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Review
. 2021 Mar 21;10(6):1292.
doi: 10.3390/jcm10061292.

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

Madleen Lemaitre  1   2 , Sebastien Aubert  3 , Benjamin Chevalier  1 , Arnaud Jannin  1 , Julien Bourry  1 , Gaetan Prévost  4 , Herve Lefebvre  4 , Marie-Christine Vantyghem  1   2   5
Affiliations
Review

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

Madleen Lemaitre et al. J Clin Med. .

Abstract

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.

Keywords: Dercum’s disease; Roch-Leri mesosomatous lipomatosis; adipose tissue; lipodystrophy; lipomatosis.

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Conflict of interest statement

The authors declare that they have no conflict of interest concerning the topic of the study.

Figures

Figure 1
Figure 1
Dercum’s lipomatosis: Multicicatricial abdomen linked to multiple excisions and multiple encapsulated lipomas, associated with pain in a woman with Dercum’s disease progressing in a context of obesity.
Figure 2
Figure 2
Roch-Leri mesosomatous lipomatosis. (A) Lipomas distributed over the abdomen, painless and non-tender to palpation, with progressive onset of several years. (B) Multiple encapsulated lipomas, painless, disseminated on the thighs with an ecchymotic tendency.
See this image and copyright information in PMC

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