Maladie de Dercum : une complication grave au cours d'une maladie rare. À propos d'un cas

Haddad, D.; Athmani, B.; Costa, Ana; Cartier, S.

doi:10.1016/j.anplas.2004.12.007

Cited by 9 publications

(4 citation statements)

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“…Abdominal pain as a main symptom of the disease [17] and arthralgia as a presenting symptom of Dercum’s disease [18] were reported in rare cases. A patient with Dercum’s disease complicated by steato-cutaneous necrosis and septic shock was reported by Haddad et al [19]. Kawale et al [20] described a patient with fatty lumps of the scalp with severe headache.…”

Section: Clinical Presentationmentioning

confidence: 99%

Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management

Kucharz¹,

Kopeć-Mędrek²,

Kramza³

et al. 2019

Reumatologia

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Dercum's disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful subcutaneous adipose tissue deposits with various localization over the body. The deposits occur histologically as lipomas and are associated with overweight or obesity and a variety of psychiatric disturbances (anxiety, depression, sleep disturbances). Classification of Dercum's disease is related to size and location of adipose nodules (generalized diffuse, generalized nodular, localized nodular and juxta-articular forms). Diagnosis in based on clinical presentation and exclusion of a number of other disorders associated with lipomas. There is no generally accepted management of the patients. Liposuction or lidocaine application has been reported successful in some cases. Other therapeutic methods have been reported but their effectiveness is based on anecdotal descriptions only, and were not confirmed in clinical trials.

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Section: Clinical Presentationmentioning

confidence: 99%

Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management

Kucharz¹,

Kopeć-Mędrek²,

Kramza³

et al. 2019

Reumatologia

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“…[Haddad et al 30] reported a case of septic shock secondary to a steatocutanous necrosis in a lipoma in a patient with Dercum’s disease. The patient had a BMI of 46.4 kg/m 2 and adipose tumours on the abdomen and on the thighs and arms.…”

Section: Definition and Clinical Descriptionmentioning

confidence: 99%

Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management

Hansson

Svensson

Brorson

2012

Orphanet J Rare Dis

105

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Definition and clinical picture: We propose the minimal definition of Dercum's disease to be generalised overweight or obesity in combination with painful adipose tissue. The associated symptoms in Dercum's disease include fatty deposits, easy bruisability, sleep disturbances, impaired memory, depression, difficulty concentrating, anxiety, rapid heartbeat, shortness of breath, diabetes, bloating, constipation, fatigue, weakness and joint aches. Classification:We suggest that Dercum's disease is classified into: I. Generalised diffuse form A form with diffusely widespread painful adipose tissue without clear lipomas, II. Generalised nodular form -a form with general pain in adipose tissue and intense pain in and around multiple lipomas, and III. Localised nodular form -a form with pain in and around multiple lipomas IV. Juxtaarticular form -a form with solitary deposits of excess fat for example at the medial aspect of the knee.Epidemiology: Dercum's disease most commonly appears between the ages of 35 and 50 years and is five to thirty times more common in women than in men. The prevalence of Dercum's disease has not yet been exactly established.Aetiology: Proposed, but unconfirmed aetiologies include: nervous system dysfunction, mechanical pressure on nerves, adipose tissue dysfunction and trauma. Diagnosis and diagnostic methods: Diagnosis is based on clinical criteria and should be made by systematic physical examination and thorough exclusion of differential diagnoses. Advisably, the diagnosis should be made by a physician with a broad experience of patients with painful conditions and knowledge of family medicine, internal medicine or pain management. The diagnosis should only be made when the differential diagnoses have been excluded. Differential diagnosis: Differential diagnoses include: fibromyalgia, lipoedema, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis, familial multiple lipomatosis, and adipose tissue tumours. Genetic counselling: The majority of the cases of Dercum's disease occur sporadically. A to G mutation at position A8344 of mitochondrial DNA cannot be detected in patients with Dercum's disease. HLA (human leukocyte antigen) typing has not revealed any correlation between typical antigens and the presence of the condition. Management and treatment: The following treatments have lead to some pain reduction in patients with Dercum's disease: Liposuction, analgesics, lidocaine, methotrexate and infliximab, interferon α-2b, corticosteroids, calcium-channel modulators and rapid cycling hypobaric pressure. As none of the treatments have led to long lasting complete pain reduction and revolutionary results, we propose that Dercum's disease should be treated in multidisciplinary teams specialised in chronic pain. Prognosis: The pain in Dercum's disease seems to be relatively constant over time.

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“…There are no cases in literature regarding Dercum's disease and lipomatous compression of peripheral vasculature. Thus far, reported complications of the disease include mastalgia, 13 steatocutaneous necrosis of a lipoma resulting in septic shock, 14 and a lipomatous hypertrophy of the interatrial septum. 15 …”

Section: Discussionmentioning

confidence: 99%

Femoral canal lipoma presenting as chronic venous insufficiency

Teh,

Picazo Pineda,

Sieunarine

2023

Journal of Vascular Surgery Cases, Innovations and Techniques

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Maladie de Dercum : une complication grave au cours d'une maladie rare. À propos d'un cas

Cited by 9 publications

References 10 publications

Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management

Dercum’s disease (adiposis dolorosa): a review of clinical presentation and management

Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management

Femoral canal lipoma presenting as chronic venous insufficiency

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