Deoxycholic Acid for Dercum Disease: Repurposing a Cosmetic Agent to Treat a Rare Disease.

Dercum disease is a rare condition characterized by multiple painful fatty tumors distributed throughout the body. There currently are no US Food and Drug Administration-approved treatments for Dercum disease, and the treatments tried have shown little to no efficacy, leaving many patients with a profoundly negative impact on quality of life. We present a case series of 3 patients who were diagnosed with Dercum disease and were treated with deoxycholic acid (DCA), a therapy approved for adipolysis of submental fat. The patients experienced a reduction in tumor size with radiographic evidence as well as a notable reduction in symptoms.

Trauma-induced adiposis dolorosa (Dercum's disease).

We present a case of a 39-year-old man who presented with chronic bilateral upper extremity pain associated with innumerable angiomyolipomas that developed 5 years after a motor vehicle accident involving his upper extremities. Our case notes the rare nature of painful adipose tissue deposits and the diagnostic challenges.

Low-Dose d-Amphetamine Induced Regression of Liver Fat Deposits in Dercum Disease.

BACKGROUND: Dercum disease is a rare disorder of painful subcutaneous adipose tissue masses typically presenting as a constellation of signs and symptoms affecting most organs, including slow lymphatic flow and fatty liver. METHOD: The University of Arizona Institutional Review Board considered this report exempt after patient consent. Multislice, multisequence magnetic resonance imaging (MRI) of the abdomen and pelvis was performed prior to and after d-amphetamine, with and without intravenous gadolinium. RESULTS: Initial MRI demonstrated hepatic steatosis in Case 1; Case 2 had 2-subcentimeter lipid foci within the liver. Initiation of 10-20 mg d-amphetamine decreased liver lipid deposition from 16% to 4% in Case 1 and resolved fat deposits in Case 2 after ~1 year. CONCLUSION: There is a dire need for novel treatment options for nonalcoholic fatty liver disease to prevent progression to cirrhosis. Reduction of liver fat by d-amphetamine suggests a potential therapeutic role in nonalcoholic fatty liver disease.

A successful case of pain management using metformin in a patient with adiposis dolorosa.

In this case report, we describe a patient with Dercum's disease who was successfully managed with metformin. The administration of metformin reduced pain intensity from 9/10 to 3/10 and favorably affected the profile of inflammatory cytokines (i.e., TNF a, IL-1ß, IL-6, and IL-10), adipokines (i.e., adiponectin, leptin, and resistin), and ß-endorphin. Because each variable was affected moderately by the drug, in the range of 20 - 30%, it follows that these effects are additive, i.e., they act independently of each other. However, taking into account advances in the pharmacology of metformin, it seems that other phenomena, such as modulation of synaptic plasticity, activation of microglia, and autophagy of the afferents supplying painful lipomas should be taken into consideration. Nonetheless, metformin deserves further exploration in the biology of pain.

Treatment of pain in Dercum's disease with Lidoderm (lidocaine 5% patch): a case report.

INTRODUCTION: Dercum's disease is a rare disorder characterized by multiple painful subcutaneous lipomas on the trunk and extremities. It most commonly occurs in obese, postmenopausal women. The pain associated with this condition is postulated to arise from enlarging lipomas producing pressure on peripheral nerves, thereby initiating pain and sometimes paresthesias. Treatment has been challenging due to the rarity of this condition. CASE: A patient with Dercum's disease successfully treated with transdermal lidocaine 5% patches. The patient's pain was initially rated as an 8/10. At follow-up examination after 1 month, the patient rated her pain as 3/10--a >60% reduction in pain; this pain reduction persisted at subsequent 1-month follow-up intervals. CONCLUSION: Current therapeutic options in the treatment of Dercum's disease have proven either ineffective or cumbersome. The use of transdermal lidocaine is a safe and non-invasive treatment modality that has been efficacious in alternate forms. The use of this medication might prove preferable to more invasive or risky treatment and warrants further investigation.

[An uncommon cause of recurrent abdominal pain in a 63-year-old obese woman]. / Ungewöhnlicher Grund für rezidivierende Bauchschmerzen bei einer adipösen 63-jährigen Patientin.

HISTORY AND CLINICAL FINDINGS: A 63-year-old woman was admitted because of abdominal pain for 12 months, associated with an increasing abdominal distension. One month before a gastroscopy had revealed mild gastritis. The taking of proton pump inhibitors did not improve her symptoms. There was no relevant neurological or psychiatric past history. INVESTIGATIONS: Ultrasound and laboratory tests demonstrated a fatty liver and increased serum levels of lipoproteins. Magnetic resonance imaging revealed an increase in subcutaneous and intraperitoneal adipose tissue. Endoscopy did not show any additional significant findings. DIAGNOSIS AND TREATMENT: The patient had adiposis dolorosa. Initially she was given daily a combination of mexiletine 400 mg and 75 mg Amitriptyline. After a few days the ailment decreased and had disappeared completely after one week. Three months later the dosage was reduced to 100 mg mexiletine and 25 mg Amitriptyline daily. After another two months all medication was discontinued. At follow-up after 12 months the symptoms had not recurred. CONCLUSION: This case demonstrates that abdominal pain is not always caused by diseases of the visceral hollow organs. It must also be borne in mind that abdominal pain may be associated with adipose tissue, as is the case in adiposis dolorosa. The disease can be successfully treated, as in this case, using a combination of mexiletine and Amitriptyline.

[Painful lipoma?]. / Schmerzhafte Lipome?!

The adiposis dolorosa (Dercum's disease) was described already in 1892. Many questions about its physiopathological mechanism are still unresolved. Presentation of a patient with this disease is reported, followed by a brief review of the literature, where the pathogenesis, the differential diagnosis and the therapeutical approaches of this syndrome are especially pointed out.

Dercum's disease (adiposis dolorosa).

Dercum's disease (adiposis dolorosa) is a rare condition characterized by progressively painful fatty deposits, usually, in menopausal women with obesity, asthenia and mental phenomena. We report a case of a 48-year-old woman with recurrent neck swelling and pain in the neck and parotid region, and a review of management of this uncommon problem.

[Adiposis dolorosa (Dercum's disease)]. / Adiposis dolorosa (enfermedad de dercum).

Adiposis dolorosa (Dercum's disease) is a rare fat-tissue malady most frequently presenting in middle-aged women as fatty lumps in the body. Its etiology and pathogenesis isn't known. Two female patients are reported who had symptomatic adiposis dolorosa (32 and 62-year-old).

[A case of adiposis dolorosa--Dercum's disease]. / Et tilfaelde af adiposis dolorosa--morbus Dercum.

Adiposis dolorosa or Dercum's disease consists of a painful progressive localized state of obesity with four cardinal symptoms: a) painful circumscribed or diffuse fatty deposits, b) generalized obesity in women usually of menopausal age, c) asthenia, weakness and frequently tendency to fatigue and d) mental phenomena including emotional instability, depression, epilepsy, mental confusion and true dementia. Only a few cases in men have been described. The pain may be treated with intravenous administration of lignocaine or oral mexitil while no causal treatment is known. An illustrative case is reported.

Juxta-articular adiposis dolorosa associated with rheumatoid arthritis. Report of 2 cases with good response to local corticosteroid injection.

Juxta-articular adiposis dolorosa, i.e., painful fatty deposits around the joint may occur in postmenopausal obese women. The association of the syndrome with osteoarthritis has been described. The present report describes 2 women suffering from rheumatoid arthritis with painful fatty deposits around the knees. Both responded to local injection of corticosteroid. The possible pathogenesis of the source of pain is discussed.