Rare Presentation of Dercum's Disease in a Child with Abnormalities in Lipoprotein
            Metabolism

Izar, Maria Cristina de Oliveira; Fonseca, Henrique Andrade Rodrigues da; França, Carolina Nunes; Machado, Valéria A.; Ferreira, Carlos Eduardo dos Santos; Fonseca, Francisco Antônio Helfenstein

doi:10.5935/abc.20180191

Cited by 3 publications

(4 citation statements)

References 12 publications

(22 reference statements)

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“…Dercum’s disease is a rare disorder appearing almost exclusively in adults, and is predominant in individuals aged 35–50 years [7, 8]. A very few cases of affected children have been published only [9]. It is predominantly a disease affecting women, and the ratio of women to men is estimated at 5–30 : 1 [4].…”

Section: Epidemiologymentioning

confidence: 99%

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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: Epidemiologymentioning

confidence: 99%

“…Izar et al [9] reported an eight-year-old girl suffering from Dercum’s disease and hyperbetalipoproteinemia as well as hyperinsulinemia.…”

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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“…The age of occurrence of the two diseases was very close around 30 years old, which is similar to that mentioned in literature for LMS [ 5 ]. In contrast, it is much younger for Dercum’s disease, which is usually considered a postmenopausal illness [ 21 ], though at least one case has been reported in a child [ 22 ]. Interestingly, this young age of occurrence in our study could argue for a gender difference related to sex hormone status, or for a genetic anomaly—at least in some patients—since in addition 11% of Dercum’s and 27% of LMS cases were apparently familial.…”

Section: Discussionmentioning

confidence: 99%

Metabolic and immunological phenotype of rare lipomatoses: Dercum’s disease and Roch-Leri mesosomatic lipomatosis

Lemaître

Chevalier

Jannin

et al. 2021

Orphanet J Rare Dis

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Context Dercum’s disease (DD) and Roch-Leri mesosomatic lipomatosis (LMS) are rare and poorly characterized diseases. The clinical presentation combines multiple lipomas, painful in DD in contrast with LMS, without lipoatrophy. Objective To identify any specific metabolic and immune phenotype of DD and LMS. Design and patients This monocentric retrospective study included 46 patients: 9 DD, 11 LMS, 18 lean and 8 obese controls. Metabolic and immunohematological characteristics of each group were compared. Results The median age of the patients was similar in the 3 groups (31 years). The number of women, and of basophils, and CD3+, CD4+ and CD8+ T lymphocytes was significantly higher in the DD versus the LMS group, without any difference of the metabolic parameters. Weight, BMI, blood pressure, gamma-GT, leptin, fasting insulin and C-peptide levels, fat mass percentage, and intra/total abdominal fat ratio were significantly higher in each lipomatosis group compared with the lean group. Compared with the lean group, the DD group had significantly higher fasting blood glucose, LDL-cholesterol, platelets, leukocytes, basophils, and a lower NK cell count, whereas the LMS group had a significantly lower rate of CD3, CD4, and CD8 lymphocytes. Compared with the obese controls, basophils remained higher in DD and T lymphocytes subpopulations lower in LMS groups. Conclusion DD and LMS show a common background of obesity and metabolic phenotype, but a distinct immunohematological profile characterized by a higher number of basophils in DD patients, an inflammatory profile that could contribute to pain. T lymphocyte depletion was present in LMS. These findings could offer specific therapeutic opportunities, especially for painful DD.

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“…On the metabolic level, the presence of larger-than-normal particles of High Density Lipoprotein (HDL)-c3 (which are less cardioprotective than HDL-2), as well as an increase in Lp-PLA2 (phospholipase A2 associated with lipoproteins) were found in one pediatric case and were associated with hyperinsulinism [ 34 ]. Other case reports have described hypercholesterolemia in adults with Dercum’s disease [ 35 , 36 ].…”

Section: Dercum’s Diseasementioning

confidence: 99%

Rare Forms of Lipomatosis: Dercum’s Disease and Roch-Leri Mesosomatous Lipomatosis

Lemaître

Aubert

Chevalier³

et al. 2021

JCM

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In contrast to obesity, which is very frequent, lipomatosis and lipodystrophy syndromes are rare diseases of adipose tissue. Lipodystrophy syndromes are characterized by metabolic abnormalities associated with partial or generalized lipoatrophy. Lipomatosis is defined by the presence of several body lipomas without lipoatrophy. Dercum’s disease (DD) and Roch-Leri mesosomatous lipomatosis (RLML) are rare and poorly characterized forms of lipomatosis. They have raised little clinical interest despite the non-negligible consequences of DD on quality of life. The main clinical presentation of these diseases includes multiple lipomas, which are painful in DD (in contrast to RLML). The two diseases are frequently associated with obesity and metabolic syndrome, with hypertension, diabetes, or dyslipidemia. The long-term course of the diseases remains poorly described. DD affects mainly women, whereas RLML mostly affects men. In both diseases lipomas are found on the back and thighs, as well as on the abdomen in DD and the forearms in RLML. The painful lipomas tend to recur after surgery in DD (in contrast to RLML). Most cases are sporadic. No specific treatment has been identified, as the pathophysiology remains unknown. Nevertheless, low-grade fat inflammation and specific abnormalities such as hyperbasophilia deserve further investigation. The aim of this review is to analyze the available literature on the topic.

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Rare Presentation of Dercum's Disease in a Child with Abnormalities in Lipoprotein Metabolism

Cited by 3 publications

References 12 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

Metabolic and immunological phenotype of rare lipomatoses: Dercum’s disease and Roch-Leri mesosomatic lipomatosis

Rare Forms of Lipomatosis: Dercum’s Disease and Roch-Leri Mesosomatous Lipomatosis

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