Lurking Under the Surface: Dercum’s Disease

Abstract

Adiposis dolorosa, also known as Dercum's disease, is a rare disorder characterized by debilitating painful lipomas throughout the body. The prevalence and etiology of Dercum's disease are unknown as mentioned in the National Organization of Rare Disorders. We present an interesting case of Dercum's disease in a 53year-old female who initially presented with a six-week history of painful subcutaneous masses. Ultrasound findings were suggestive of lipomas, however, her symptoms were debilitating beyond that of benign lipomas. She then represented with a rapidly increasing number of soft tissue masses manifesting throughout her body, as well as significant diffuse pain concentrating around these lesions within a short period of time following her initial presentation. The patient underwent surgical excision of a select number of these masses, with histopathology consistent with lipomas. Most cases of Dercum's disease are sporadic, and no guidelines exist regarding the treatment of the disease. Due to the rarity of this condition, in conjunction with simple lipomas typically presenting as painless masses, many patients may be misdiagnosed and neglected due to being falsely labeled as pain seeking or having their symptoms attributed to psychological disorders. Management, therefore, is complex and currently consists of a multidisciplinary approach employing multimodal treatments, including pain control, surgical excision, and psychotherapy. Although this condition has been described in the literature for over 100 years, there have been minimal advancements towards alleviating the suffering of these patients. We aimed to unearth and bring to light the reality and the suffering experienced by patients with Decrum's disease.

Dercum's Disease: A Rare Disease of Painful Fatty Lumps

“…Since there are currently no known biomarkers linked to Dercum's disease, the final diagnosis is determined based on the results of the histology. The histopathology results of tissue specimens are consistent with those of fatty connective tissues and are identical to lipomas [ 8 ], as seen in the histopathology results of our patient (Figure 2 ). Radiological investigations also aid in the detection of subcutaneous nodules [ 9 ].…”

Lipomas: genetic basis of common skin lesions and their occurrence in rare diseases

“…Treatment includes surgical procedures such as excision of lipomas or liposuction, as well as various pharmacological agents [63]. Excision of the lesions is often ineffective and results in only a partial improvement due to the appearance of new lipomas [69], while the long-term efficacy of liposuction is difficult to assess because of the simultaneous use of different forms of treatment by patients [67]. Pharmacological treatment includes non-steroidal anti-inflammatory drugs (which are not very effective), narcotic drugs, lidocaine (both systemic and in the form of a patch), interferon alpha-2b, corticosteroids, and calcium channel modulators, such as pregabalin and oxcarbazepine [63].…”

An unusual presentation of Dercum’s disease to the emergency department

“…The prevalence is unknown but is thought to occur commonly in obese woman during adulthood. It is classified into four distinct varieties: type I—is where there is widespread painful adipose tissue with no apparent lipomas; type II—is the generalized nodular form; type III—is the localized nodular form and type IV—is the juxta articular form [ 1 ].…”