Dercum’s disease

Dercum’s disease, or adiposis dolorosa, is a rare, chronic condition characterised by painful subcutaneous adipose deposits, often accompanied by obesity and neuropsychiatric disturbances. The hallmark symptom is persistent, often debilitating pain in subcutaneous fat, variably described as burning, aching, or stabbing. These symptoms can be generalised or localised to palpable lipomas, which may occur across the trunk, proximal limbs, buttocks, or periarticular regions.

Clinical Epidemiology

• Age of Onset: Typically 35–50 years, though cases reported outside this range.
• Sex Ratio: Females affected 5–30× more frequently than males.
• Prevalence: Undefined in the U.S.; listed as a rare disease (<200,000 affected) by NORD Population studies are lacking.
• Geographic Clusters: Notably reported in Sweden (~10,000 sufferers)

Diagnosis

Diagnosis is clinical and by exclusion. Laboratory tests may reveal minor elevations in ESR or cholesterol, but no definitive biomarkers exist. Imaging (MRI, ultrasound) may detect superficial lipomatous changes not readily apparent on exam. Histologically, lesions resemble angiolipomas or inflamed lipomas with lymphocytic infiltrates.

Treatment Overview

A. Symptom‑Directed and Pain Control

• Analgesics/NSAIDs: Mixed responses—up to 89 % reported pain relief with NSAIDs and even higher with opioids, though most cases are refractory
• Lidocaine (topical/intravenous): Intralesional procaine historically effective; 5 % patch or cream relief; IV infusions can yield relief lasting hours to months
• Systemic/Local Corticosteroids: Variable responses; intralesional methylprednisolone showed notable improvement in some cases

B. Surgical Interventions

• Liposuction/Excision: Often provides transient pain relief but recurrences are common
• Techniques like tumescent microcannular liposuction have been reported in isolated case series

C. Immunomodulatory and Adjunct Therapies

• Methotrexate, Infliximab, Interferon α‑2b: Case reports show partial relief; immune-mediated mechanism hypothesised
• Calcium‑channel modulators & CVAC hypobaric therapy: Cyclic hypobaric conditioning offered short-term pain reduction in small pilot study

Overall, management is multidisciplinary, aimed at symptom control and improved quality of life

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History of Dercum’s disease

1888, Francis Xavier Dercum (1856–1931), professor of nervous diseases at the University of Pennsylvania, first described the syndrome at a local medical meeting. He presented a 51-year-old woman with subcutaneous fat deposits, asthenia, and nervous symptoms. He published his findings of “A Subcutaneous connective tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema“ in the Boston Medical and Surgical Journal

Dercum emphasized that the condition was not merely obesity or myxoedema, stating:

Evidently the disease is not simple obesity… All of these cases lack the peculiar physiognomy… It would seem then, that we have here to deal with a connective tissue dystrophy… associated with symptoms suggestive of an irregular and fugitive irritation of nerve-trunks—possibly a neuritis

Dercum 1888

1892, Dercum formally proposed the term Adiposis Dolorosa in his seminal article “Three cases of hitherto unclassified affection resembling in its grosser aspects obesity“. He documented three further patients and underscored the nervous system’s role in the condition.

1901, French physicians Roux and Vitaut described Maladie de Dercum (adiposis dolorosa) in Revue Neurologique as a constellation of four cardinal features: painful lipomas, obesity, fatigue, and neuropsychiatric disturbances. Their thesis cemented adiposis dolorosa as a discrete clinical entity in European neurology.

1930s, pain relief was first attempted via intralesional procaine (Novocain), with early reports showing success in a small number of cases.

1980s marked a key shift in pain management, with reports of intravenous lidocaine offering temporary relief ranging from hours to months. This led to the hypothesis of altered peripheral nerve signalling or sympathetic dysregulation as underlying mechanisms.

1996, Swedish clinicians Håkan Brorson and Peter Fagher published new insights on immune and lymphatic involvement, including reports of Dercum’s clusters in Sweden suggesting a potentially under-recognized prevalence.

2012, Hansson, Svensson, and Brorson proposed a minimal definition and structured classification system in the Orphanet Journal of Rare Diseases. They recommended two diagnostic pillars: generalized overweight or obesity, and chronic pain in adipose tissue. The authors also mapped subtypes and noted a lack of clear laboratory or imaging criteria.

2020s, CVAC (Cyclic Variations in Adaptive Conditioning) and systemic immunomodulators (methotrexate, infliximab, interferon α-2b) have been trialled in small series, yet no standardised, evidence-based treatment protocol exists. Despite WHO recognition in ICD-10 and inclusion by NORD and Orphanet, Dercum’s disease remains poorly understood and underdiagnosed.

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Associated Persons

• Francis Xavier Dercum (1856–1931): American neurologist; first described adiposis dolorosa, coined the eponym, and documented early case series

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References

Historical references

• Dercum FX. A Subcutaneous Connective Tissue Dystrophy of the Arms and Back, Associated with Symptoms Resembling MYXŒDEMA. The University Medical Magazine, Philadelphia
  1888; 1: 140-151
• Dercum FX. A Subcutaneous connective tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema. Boston Medical and Surgical Journal 1888; 119(14): 329
• Dercum FX. Three cases of hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special nervous symptoms, adiposis dolorosa. American Journal of the Medical Sciences 1892; 104(5): 521-535
• Roux J, Vitaut L. Maladie de Dercum (adiposis dolorosa). Revue neurologique. 1901; 9: 881-888.

Eponymous term review

• Hansson E, Svensson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012 Apr 30;7:23. 
• Kucharz EJ, Kopeć-Mędrek M, Kramza J, Chrzanowska M, Kotyla P. Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management. Reumatologia. 2019;57(5):281-287.
• Asdourian MS, Shah N, Jacoby TV, Tiao J, Fedeles F. Dercum’s disease: A retrospective cohort study. J Am Acad Dermatol. 2023 Mar;88(3):738-741. 
• Cook JC, Gross GP. Adiposis Dolorosa. 2023 Apr 10. In: StatPearls [Internet].