Low dose d-amphetamine induced regression of liver fat deposits in Dercum disease

Clinical Significance
• D-amphetamine anecdotally improves the painful fat/lymphatic disorder, Dercum’s disease.
• Two patients with Dercum’s disease were treated with d-amphetamine to improve lymphatic function through the sympathetic nervous system.
• Both patients lost weight, and fatty liver in a man and liver lipomas in a woman resolved in less than a year on ≤20 mg of D-amphetamine daily.
• Low dose d-amphetamine may improve fatty liver and Dercum’s disease.
Background/Aim
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 before and after d-amphetamine, with and without intravenous gadolinium.
Results
Initial MRI demonstrated hepatic steatosis in Case 1; Case 2 had two-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 ~one year.
Conclusion
There is a dire need for novel treatment options for non-alcoholic fatty liver disease to prevent progression to cirrhosis. Reduction of liver fat by d-amphetamine suggests a potential therapeutic role in non-alcoholic fatty liver disease.

S. Ghazala, J. Bilal, E. Ross, B. Kalb, I.B Riaz, K.L. Herbst

http://www.amjmed.com/article/S0002-9343(18)30015-9/abstract

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Adipocytic Tumors

Adipocytic tumors form a significant proportion of all benign and malignant musculoskeletal masses. This chapter focuses on the pathophysiology and clinical and imaging characteristics of the different benign and malignant subtypes presented according to the current WHO classification (Fletcher et al., World health organisation classification of tumors of soft tissue and bone, 4th edn. IARC Press, Lyon, 2013). MRI is emphasized throughout as this imaging technique, in particular, is most useful in this tumor group due to its ability to specifically characterize normal adipose tissue.

https://link.springer.com/chapter/10.1007/978-3-319-46679-8_12

Widespread painful nodules in a patient with rheumatoid arthritis

A 60-year-old woman affected by rheumatoid arthritis (RA) reported a 12-month history of progressive growth of multiple, painful and symmetrically distributed subcutaneous nodules affecting forearms, thighs and lower back. In the last months the size and the number of nodularities markedly increased, most recently with the appearance of a new lump every other week. These painful swellings had first appeared in the left forearm, with a single lesion progressively increasing in size, but later on similar lesions occurred in the other arm. On examination, multiple, tender and movable subcutaneous nodules with an increased consistency of sizes varying from 0.5–1.8cm were present on both forearms, thighs and lower back. The overlying skin did not show any abnormality but pain was evoked upon palpation with light pressure. The patient described the pain as burning, dull and persistent, slightly reduced during the night, ranging from 5 to 8/10 on a numeric rating scale in the last week. The histologic examination of a nodule of the left forearm showed only adipose tissue. Dercum’s disease (adiposis dolorosa) is a rare disorder of unknown etiology, first described in 1888, characterized by the development of multiple painful nodules of adipose tissue on the trunk and proximal parts of the extremities, most commonly affecting obese postmenopausal women.

http://www.ejinme.com/retrieve/pii/S095362051730170X
Devis Benfaremo, Michele Maria Luchetti, Armando Gabrielli
Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy
http://www.ejinme.com/article/S0953-6205(17)30170-X/fulltext

Prominent neurological involvement in Dercum disease

Prominent neurological involvement in Dercum disease

(Abstract not provided, article behind paywall)
http://link.springer.com/article/10.1007/s00415-017-8415-1

 

Case Report

52 year old obese postmenopausal female presented with complaints of multiple painful lumps over the legs and thighs of 2 years days duration. Clinical examination revealed multiple tender plaques and nodules over both the legs and thighs of different sizes. Biopsy suggestive of lipoma. These features were diagnostic of a rare lipomatosis, dercum’s disease. https://www.omicsgroup.org/journals/dercums-diseaseadiposis-dolorosa-2376-0427-1000252.pdf

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