Introduction

Dercum’s Disease (DD), also known as adiposis dolorosa, is a rare condition characterized by the presence of multiple painful, fatty and benign (non-cancerous) tumors (lipomas) that can grow anywhere in the subcutaneous fat of the body. In Dercum’s disease, lipomas typically occur on the trunk, arm and leg. 

Dercum’s disease has a higher incidence in women than men. Some publications show the incidence in women may range from anywhere between five to 30 times more than in men. Although the disease can manifest in patients of any age, studies reveal that the median age of onset is 34.¹ It is extremely rare in children, with symptoms typically revealing itself in adulthood. Due to the rarity in pediatric and adolescent patients, symptoms such as chronic pain, fatigue and emotional concern are often considered as growth-related issues, resulting in delayed diagnosis and untimely treatment. Early diagnosis and prompt, appropriate care would not only control the symptoms but also improve the patient's quality of life.

Clinical features and diagnosis of Dercum’s disease

Dercum’s disease is characterized by the presence of four hallmark symptoms:²

• Presence of multiple painful lipomatous masses
• Generalized obesity or abnormal fat distribution
• Muscle weakness and pronounced fatigue (asthenia)
• Neuropsychiatric symptoms, including emotional instability, depressive episodes, cognitive dysfunction (such as confusion and dementia), and in some cases, seizure activity
• Some DD patients may present with swelling in different body parts, especially the hands

The diagnosis of Dercum’s disease is performed based on a physical examination and a review of the patient’s medical history. Ultrasound and magnetic resonance imaging (MRI) can also be used to diagnose Dercum’s disease.³

Dercum’s disease is a rare condition and its features often overlap with other conditions, including fibromyalgia, familial multiple lipomatosis and angiolipomatosis. In order to diagnose Dercum’s disease, medical practitioners must distinguish between conditions that present similarly to DD.

• Patients with fibromyalgia may present with symptoms of muscle pain and chronic fatigue. However, they lack the painful, fatty lipoma that is typical of a patient with Dercum’s disease
• Familial multiple lipomatosis patients have multiple benign growths of fatty tissue. However, these lipomas typically do not cause any pain
• Symptoms of angiolipomatosis include the presence of lipoma and pain, which can mimic that of Dercum’s disease

Treatment and management approaches for Dercum’s disease

While there is no cure for Dercum’s disease, various treatment methods have been developed to manage the symptoms of DD. Treatment for Dercum’s disease can be divided into four categories: Surgical interventions, pharmacologic therapies, alternative treatment and multidisciplinary care. 

• Surgical interventions: In Dercum’s disease patients, surgical intervention often involves the excision of lipomas, which may temporarily relieve pain and improve quality of life. However, this treatment is not curative, and the disease often recurs after surgery.
• Pharmacologic therapies: Various pharmacologic therapies, including traditional analgesics and
  • Traditional analgesics: The pain caused by Dercum’s Disease is usually resistant to analgesics and nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Lidocaine:  Several studies show that Lidocaine could relieve pain, but the mechanism of relief is still unknown
  • Methotrexate and infliximab: Patients receive two medicines that have already relieved the Dercum’s Disease. Infliximab’s mechanism of action is thought to be mediated by inhibition of tumor necrosis factor. Methotrexate’s mechanism is still unknown

Besides these medicines, the Interferon α-2b and Corticosteroids also been used to relieve the DD symptoms.

• Alternative treatment (CVAC): CVAC is a contactless treatment that stimulates the body to improve circulation and reduce edema through periodic low air pressure changes. Studies show that CVAC can reduce patient pain, but requires further randomize clinical trials to confirm the efficacy and safety
• Multidisciplinary care: Psychological therapy and consultation with a pain management specialist may help patients cope with long-term, severe pain. Other treatments include symptomatic treatment and supportive care

Psychosocial impact and research gaps

Dercum’s Disease can have a prolonged psychological impact on pediatric and adolescent patients. Chronic pain and fatigue may affect the patient’s school attendance, academic performance and peer relationships. These concerns may lead to isolation, low self-esteem, depression and anxiety. However, due to the lack of disease awareness, patients may not get proper treatment and emotional support.

In addition, the unpredictability and persistence of symptoms may cause frustration and a sense of helplessness among young patients, especially when peers or teachers fail to understand the nature of an invisible illness. Adolescents, in particular, are at a critical stage of emotional and social development. The presence of ongoing pain and body image concerns associated with lipomas or weight gain may contribute to embarrassment, bullying, or withdrawal from group activities. Families may also face emotional and financial stress due to repeated healthcare visits, uncertainty about the condition, and challenges in accessing appropriate care. Therefore, the psychosocial burden of Dercum’s Disease extends beyond the individual and affects the entire support system, underlining the urgent need for integrated medical and psychological support services for this age group.

Despite the heavy burden of the disease, there are currently no clear diagnostic criteria for pediatric patients, making early identification more difficult. Existing diagnostic definitions are mostly derived from adult patients and may not accurately reflect the disease characteristics of children and adolescents. Considering the rarity of this disease, current research lacks sufficient sample size and long-term studies, which limits in-depth understanding of the natural course, progression mechanism and treatment response of the disease. Moreover, the lack of pediatric-focused clinical guidelines results in inconsistent approaches to assessment and management, often relying on trial-and-error treatments. The absence of robust epidemiological data also hinders awareness campaigns and funding opportunities, perpetuating the cycle of underdiagnosis. To improve outcomes for pediatric and adolescent patients, future research should prioritize age-specific diagnostic frameworks, the development of tailored outcome measures, and the establishment of national and international patient registries.

Conclusion

Dercum’s disease is a rare chronic condition that is particularly challenging to diagnose and treat in children and adolescents. Although the disease is more common in adult women, its occurrence in young patients cannot be ignored. Early identification is crucial for alleviating pain, preventing psychosocial problems, and improving long-term prognosis. However, there is currently a lack of clear diagnostic criteria and unified treatment plans for the pediatric population, which limits timely intervention and effective management. Therefore, there is an urgent need to improve clinical awareness, promote multidisciplinary collaboration, and establish a variety of long-term follow-up studies to guide future clinical practice and scientific research for this population.