Dercum's disease: a severe complication in a rare disease. [pascal-francis.inist.fr]

  Read More Obesity and the skin: skin physiology and skin manifestations of obesity. [pubfacts.com]

• Subcutaneous Mass

  Diffuse subcutaneous induration was present in all her extremities. Markedly tender, well-circumscribed, fist-sized subcutaneous masses superior to both knees were noted (Fig 2). There was no effusion in the knees. [dercum.tripod.com]

  The condition encompasses painful subcutaneous masses and is therefore a possible differential diagnosis to Dercum’s disease. [doi.org]

• Skin Rash

  Or, ‘What will show up on my body today, more lumps, facial rashes, skin rashes, my skin rupturing open for no reason?’ I have great support from my family and friends, thank God. It’s not easy for them to watch me in pain. [warwickonline.com]

  Histopathologic findings in full-thickness skin biopsy specimen of clinically involved skin show markedly thickened fascia. [dercum.tripod.com]

• Confusion

  This disorder usually occurs in obese, postmenopausal women and is associated with weakness and mental disturbances such as depression, confusion, lethargy, and dementia. The cause is unknown, and there is no specific treatment. [ncbi.nlm.nih.gov]

  Other signs and symptoms that have been reported to occur with adiposis dolorosa include general weakness and tiredness (fatigue), depression, irritability, confusion, recurrent seizures (epilepsy), and a progressive decline in intellectual function [rarediseases.oscar.ncsu.edu]

  Adiposis dolorosa or Dercum's disease is characterised by the development of multiple, painful, subcutaneous lipomas in association with obesity, asthenia and fatigue, and range of mental disturbances including instability, depression, confusion, dementia [monarchinitiative.org]

• 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]

  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]

  Symptoms are asthenia, headache, frequently amenorrhoea and ecchymoses; diminution of sweating. Mental depression and deterioration have been observed in an appreciable number of these patients. Long progressive course. [whonamedit.com]

  (e.g., tension headaches, classic migraine, neck headaches) Diagnostic Modalities Laboratory tests are very nonspecific. [academic.oup.com]

  […] 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]

• Paresthesia

  Paresthesia in the overlying skin is common. Lipomas about the joints cause mechanical arthralgia. The diagnosis, which is often delayed, rests on ultrasonography and, above all, magnetic resonance imaging. [em-consulte.com]

  Paresthesia and ecchymosis have been noted in the skin overlying the fatty deposits. Sparse pubic and axillary hair have also been reported. [orpha.net]

  In addition to being painful, it is also common for paresthesia of the overlying skin of the individual lipomas to be present. [visualdx.com]

• Memory Impairment

  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]

  Memory impairment, difficulties in thought processes, sleep derangements, depression, anxiety, and other psychiatric disorders can occur. [symptoma.com]

  Bleeding or Clotting Disorders, Anxiety, Cardiovascular Disease, Vision And Hearing Disturbances, Migraines, Neuralgia, Extreme Joint Pain, Mast Cell Disorders, Memory Impairment, Brain Fog, Neuropathy; the list goes on and on. [change.org]

• Insomnia

  She had been taking L-tryptophan (4 g/d) for 3 months for treatment of insomnia. Her physical examination was notable for proximal muscle weakness and cutaneous induration of the arms and legs; the hands were spared. [dercum.tripod.com]

  […] 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 to supraventricular tachycardia requiring beta-blockers Sleep disturbance; insomnia [web.archive.org]

  […] bowel syndrome and other gastrointestinal problems migraines feeling of fullness joint pain and/or stiffness (especially when fat deposits are present) muscle pain and stiffness shortness of breath tachycardia (rapid heart rate) sleep disturbances (insomnia [diseasesdic.com]

  CBD oil provides undisturbed sleep by preventing insomnia. Proper sleep is very much important for better health. Following are the benefits of CBD oil. It helps in reducing depression and anxiety. Provide undisturbed sleep at the night. [thefreshcarrot.ca]

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].

26919646 ) Yu R....Wachsman A. 2016 8 Imaging findings of adiposis dolorosa vs. massive localized lymphedema. ( 25672948 ) Petscavage-Thomas J.M....Bennett J. 2015 9 Juxta-articular adiposis dolorosa (Dercum's disease type IV): report of four cases and treatment [malacards.org]

The L-tryptophan therapy was discontinued, and treatment with prednisone (60 mg/d) was started. Six weeks later, because of the lack of response, the dosage of prednisone was increased to 80 mg/d. [dercum.tripod.com]

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]

Treatment consisted in 4 cycles of 30 minutes FREMS sessions over a 6-month period. [ncbi.nlm.nih.gov]

Prognosis The disease course is chronic and progressive. The documents contained in this web site are presented for information purposes only. [orpha.net]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Consultations Enhancing Healthcare Team Outcomes Review Questions [pubmed.ncbi.nlm.nih.gov]

Prognosis Adiposis dolorosa tends to be a chronic disease that does not abate over time and may progress to become more debilitating as time goes on. [statpearls.com]

Prognosis - Adiposis dolorosa Not supplied. Treatment - Adiposis dolorosa Resources - Adiposis dolorosa [checkorphan.org]

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 etiology remains unknown but several hypotheses have been proposed including endocrine dysfunction, nervous system dysfunction, mechanical pressure on the nerves, adipose tissue dysfunction, inflammation, infection and induced by trauma or [orpha.net]

- Dercum's disease is a rare condition of unknown etiology characterized by multiple, often painful lipomas. Paresthesia in the overlying skin is common. Lipomas about the joints cause mechanical arthralgia. [em-consulte.com]

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]

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]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Consultations Enhancing Healthcare Team Outcomes Review Questions [pubmed.ncbi.nlm.nih.gov]

Most cases of the disease are found to be sporadic with no specific genetic mutations. [3] [4] [5] Epidemiology No significant epidemiologic data is available but based on case reports adiposis dolorosa is found more frequently in obese females ages 35 [statpearls.com]

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]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Consultations Enhancing Healthcare Team Outcomes Review Questions [pubmed.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]

Possible risk factors include obesity, alcohol abuse, liver disease, glucose intolerance, and soft tissue trauma (see “Etiology and Pathophysiology”). [unboundmedicine.com]

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]

Prevention There are currently no proofs and studies available, that support the prevention of the Dercum’s Disease or Adiposis Dolorosa. [diseasesdic.com]

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]

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.