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Published in: Aesthetic Plastic Surgery 3/2020

Open Access 01-06-2020 | Original Article

Adipose Tissue in Multiple Symmetric Lipomatosis Shows Features of Brown/Beige Fat

Authors: Daniel Schiltz, Sebastian Tschernitz, Christine Ortner, Alexandra Anker, Silvan Klein, Oliver Felthaus, Niklas Biermann, Julia Schreml, Lukas Prantl, Stephan Schreml

Published in: Aesthetic Plastic Surgery | Issue 3/2020

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Abstract

Introduction

Multiple symmetric lipomatosis (MSL) (syn.: Launois–Bensaude Syndrome, benign symmetric lipomatosis) is a rare disease of fatty tissue. The pathophysiology of MSL still remains unclear, although several approaches have been described in order to understand it. Beside morphological characteristics and some molecular cell biological approaches, little is known about the histological and immunohistochemical characterization of adipose tissue from patients with MSL.

Methods

From the 45 patients with MSL in our database, 10 were included in the study. Fat tissue samples were collected from affected and unaffected areas. The forearm served as a control area as this area is not affected in MSL. The specimens were analyzed after selected stainings were taken (hematoxylin–eosin = HE, Elastica van Gieson, Ladewig, CD200, CIDEA, myf5, p107, Prdm16, Sca-1, syndecan, UCP1, MAC387, Glut4).

Results

In patients suffering from MSL, no macroscopic or microscopic morphological difference could be found between affected and unaffected adipose tissue in HE stainings. The majority of samples showed positivity for UCP1 (9/10 clinically affected tissues, 7/10 clinically unaffected tissues) and CD200.

Conclusion

Marker profiles support the hypothesis that affected adipose tissue derives from brown or beige adipose tissue rather than from white fat.

Level of Evidence IV

This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.​springer.​com/​00266.
Appendix
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Literature
1.
go back to reference Enzi G, Busetto L, Ceschin E, Coin A, Digito M, Pigozzo S (2002) Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study. Int J Obes Relat Metab Disord 26:253–261CrossRef Enzi G, Busetto L, Ceschin E, Coin A, Digito M, Pigozzo S (2002) Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study. Int J Obes Relat Metab Disord 26:253–261CrossRef
2.
go back to reference Kan Y, Yao P, Xin W, Chen Q, Wang J, Yue J, Zhu J (2010) Recent progress on diagnosis and treatment of benign symmetric lipomatosis. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 24:105–107PubMed Kan Y, Yao P, Xin W, Chen Q, Wang J, Yue J, Zhu J (2010) Recent progress on diagnosis and treatment of benign symmetric lipomatosis. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 24:105–107PubMed
3.
go back to reference Enzi G (1984) Multiple symmetric lipomatosis: an updated clinical report. Medicine (Baltimore) 63:56–64CrossRef Enzi G (1984) Multiple symmetric lipomatosis: an updated clinical report. Medicine (Baltimore) 63:56–64CrossRef
4.
go back to reference Schiltz D, Anker A, Ortner C, Tschernitz S, Koller M, Klein S, Felthaus O, Schreml J, Schreml S, Prantl L (2018) Multiple symmetric lipomatosis: new classification system based on the largest german patient cohort. Plast Reconstr Surg Glob Open 6:e1722CrossRef Schiltz D, Anker A, Ortner C, Tschernitz S, Koller M, Klein S, Felthaus O, Schreml J, Schreml S, Prantl L (2018) Multiple symmetric lipomatosis: new classification system based on the largest german patient cohort. Plast Reconstr Surg Glob Open 6:e1722CrossRef
5.
go back to reference Pinto CI, Carvalho PJ, Correia MM (2017) Madelung’s disease: revision of 59 surgical cases. Aesthetic Plast Surg 41:359–368CrossRef Pinto CI, Carvalho PJ, Correia MM (2017) Madelung’s disease: revision of 59 surgical cases. Aesthetic Plast Surg 41:359–368CrossRef
6.
go back to reference Plummer C, Spring PJ, Marotta R, Chin J, Taylor G, Sharpe D, Athanasou NA, Thyagarajan D, Berkovic SF (2013) Multiple symmetrical lipomatosis—a mitochondrial disorder of brown fat. Mitochondrion 13:269–276CrossRef Plummer C, Spring PJ, Marotta R, Chin J, Taylor G, Sharpe D, Athanasou NA, Thyagarajan D, Berkovic SF (2013) Multiple symmetrical lipomatosis—a mitochondrial disorder of brown fat. Mitochondrion 13:269–276CrossRef
7.
go back to reference Ruzicka T, Vieluf D, Landthaler M, Braun-Falco O (1987) Benign symmetric lipomatosis Launois-Bensaude: report of ten cases and review of the literature. J Am Acad Dermatol 17:663–674CrossRef Ruzicka T, Vieluf D, Landthaler M, Braun-Falco O (1987) Benign symmetric lipomatosis Launois-Bensaude: report of ten cases and review of the literature. J Am Acad Dermatol 17:663–674CrossRef
8.
go back to reference Donhauser G, Vieluf D, Ruzicka T, Braun-Falco O (1991) Benign symmetric Launois-Bensaude type III lipomatosis and Bureau-Barriere syndrome. Hautarzt 42:311–314PubMed Donhauser G, Vieluf D, Ruzicka T, Braun-Falco O (1991) Benign symmetric Launois-Bensaude type III lipomatosis and Bureau-Barriere syndrome. Hautarzt 42:311–314PubMed
9.
go back to reference Chalk CH, Mills KR, Jacobs JM, Donaghy M (1990) Familial multiple symmetric lipomatosis with peripheral neuropathy. Neurology 40:1246–1250CrossRef Chalk CH, Mills KR, Jacobs JM, Donaghy M (1990) Familial multiple symmetric lipomatosis with peripheral neuropathy. Neurology 40:1246–1250CrossRef
10.
go back to reference Klopstock T, Naumann M, Schalke B, Bischof F, Seibel P, Kottlors M, Eckert P, Reiners K, Toyka KV, Reichmann H (1994) Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. Neurology 44:862–866CrossRef Klopstock T, Naumann M, Schalke B, Bischof F, Seibel P, Kottlors M, Eckert P, Reiners K, Toyka KV, Reichmann H (1994) Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. Neurology 44:862–866CrossRef
11.
go back to reference Prantl L, Schreml J, Gehmert S, Klein S, Bai X, Zeitler K, Schreml S, Alt E, Gehmert S, Felthaus O (2016) Transcription profile in sporadic multiple symmetric lipomatosis reveals differential expression at the level of adipose tissue-derived stem cells. Plast Reconstr Surg 137:1181–1190CrossRef Prantl L, Schreml J, Gehmert S, Klein S, Bai X, Zeitler K, Schreml S, Alt E, Gehmert S, Felthaus O (2016) Transcription profile in sporadic multiple symmetric lipomatosis reveals differential expression at the level of adipose tissue-derived stem cells. Plast Reconstr Surg 137:1181–1190CrossRef
12.
go back to reference Kazumi T, Ricquier D, Maeda T, Masuda T, Hozumi T, Ishida Y, Yoshida M (1994) Failure to detect brown adipose tissue uncoupling protein mRNA in benign symmetric lipomatosis (Madelung’s disease). Endocr J 41:315–318CrossRef Kazumi T, Ricquier D, Maeda T, Masuda T, Hozumi T, Ishida Y, Yoshida M (1994) Failure to detect brown adipose tissue uncoupling protein mRNA in benign symmetric lipomatosis (Madelung’s disease). Endocr J 41:315–318CrossRef
13.
go back to reference Shibasaki HI II, Shibasaki NTD, Baccan FS, Raposo LS (2014) Multiple symmetrical lipomatosis (Madelung’s disease). Braz J Otorhinolaryngol 80:90–91CrossRef Shibasaki HI II, Shibasaki NTD, Baccan FS, Raposo LS (2014) Multiple symmetrical lipomatosis (Madelung’s disease). Braz J Otorhinolaryngol 80:90–91CrossRef
14.
go back to reference Tadisina KK, Mlynek KS, Hwang LK, Riazi H, Papay FA, Zins JE (2015) Syndromic lipomatosis of the head and neck: a review of the literature. Aesthetic Plast Surg 39:440–448CrossRef Tadisina KK, Mlynek KS, Hwang LK, Riazi H, Papay FA, Zins JE (2015) Syndromic lipomatosis of the head and neck: a review of the literature. Aesthetic Plast Surg 39:440–448CrossRef
15.
go back to reference Agostini T, Perello R, Bani D, Mori A, Quattrini Li A, Russo GL (2014) Histomorphometry of dystrophic fat in a patient suffering Madelung’s disease. J Plast Reconstr Aesthet Surg 67:132–134CrossRef Agostini T, Perello R, Bani D, Mori A, Quattrini Li A, Russo GL (2014) Histomorphometry of dystrophic fat in a patient suffering Madelung’s disease. J Plast Reconstr Aesthet Surg 67:132–134CrossRef
16.
go back to reference Pollock M, Nicholson GI, Nukada H, Cameron S, Frankish P (1988) Neuropathy in multiple symmetric lipomatosis. Madelung’s Dis Brain 111(Pt 5):1157–1171 Pollock M, Nicholson GI, Nukada H, Cameron S, Frankish P (1988) Neuropathy in multiple symmetric lipomatosis. Madelung’s Dis Brain 111(Pt 5):1157–1171
17.
go back to reference Ricquier D (2017) UCP1, the mitochondrial uncoupling protein of brown adipocyte: a personal contribution and a historical perspective. Biochimie 134:3–8CrossRef Ricquier D (2017) UCP1, the mitochondrial uncoupling protein of brown adipocyte: a personal contribution and a historical perspective. Biochimie 134:3–8CrossRef
18.
go back to reference Nicholls DG (2017) The hunt for the molecular mechanism of brown fat thermogenesis. Biochimie 134:9–18CrossRef Nicholls DG (2017) The hunt for the molecular mechanism of brown fat thermogenesis. Biochimie 134:9–18CrossRef
19.
go back to reference Nisoli E, Regianini L, Briscini L, Bulbarelli A, Busetto L, Coin A, Enzi G, Carruba MO (2002) Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 198:378–387CrossRef Nisoli E, Regianini L, Briscini L, Bulbarelli A, Busetto L, Coin A, Enzi G, Carruba MO (2002) Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 198:378–387CrossRef
20.
go back to reference Ong WK, Tan CS, Chan KL, Goesantoso GG, Chan XH, Chan E, Yin J, Yeo CR, Khoo CM, So JB, Shabbir A, Toh SA, Han W, Sugii S (2014) Identification of specific cell-surface markers of adipose-derived stem cells from subcutaneous and visceral fat depots. Stem Cell Rep 2:171–179CrossRef Ong WK, Tan CS, Chan KL, Goesantoso GG, Chan XH, Chan E, Yin J, Yeo CR, Khoo CM, So JB, Shabbir A, Toh SA, Han W, Sugii S (2014) Identification of specific cell-surface markers of adipose-derived stem cells from subcutaneous and visceral fat depots. Stem Cell Rep 2:171–179CrossRef
21.
go back to reference Harms MJ, Ishibashi J, Wang W, Lim HW, Goyama S, Sato T, Kurokawa M, Won KJ, Seale P (2014) Prdm16 is required for the maintenance of brown adipocyte identity and function in adult mice. Cell Metab 19:593–604CrossRef Harms MJ, Ishibashi J, Wang W, Lim HW, Goyama S, Sato T, Kurokawa M, Won KJ, Seale P (2014) Prdm16 is required for the maintenance of brown adipocyte identity and function in adult mice. Cell Metab 19:593–604CrossRef
22.
go back to reference Tapscott SJ, Weintraub H (1991) MyoD and the regulation of myogenesis by helix-loop-helix proteins. J Clin Invest 87:1133–1138CrossRef Tapscott SJ, Weintraub H (1991) MyoD and the regulation of myogenesis by helix-loop-helix proteins. J Clin Invest 87:1133–1138CrossRef
23.
go back to reference Sabourin LA, Rudnicki MA (2000) The molecular regulation of myogenesis. Clin Genet 57:16–25CrossRef Sabourin LA, Rudnicki MA (2000) The molecular regulation of myogenesis. Clin Genet 57:16–25CrossRef
24.
go back to reference Scime A, Soleimani VD, Bentzinger CF, Gillespie MA, Le Grand F, Grenier G, Bevilacqua L, Harper ME, Rudnicki MA (2010) Oxidative status of muscle is determined by p107 regulation of PGC-1alpha. J Cell Biol 190:651–662CrossRef Scime A, Soleimani VD, Bentzinger CF, Gillespie MA, Le Grand F, Grenier G, Bevilacqua L, Harper ME, Rudnicki MA (2010) Oxidative status of muscle is determined by p107 regulation of PGC-1alpha. J Cell Biol 190:651–662CrossRef
25.
go back to reference Ewen ME (1994) The cell cycle and the retinoblastoma protein family. Cancer Metastasis Rev 13:45–66CrossRef Ewen ME (1994) The cell cycle and the retinoblastoma protein family. Cancer Metastasis Rev 13:45–66CrossRef
26.
go back to reference Porras DP, Abbaszadeh M, Bhattacharya D, D’Souza NC, Edjiu NR, Perry CGR, Scime A (2017) p107 determines a metabolic checkpoint required for adipocyte lineage fates. Stem Cells 35:1378–1391CrossRef Porras DP, Abbaszadeh M, Bhattacharya D, D’Souza NC, Edjiu NR, Perry CGR, Scime A (2017) p107 determines a metabolic checkpoint required for adipocyte lineage fates. Stem Cells 35:1378–1391CrossRef
27.
go back to reference Wu L, Zhou L, Chen C, Gong J, Xu L, Ye J, Li D, Li P (2014) Cidea controls lipid droplet fusion and lipid storage in brown and white adipose tissue. Sci China Life Sci 57:107–116CrossRef Wu L, Zhou L, Chen C, Gong J, Xu L, Ye J, Li D, Li P (2014) Cidea controls lipid droplet fusion and lipid storage in brown and white adipose tissue. Sci China Life Sci 57:107–116CrossRef
28.
29.
go back to reference Carey DJ (1997) Syndecans: multifunctional cell-surface co-receptors. Biochem J 327(Pt 1):1–16CrossRef Carey DJ (1997) Syndecans: multifunctional cell-surface co-receptors. Biochem J 327(Pt 1):1–16CrossRef
30.
go back to reference Kasza I, Suh Y, Wollny D, Clark RJ, Roopra A, Colman RJ, MacDougald OA, Shedd TA, Nelson DW, Yen MI, Yen CL, Alexander CM (2014) Syndecan-1 is required to maintain intradermal fat and prevent cold stress. PLoS Genet 10:e1004514CrossRef Kasza I, Suh Y, Wollny D, Clark RJ, Roopra A, Colman RJ, MacDougald OA, Shedd TA, Nelson DW, Yen MI, Yen CL, Alexander CM (2014) Syndecan-1 is required to maintain intradermal fat and prevent cold stress. PLoS Genet 10:e1004514CrossRef
Metadata
Title
Adipose Tissue in Multiple Symmetric Lipomatosis Shows Features of Brown/Beige Fat
Authors
Daniel Schiltz
Sebastian Tschernitz
Christine Ortner
Alexandra Anker
Silvan Klein
Oliver Felthaus
Niklas Biermann
Julia Schreml
Lukas Prantl
Stephan Schreml
Publication date
01-06-2020
Publisher
Springer US
Published in
Aesthetic Plastic Surgery / Issue 3/2020
Print ISSN: 0364-216X
Electronic ISSN: 1432-5241
DOI
https://doi.org/10.1007/s00266-020-01666-6

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