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Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management
 
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Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland
 
 
Submission date: 2019-07-23
 
 
Final revision date: 2019-08-07
 
 
Acceptance date: 2019-09-17
 
 
Online publication date: 2019-10-31
 
 
Publication date: 2019-11-19
 
 
Reumatologia 2019;57(5):281-287
 
KEYWORDS
TOPICS
ABSTRACT
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.
 
REFERENCES (49)
1.
Patel DA, Swan KG. Francis Xavier Dercum: a man for all seasons. Ann Clin Transl Neurol 2014; 1: 233-237.
 
2.
Dercum FX. A subcutaneous connective tissue dystrophy of the arms and back, associated with symptoms resembling myxedema. Univ Med Mag (Philadelphia) 1888; 1: 1-11.
 
3.
Dercum FX. There cases of a hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special nervous symptoms – adiposis dolorosa. Am J Med Sci 1892; 101: 521-523.
 
4.
Anders JM. A Textbook Of The Practice Of Medicine. W.B. Saunders & Company, Philadelphia 1897.
 
5.
White WH. A case of adiposis dolorosa. Br Med J 1899; 2: 1533-1534.
 
6.
Hansson E, Svensson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rave Dis 2012; 7: 23.
 
7.
Herbst KL, Asare-Bediako S. Adiposis Dolorosa in More than Painful Fat. Endocrinologist 2007; 17: 326-334.
 
8.
Wortham NC, Tomlinson IP. Dercum’s disease. Skinmed 2005; 4: 157-162.
 
9.
Izar MCO, da Fonseca HAR, França CN, et al. Rare presentation of Dercum’s Disease in a child with Abnormalities in Lipoprotein Metabolism. Arq Bras Cardiol 2018; 111: 755-757.
 
10.
Campen R, Mankin H, Louis DN, et al. Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 2001; 44: 132-136.
 
11.
Cantu JM, Ruiz-Barquin E, Jimenez M, et al. Autosomal dominant inheritance in adiposis dolorosa (Dercum’s disease). Humangenetik 1973; 18: 89-91.
 
12.
Lynch HT, Harlan WL. Hereditary factors in Adiposis Dolorosa (Dercum’s Disease). Am J Hum Genet 1963; 15: 184-190.
 
13.
Roux J, Vitaut M. Maladie de Dercum (Adiposis dolorosa). Revue Neurol (Paris) 1901; 9: 881-888.
 
14.
Cantone M, Lanza G, Pennisi M, et al. Prominent neurological involvement in Dercum disease. J Neurol 2017; 264: 796-798.
 
15.
Hansson E, Svansson H, Brorson H. Depression in Dercum’s disease and in obesity: a case control study. BMC Psychiatry 2012; 12: 74.
 
16.
Trentin C, Di Nubila B, Cassano E, Bellomi M. A rare cause of mastalgia: Dercum’s disease (adiposis dolorosa). Tumori 2008; 94: 762-764.
 
17.
Atkinson RL. Adiposis dolorosa as an etiology of abdominal pain. Wien Klin Wochenschr 2008; 120: 251.
 
18.
Amine B, Leguilchard F, Benhamou CL. Dercum’s disease (adiposis dolorosa): a new case-report. Joint Bone Spine 2004; 71: 147-149.
 
19.
Haddad D, Athmani B, Costa A, Cartier S. Maladie de Dercum: une complication grave au cours d’une maladie rare. À propos d’un cas. Ann Chir Plast Estét 2005; 50: 247-250.
 
20.
Kawale J, Mahore A, Dange N, Bhoyar K. Adiposis dolorosa of scalp presenting with severe headache: an unusual case. J Headache Pain 2010; 11: 539-541.
 
21.
Szypuła I, Kotulska A, Szopa M, et al. Adiposis dolorosa with hypercholesterolemia and premature severe generalized atherosclerosis. Wiad Lek 2009; 62: 64-65.
 
22.
Łabuzek K, Liber S, Suchy D, Okupień B. A successful case of pain management using methformin in a patient with adiposis dolorosa. Int J Clin Pharmacol Ther 2013; 51: 517-524.
 
23.
Hansson E, Manjer J, Svensson H, et al. Neuropeptide levels in Dercum’s disease (adiposis dolorosa). Reumatismo 2012; 64: 134-141.
 
24.
Hao D, Olugbodi A, Udechukwu N, Donato AA. Trauma-induced adiposis dolorosa (Dercum’s disease). BMJ Case Rep 2018; 2018: pii; bcr-2017-223869.
 
25.
Rasmussen JC, Herbst KL, Aldrich MB, et al. An abnormal lymphatic phenotype is associated with subcutaneous adipose tissue deposits in Dercum’s disease. Obesity 2014; 22: 2186-2192.
 
26.
Kosseifi S, Anaya E, Dronovalli G, Leicht S. Dercum’s disease an unusual presentation. Pain Med 2010; 11: 1430-1434.
 
27.
Kucharz EJ, Kramza J, Kotyla P, Kotulska A. Mixed generalized/juxta-articular form of Dercum’s disease. Reumatologia 2016; 54: 212.
 
28.
Herbst KL, Coviello AD, Chang A, et al. Lipomatosis-associated inflammation and excess collagen may contribute to lower relative resting energy expenditure in women with adiposis dolorosa. Int J Obes (Lond) 2009; 33: 1031-1038.
 
29.
Hansson E, Svensson H, Stenram U, Brorson H. Histology of adipose tissue inflammation in Dercum’s disease, obestity and normal weight controls: a case control study. J Inflamm (Lond) 2011; 8: 24.
 
30.
Tins BJ, Matthews C, Haddaway M, et al. Adiposis dolorosa (Dercum’s disease): MRI and ultrasound appearances. Clin Radiol 2013; 68: 1047-1053.
 
31.
Chen SG, Hsu SD, Chen TM, Wang HJ. Painful fat syndrome in a male patient. Br J Plast Surg 204; 57: 282-286.
 
32.
Buck DW II, Herbst KL. Lipedema: A Relatively Common disease with Extremely Common Misconceptions. Plast Reconstr Surg Glob Open 2016; 4: e1043.
 
33.
Petscavage-Thomas JM, Walker EA, Bernard SA, Bennett J. Imaging finding of adiposis dolorosa vs. massive localized lymphedema. Skeletal Radiol 2015; 44: 839-847.
 
34.
Wollina U, Goldman A, Heinig B. Microcannular tumescent liposuction in advanced lipedema and Dercum’s disease. G Ital Dermatol Venerol 2010; 145: 151-159.
 
35.
Hansson E, Svensson H, Brorson H. Liposuction may reduce pain in Dercum’s disease (adiposis dolorosa). Pain Med 2011; 12: 942-952.
 
36.
Hansson E, Manjer J, Svensson H, Brorson H. Quality-of-life in patients with Dercum’s disease – before and after liposuction. J Plast Surg Hand Surg 2012; 46: 252-256.
 
37.
Hansson E, Svensson H, Rosén I, Brorson H. Thermal and vibratory thresholds after liposuction in patients with Dercum’s disease. J Plast Surg Hand Surg 2011; 45: 72-76.
 
38.
De Silva M, Earley MJ. Liposuction in the treatment of juxtaarticular adiposis dolorosa. Ann Rheum Dis 1990; 49: 403-404.
 
39.
Wollina U, Heining B, Langer D, Nowak A. Juxta-articular adiposis dolorosa (Dercum’s disease type IV): report of four cases and treatment by dermolipectomy. Wien Med Wochenschr 2015; 165: 374-377.
 
40.
Held JL, Andrew JA, Kohn SR. Surgical Amelioration of Dercum’s Disease: a Report and Review. J Dermatol Surg Oncol 1989; 15: 1294-1296.
 
41.
Ibarra M, Eekema A, Ussery C, et al. Subcutaneous adipose tissue therapy reduces fat by dual X-ray absorptiometry scan and improves tissue structure by ultrasound in women with lipoedema and Dercum disease. Clin Obes 2018; 8: 398-406.
 
42.
Desai MJ, Siriki R, Wang D. Treatment of pain in Dercum’s disease with Lidoderm (Lidocaine 5% patch): a case report. Pain Med 2008; 9: 1224-1226.
 
43.
Petersen P, Kastrup J. Dercum’s disease (adiposis dolorosa). Treatment of the severe pain with intravenous lidocaine. Pain 1987; 28: 77-80.
 
44.
Martinenghi S, Caretto A, Losio C, et al. Successful treatment of Dercum’s disease by Transcutaneous Electrical Stimulation: a Case Report. Medicine (Baltimore) 2015; 94: e950.
 
45.
Lange U, Oelzer P, Uhlemann C. Dercum’s disease (Lipomatosis dolorosa): successful therapy with pregabalin and manual lymphatic drainage and a current overview. Rheumatol Int 2008; 29: 17-22.
 
46.
Gonciarz Z, Mazur W, Hartleb J, et al. Interferon alfa-2b induced long-term relief of pain in two patients with adiposis dolorosa and chronic hepatitis C. J Hepatol 1997; 27: 1141.
 
47.
Singal A, Janiga JJ, Bossenbroek NM, Lim HW. Dercum’s disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate. J Eur Acad Dermatol Venereol 2007; 21: 717.
 
48.
Herbst KL, Rutledge T. Pilot study: rapidly cycling hypobaric pressure improves pain after 5 days in adiposis dolorosa. J Pain Res. 2010; 3: 147-153.
 
49.
Wipf A, Lofgreen S, Miller DD, Farah RS. Novel Use of Deoxycholic Acid for Adiposis Dolorosa (Dercum disease). Dermatol Surg 2019; 10.1097/DSS.0000000000001800 [Epub ahead of print].
 
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