Painful Adipose Tissue Disorder: A Systematic Review of Interventions for Dercum's Disease.

Abstract

Dercum's disease (DD) is a rare connective tissue disorder characterized by multiple painful progressive adipose deposits. No definitive treatment for DD exists; therefore, the purpose of this study was to aggregate the current treatment options and outcomes in the literature to guide clinical decision-making regarding intervention. A comprehensive search was conducted across the selected databases, PubMed, Web of Science, and clinicaltrials.gov , for studies including at least 3 patients with DD without concurrent lipedema. Given the rarity of the pathology, descriptive analysis was primarily utilized if there were no homogenous endpoints. A total of 837 studies were extracted from the databases, of which 10 met the inclusion criteria. The majority of studied interventions included liposuction (4 studies), followed by dermolipectomy, transcutaneous electrical stimulation, cyclic pneumatic compression, and injections with either Tapencarium (RZL-012) or deoxycholic acid, and prostigmine and aminoacetic acid. One hundred ninety patients underwent these intervention types, of which 95.3% were female, with a mean age of 51.7 years and an average body mass index of 34.0 kg/m 2 . Four intervention types (liposuction, pneumatic compression, dermolipectomy, and electrical stimulation) included pain assessments utilizing a visual analog scale. All interventions demonstrated a decrease in pain scores, although the effect of electrical stimulation did not reach significance. Of the 2 studies assessing liposuction, there were significantly decreased visual analog scale scores at the following time points: 3 and 6 months, and 3 and 5 years, with nonsignificant reductions at 1 and 2 years. Dercum's disease is a rare pathology that continues to cause pain and distress in a primarily middle-aged, obese female population. Our systematic review calls for further investigation for optimal management of an often debilitating disease.