MRI and ultrasound appearances

Adiposis dolorosa (Dercum’s disease): MRI and ultrasound appearances

http://www.clinicalradiologyonline.net/article/PIIS0009926013001992/abstract

Nerve Compression

Pain & weakness from Dercum’s Disease (adiposis dolorosa) is  often attributed to lipomas (fatty deposits) applying pressure to nerves.  This explanation is included in many definitions of the disease [1,2,3].  This theory has not been confirmed histopathologically in patients with Dercum’s Disease[4]. However, nerve compression secondary to adjacent lipomas is a recognized medical condition[5,6,7]. Most lipomas are asymptomatic, but they can cause pain when they compress nerves[8]. In fact, neuropathic pain related to subcutaneous lipoma without direct nerve compression is possible[9]. Lipomas exhibit a different cytokine profile than normal adipose, which may contribute to neural inflammation or microvascular changes[9].

1.http://misc.medscape.com/pi/android/medscapeapp/html/A1082083-business.html 
2.http://www.emergingworlds.com/ch_conditions_detail.cfm?vPageid=157 
3. http://www.genome.gov/17516629 
4.http://www.ojrd.com/content/pdf/1750-1172-7-23.pdf 
5.http://www.ncbi.nlm.nih.gov/pubmed/17320633 
6.http://www.med.nyu.edu/neurosurgery/pns/conditions/tumors/lipomas.html 
7.http://scholar.google.com/scholar?hl=en&q=lipoma+nerve+compression&btnG=&as_sdt=1%2C18&as_sdtp= 
8.http://www.patient.co.uk/doctor/Lipoma.htm
9. http://www.painmed.org/library/posters/poster-261/

Liposuction

Quality-of-life in patients with Dercum’s disease-before and after liposuction.

J Plast Surg Hand Surg. 2012 Sep;46(3-4):252-6

Authors: Hansson E, Manjer J, Svensson H, Brorson H

Dercum’s disease is characterised by obesity, pronounced pain in the adipose tissue, and a number of associated symptoms. Liposuction has been suggested as a treatment. However, the effect on quality-of-life after liposuction in Dercum’s disease has never been investigated. The objective of this study was to examine the quality-of-life in Dercum’s disease before and after liposuction. A total of 114 women fulfilling the clinical criteria of Dercum’s disease were included. Of the 114 women, 53 were operated on with liposuction and 61 were Dercum controls. In addition, 41 obese healthy women operated on with abdominoplasty were recruited as controls. Health-related quality-of-life (HRQoL) was measured with the Nottingham Health Profile (NHP) and the Psychological General Well-Being index (PGWB). The Dercum group had lower HRQoL than the abdominoplasty controls. After liposuction, a slight improvement could be seen in HRQoL in the operated patients compared with preoperatively, but it did not become as high as in the abdominoplasty patients. In conclusion, the findings could suggest that liposuction improves the quality-of-life slightly in Dercum’s disease. Nonetheless, the causality is unclear and the improvement is not big enough to warrant operation.

http://www.ncbi.nlm.nih.gov/pubmed/22909243?dopt=Abstract

Pelvic Lipomatosis

Pelvic lipomatosis is a rare disorder where fat tissue deposition is observed in spaces of the pelvic area, causing extrinsic compression of the bladder, rectum and blood vessels.

Pelvic lipomatosis might represent a variant of Dercum’s disease.

The clinical manifestations result from the extrinsic compression of the structures comprising the urinary system, the lower intestinal tract and the vascular system. Thus, the occurrence of dysuria, pollakiuria, nocturia, hematuria (less frequently), urgency, urinary incontinence and retention, besides repeated urological infections may be observed. Equally, constipation, tenesmus, diarrhea, lower limbs edema and thrombophlebitis, low back pain, suprapubic and perineal pain, painful ejaculation, epidydimitis and orchitis may be observed.

http://www.scielo.br/scielo.php?pid=S0100-39842012000300012&script=sci_arttext

Depression in Dercum’s disease and in obesity

A case control study

Emma HanssonHenry Svensson and Håkan Brorson

BMC Psychiatry 2012, 12:74 doi:10.1186/1471-244X-12-74

Published: 3 July 2012
Background

Dercum’s disease is characterised by pronounced pain in the adipose tissue and a number of associated symptoms. The condition is usually accompanied by generalised weight gain. Many of the associated symptoms could also be signs of depression. Depression in Dercum’s disease has been reported in case reports but has never been studied using an evidence-based methodology. The aim of this study was to examine the presence of depression in patients with Dercum’s disease compared to obese controls that do not experience any pain.

Patients with depression are often diagnosed with chronic pain conditions and vice versa. Both disorders activate common neurocircuitries, such as the hypothalamicpituitary-adrenal axis, limbic and paralimbic structures, ascending and descending pain
pathways, and mutual neurotransmitters, and it is therefore sometimes difficult to determine whether the pain disorder or the psychiatric condition is the primary diagnosis. Symptoms that could be attributed to depression have been described in patients in several reports to date.

Methods

A total of 111 women fulfilling the clinical criteria of Dercum’s disease were included. As controls, 40 obese healthy women were recruited. To measure depression, the Montgomery Asberg Depression Rating Scale (MADRS) was used.

Results

According to the total MADRS score, less than half of the patients were classified as having “no depression” (44%), the majority had “light” or “moderate depression” (55%) and one individual had “severe depression” in the Dercum group. In the control groups, the majority of the patients were classified as having “no depression” (85%) and a small number had “light depression” (15%). There was a statistically significant difference for the total MADRS score between the two groups (p=0.014).

Discussion

Dercum’s disease is characterised by obesity, chronic pain and other associated symptoms. Some of the associated symptoms, previously described in case reports on Dercum’s disease, include depression and symptoms associated with depression, such as asthenia, weakness, fatigue, emotional instability, mental confusion, dementia, poor sleep quality and changes in appetite. Several studies have demonstrated that there is a significant association between depression and pain, as well as between depression and obesity. Patients with depression are often diagnosed with chronic pain conditions and vice versa. Some studies have demonstrated that depression predicts obesity later in life, and other studies support that obese subjects develop depression to a greater extent than subjects with lower, “normal” body weights. Nonetheless, it is unclear whether there is a causal relationship between the three entities. The possible co-morbidity could be explained by Berkson’s bias, that is, patients with an illness might seek care more often. Thus co-morbidity could be overrepresented in a group of subjects that are, as in this study, recruited from a care setting.

In summary, it is difficult to separate chronic pain from depression. The elevated MADRS scores in the Dercum patients in this study cannot be explained by obesity alone, as the distribution of the Dercum subjects’ scores was different to that of the weight-matched control patients, and there was a statistical difference for the total score between the two groups. The lack of statistical difference for a number of the items could be explained by low power, that is, by the small number of subjects in the control group. The results suggested that the obese Dercum patients experience worse depression than obese healthy controls. As all of the Dercum patients had chronic pain whilst none of the controls had any history of chronic or present acute pain, it is unclear whether the depression is due to the Dercum’s disease per se or due to the experience of pain. Furthermore, it is unclear whether the depression or the Dercum’s disease came first. Previous research has shown that antidepressants have an effect on pain and the quality of life in patients with chronic pain and that obese patients could benefit from the treatment of
any co-existing features of depression.

An example of an associated symptom in Dercum’s disease that can also be explained by depression is poor sleep quality. Poor sleep quality can diminish an individual’s ability to cope with pain and stress and can influence the onset and course of disease. In fact, a
study on patients with chronic pain conditions demonstrated that sleeping less than 8 hours per 24 hours, especially in combination with poor sleep quality, might generate stronger reactions to pain. In addition, Affleck et al. concluded that there is a correlation between sleep quality and experienced pain intensity, as well as the ability to cope with pain, among patients with fibromyalgia. It can be speculated, therefore, that poor sleep can contribute to the onset of Dercum’s disease and the maintenance of pain. Conversely, obesity can also affect sleep quality. Obstructive sleep apnoea (OSA) and Pickwick syndrome, both of which have been previously described in Dercum’s disease, can be explained by obesity, as 50% of otherwise healthy obese women with BMI >40 have OSA and more than 29% of severely obese patients have nocturnal hypoventilation. This could explain why no difference can be seen between the Dercum patients and the control subjects in this study, as all of the subjects have similar BMIs.

Conclusion

The results indicate that the patients with Dercum’s disease are more likely to suffer from depression than controls. The relationship between Dercum’s disease, chronic pain, depression, and obesity is complex and it is not possible to separate depression and chronic pain completely. However, the results of this study indicate that patients with Dercum’s disease could suffer from worse depression than equally obese controls with no history of Dercum’s disease. This fact should be kept in mind when a treatment strategy for Dercum’s disease is selected.

Abstract: http://www.biomedcentral.com/1471-244X/12/74/abstract

Provisional PDF: http://www.biomedcentral.com/content/pdf/1471-244X-12-74.pdf

Review

Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management:  Up-to-date review of Dercums Disease research. 

http://www.ojrd.com/content/pdf/1750-1172-7-23.pdf

Adipose Tissue Dysfunction

It has been proposed that Dercum’s disease is a local defect in lipid metabolism [1]. An investigation [1] of fatty acid biosynthesis in two patients with Dercum’s disease suggested that there might be a deficit in the formation of monounsaturated fatty acids in subjects affected by the disease. However, contradictory findings were revealed in another study, comprised of 13 patients with Dercum’s disease [2]. 

Regarding adipose tissue function in Dercum’s disease, the findings are inconclusive[3].

1.http://www.lipidworld.com/content/11/1/19
2.http://www.ncbi.nlm.nih.gov/pubmed/4105769
3.http://www.ojrd.com/content/pdf/1750-1172-7-23.pdf

Endocrine Dysfunction

Originally, Dercum [1] attributed the disease to an endocrine dysfunction, as he found atrophy of the thyroid gland. Similarly, Waldorp [2] proposed that the disease is caused by hypophyseal dysfunction. Furthermore, Winkelman and Eckel [3] reviewed 16 autopsies of patients affected with Dercum’s disease and noted varying abnormalities in different endocrine organs. In their study, the pituitary gland was abnormal in eight of eleven cases examined, the thyroid in twelve cases, the sex glands in nine, the adrenal glands in three and the pancreas in two cases.
However, endocrine involvement was doubted as early as in 1933 [4] and further ruled out in 1952 [5]. In addition, present-day methods have not revealed any endocrine abnormalities [6,7,8,9,10]. For example, Piementa et al. [9] have demonstrated that hormonal secretion was normal both basally and during a 24 h period in a patient with Dercum’s disease, and no normal hormonal deficiency could be detected after an integrated pituitary stimulus test. The study suggested that there are probably no alterations of the endocrine glands, as regards the pituitary gland, the adrenal glands, the thyroid and the ovaries, in patients with Dercum’s disease.

An endocrine dysfunction as the aetiology of Dercum’s disease has little support in the modern literature.[11]

http://www.ojrd.com/content/pdf/1750-1172-7-23.pdf

1. Dercum FX: Three 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. 2. Waldorp NW: An original clinical interpretation of Dercum’s disease (adiposis dolorosa). Endocrinology 1924, 8:51–60. 3. Winkelman N, Eckel JL: Adiposis dolorosa (Dercum’s disease): a clinicopathologcal study. JAMA 1925, 85:1935–1939. 4. Rosenberg B, Hurwitz A, Hermann H: Decum’s disease with unusual retroperitoneal and paravesical fatty infiltration. Surgery 1963, 54:451–455. 5. Steiger WA, Litvin H, Lasche EM, Durant TM: Adiposis dolorsa (Dercum’s disease). N Engl J Med 1952, 247:393–396. 6. Blomstrand R, Juhlin L, Nordenstam H, Ohlsson R, Werner B, Engstrom J: Adiposis dolorosa associated with defects of lipid metabolism. Acta Derm Venereol 1971, 51:243–250. 7. Jensen JJ, Kiilerich S: A case of adiposis dolorosa–Dercum’s disease. Ugeskr Laeger 1991, 153:3564. 8. Tiesmeier J, Warnecke H, Schuppert F: An uncommon cause of recurrent abdominal pain in a 63-year-old obese woman. Dtsch Med Wochenschr 2006, 131:434–437. 9. Pimenta WP, Paula FJ, Dick-de-Paula I, Piccinato CE, Monteiro CM, Brandao-Neto J, Kettelhut IC, Foss MC: Hormonal and metabolic study of a case of adiposis dolorosa (Dercum’s disease). Braz J Med Biol Res 1992, 25:889–893. 10. Palmer ED: Dercum’s disease: adiposis dolorosa. Am Fam Physician 1981, 24:155–157. 11. Hansson E, Svensson H, Brorson H: Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic. methods, classification and management Orphanet Journal of Rare Diseases 2012, 7:23 doi:10.1186/1750-1172-7-23

Nervous System Dysfunction

It has been suggested that the autonomous nervous system mediates pain in Dercum’s disease[1]. The theory is supported in that, even though the sympathetic nervous system is efferent, sympathectomy sometimes relieves pain in neuropathic pain, where evidence of damage to neural structures exists [2]. This has been explained by the formation of abnormal connections between autonomic and sensory nerves in the periphery and, as a consequence, abnormal autonomic signalling to the spinal column might activate pain fibres[1]. However, in conditions where neural damage cannot be found, the effect on pain might be caused by the placebo response [3]. Moreover, patients with Dercum’s disease could have increased sympathetic activity induced by pain. This theory is supported by a study where a patient with Dercum’s disease did not have any vasoconstrictor response to arm and leg lowering. A normalised vasoconstrictor response could be created by lidocaine infusion that is thought to decrease the local or central sympathetic vasoconstrictor tone [4]. Furthermore, visceral pain may be generated by the autonomic nervous system, and factors that induce visceral pain could also have the ability to induce pain in the adipose tissue. Examples of such factors are anoxemia, formation and accumulation of pain-producing substances, traction or compression of vessels, inflammatory states, and necrosis [1]. 

Nonetheless, any substantial evidence of nervous system dysfunction has never been found in Dercum’s disease and is hence merely a theory[5].

1.Dalziel K: The nervous system and adipose tissue. Clin Dermatol 1989, 7:62–77  2.Furlan AD, Lui PW, Mailis A: Chemical sympathectomy for neuropathic pain: does it work? Case report and systematic literature review. Clin J Pain 2001, 17:327–336.  3.Ochoa JL: Truths, errors, and lies around “reflex sympathetic dystrophy” and “complex regional pain syndrome”. J Neurol 1999, 246:875–879.  4.Skagen K, Petersen P, Kastrup J, Norgaard T: The regulation of subcutaneous blood flow in patient with Dercum’s disease. Acta Derm Venereol 1986, 66:337–339.  5.Hansson E, Svensson H, Brorson H: Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic. methods, classification and management Orphanet Journal of Rare Diseases 2012, 7:23 doi:10.1186/1750-1172-7-23

Trauma

Two cases of trauma-induced [1,2] Dercum’s disease have been described. The first patient developed a painful fatty tumour, which was very sensitive to pressure and gave rise to much spontaneous pain, after falling on a stone pavement. The painful adipose tissue lingered for a year after the disappearance of the bruises [2]. In the second case, the patient fell down a tree and landed on his right shoulder one year before the onset of Dercum’s disease. No fracture could be detected. One year after the accident, a painful adipose tissue tumour started to grow on his right shoulder. Five years after the injury, an x-ray of the painful shoulder was performed and a humeral fracture that appeared pathological was found. However, the origin of the pathological fracture is unclear [1].

All in all, cases of trauma induced Dercum’s disease seem to occur. However, in some of the cases reported in the literature the evidence is circumstantial[3].

1.Margherita G: Considerations on a case of post-traumatic adiposis dolorosa associated with a pathologic fracture. Rass Neuropsichiatr 1964, 18:211–218.  2.Hall JH, Walbrach CE: Adiposis dolorosa with report of three cases. Am J Med Sci 1904, 128:218–322.  3.Hansson E, Svensson H, Brorson H: Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic. methods, classification and management Orphanet Journal of Rare Diseases 2012, 7:23 doi:10.1186/1750-1172-7-23

Prognosis

In a study with a five year follow-up, the average pain was relatively constant over five years. Some patients experienced less pain after five years and some more. The pain in Dercum’s disease seems to be relatively constant over time.

http://www.ojrd.com/content/pdf/1750-1172-7-23.pdf

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