Dercum’s Disease: A Case Report of a Patient Having Both Type 1 and Type 2 Dercum’s Disease

Abstract

Dercum’s disease, or adiposis dolorosa, is a rare disorder which consists of multiple, painful lipomas within the subcutaneous tissue and has a distribution mainly in the abdomen and extremities. Dercum’s disease can be defined as in combination with chronic painful adipose tissue. Although the etiology of Dercum’s disease is not clear, it is thought to be a combination of a neurological and endocrine disorder. Treatment for this disease is centered at managing pain. Although there is no standard of care for managing pain, there are different pain management regimes that are promising.

Presentation of multiple painful subcutaneous nodules: Dercum’s disease, a rare variant of lipoma

“…While being described as a ‘complex, incompletely understood disorder of subcutaneous fat’,3 Dercum’s disease (also known as Adiposis Dolorosa) is characterised by the development of multiple and usually painful lipomas in the adipose tissue of the body 1. These lipomas are typically distributed around the trunk and extremities and can be accompanied by non-specific symptoms such as fatigue, weakness mental disturbances like depression and anxiety 1.…”

“…While being described as a ‘complex, incompletely understood disorder of subcutaneous fat’,3 Dercum’s disease (also known as Adiposis Dolorosa) is characterised by the development of multiple and usually painful lipomas in the adipose tissue of the body 1. These lipomas are typically distributed around the trunk and extremities and can be accompanied by non-specific symptoms such as fatigue, weakness mental disturbances like depression and anxiety 1. Additionally, they tend to have a female predilection and there are reports of it carrying an autosomal dominant mode of inheritance, noted in both the literature and as outlined on the National Organization for Rare Disorders 2 4…”

“…Dercum’s disease (also known as Adiposis Dolorosa) has been defined in the literature as the development of multiple (typically painful) lipomas in the adipose tissue that is often accompanied by a myriad of other non-specific symptoms such as mental disturbance, depression, weakness and fatigue 1. Dercum’s disease is listed on the National Organization for Rare Disorders, and while the exact epidemiology of the disease is poorly known, there has shown to be a female predilection in combination with obesity and those aged 35–50 years old, with no specific ethnicity found to be of increased risk noted in the literature (but this has not been exclusively looked into) 1 2. Further studies and case reports would need to be evaluated to establish if such a risk exists.…”

“…Diagnosis is typically clinical, although due to its hyperechoic, ‘blush-like’ nodular lesions depicted on Ultrasound sonography (USS) and MRI, many cases are first suspected by radiologists 3. Management is typically a combination of pain management and optimising comorbidities, with surgical interventions such as liposuction and lipectomies providing short-term pain relief 1…”

Lurking Under the Surface: Dercum’s Disease

“…The pain along the sites of excised lesions had improved but she continues to have significant tenderness along the areas of new masses. The patient's case was further reviewed and labeled with the diagnosis of Dercum’s disease - type 2 [ 3 ]. The patient again was seen in the surgery clinic at our institution with subsequent referral to endocrinology for further evaluation and to the pain clinic for multimodality treatment.…”