Highlights
- •Multiple Symmetric Lipomatosis (MSL) is a rare disease mostly affecting heavy drinking men.
- •Clinical features: symmetrical distribution and mediastinal occupation by lipomatous tissue.
- •Typical metabolic abnormalities: hyper-α-lipoproteinemia with high lipoprotein-lipase activity.
- •MSL could be the result of a disorder of proliferation and differentiation of BAT.
Abstract
Aim
Aim of this study is an updated review of our case series (72 patients) as well as
available literature on the Multiple Symmetric Lipomatosis (MSL), a rare disease primarily
involving adipose tissue, characterized by the presence of not encapsulated fat masses,
symmetrically disposed at characteristic body sites (neck, trunk, proximal parts of
upper and lower limbs).
Data synthesis
The disease is more frequent in males, associated to an elevated chronic alcohol consumption,
mainly in form of red wine. Familiarity has been reported and MSL is considered an
autosomic dominant inherited disease. MSL is associated to severe clinical complications,
represented by occupation of the mediastinum by lipomatous tissue with a mediastinal
syndrome and by the presence of a somatic and autonomic neuropathies. Hyper-alphalipoproteinemia
with an increased adipose tissue lipoprotein-lipase activity, a defect of adrenergic
stimulated lipolysis and a reduction of mitochondrial enzymes have been described.
The localization of lipomatous masses suggests that MSL lipomas could originate from
brown adipose tissue (BAT). Moreover, studies on cultured pre-adipocytes demonstrate
that these cells synthetize the mitochondrial inner membrane protein UCP-1, the selective
marker of BAT. Surgical removal of lipomatous tissue is to date the only validated
therapeutic approach.
Conclusions
MSL is supposed to be the result of a disorder of the proliferation and differentiation
of human BAT cells.
Keywords
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References
- Lectures illustrative of various subjects in pathology and surgery.Longman, London1846: 275-276
- Ueber den fetthals (diffuses lipom des halses).Arch Klin Chir (Berlin). 1888; 37: 106-130
- De l’adénolipomatose symétrique.Bull Mem Soc Med Hop Paris. 1898; 1: 298-303
- Multiple symmetric lipomatosis: an updated clinical report.Medicine. 1984; 63: 56-64
- Differential clinical expression of multiple symmetric lipomatosis in men and women.Int J Obes. 2003; 27: 1419-1422
- Mendelian inheritance in man.John Hopkins University Press, Baltimore1978: 242
- Brown adipose tissue in a newborn.Proc Bayl Univ Med Cent. 2008; 21: 328-330
- Evaluation by computed tomography of deep locations of lipomatosis tissue in multiple symmetric lipomatosis.Radiology. 1982; 244: 121-144
- Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study.Int J Obes. 2002; 26: 253-261
- Multiple symmetric lipomatosis with mediastinal involvement and subclinical hypothyroidism in a female patient.T Klin Med Sci. 2002; 22: 413-416
- Severe dyspnea as atypical presenting symptom of madelung's disease.Hippokratia. 2010; 14: 133-135
- Benign symmetrical lipomatosis of the tongue in madelung's disease.J Craniomaxillofac Surg. 2006; 34: 489-493
- Madelung's disease involving the tongue.J Am Acad Dermatol. 2000; 42: 511-513
- Scrotal involvement in madelung disease: clinical, ultrasound and MR findings.Abdom Imaging. 2006; 31: 503-505
- Launois-Bensaude syndrome involving the orbits.J Craniomaxillofac Surg. 2011; 39: 21-23
- Un cas nouveau de lipomatose symmétrique associée à un double mal perforant plantaire.Bull Mem Soc Hop Paris. 1937; 39: 53
- Paralisis general progresivo associada a una lipomatosis difusa simmetrica.Prensa Med Argent. 1940; 28: 135
- Sensory, motor and autonomic neuropathy in patients with multiple symmetric lipomatosis.Medicine. 1986; 64: 388-393
- Impairment of cardiovascular autonomic reflexes in multiple symmetric lipomatosis.J Auton Nerv Syst. 1984; 11: 181-188
- Neuropathy in multiple symmetric lipomatosis. Madelung's disease.Brain. 1988; 11: 1157-1161
- Neurological multisystem manifestation in multiple symmetric lipomatosis: a clinical and electrophysiological study.Neurology. 1994; 44: 862-866
- Multiple symmetric lipomatosis presenting with polyneuropathy.Isr J Med Sci. 1995; 31: 693-695
- Multiple symmetric lipomas with high levels of mDNA with the tRNA Lys G(8344) mutation as the only manifestation in a carrier of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome.Am J Hum Genet. 1993; 52: 551-556
- Multiple symmetrical lipomatosis: abnormalities in complex IV and multiple deletions in mitocondrial DNA.Neurology. 1994; 44: 862-866
- Mitocondrial dysfunction with myoclonus epilepsy and ragged red fibers point mutation in nerve, muscle and adipose tissue of a patient with multiple symmetric lipomatosis.Muscle Nerve. 1997; 20: 833-839
- Multiple lipomas, alcoholism and neuropathy: madelung's disease or MERRF?.Muscle Nerve. 2006; 33: 142-146
- Familial multiple symmetric lipomatosis associated with the A8344G mutation of mitochondrial DNA.Neurology. 1998; 51: 258-260
- Multiple symmetric lipomatosis with familial polineuropathy.Rev Neurol. 2001; 32: 1107-1111
- Familial multiple symmetric lipomatosis with peripheral neuropathy.Neurology. 1990; 40: 1246-1250
- Multiple symmetric lipomatosis: evidence for mitocondrial disfunction.J Clin Neuromusc Dis. 2000; 1: 124-130
- Defects of mitochondrial respiratory chain in multiple symmetric lipomatosis.Arch Dermatol Res. 1998; 290: 652-655
- Lipomatosis, proximal myopathy and the mitochondrial μ 8344 mutation. A lipid storage myopathy?.Muscle Nerve. 2000; 23: 538-542
- Multiple symmetric lipomatosis. An unusual cause of childhood obesity and mental retardation.Eur J Pediatr Neurol. 2000; 4: 63-67
- Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathology.Rev Neurol. 2003; 36: 1026-1029
- Cardiac noradrenergic denervation in a patient whit multiple symmetrc lipomatosis.Cardiology. 2012; 121: 160-163
- Benign symmetrical lipomatosis (launois-bensaude adenolipomatosis) with gout and hyperlipoproteinemia.Am J Med. 1970; 48: 239-245
- Metabolic abnormalities in multiple symmetric lipomatosis: elevated lipoproteinlipase activity in adipose tissue with hyperalphalipoproteinemia.J Lipid Res. 1983; 24: 566-574
- Lipoprotein metabolism in patients with elevated lipoprotein lipase activity in adipose tissue.Int J Obes. 1985; 9: 173-176
- Inverse association bitween abitual polyphenol intake and incidence of cardiovascular events in PREDIMED study.Nutr Med Cardiovasc Dis. 2014; 24: 639-647
- Harmful and beneficial relationships between alcohol consumption and subclinical atherosclerosis.Nutr Metab Cardiovasc Dis. 2014; 24: 767-776
- Total and regional body composition and energy expenditure in multiple symmetric lipomatosis.Clin Nutr. 2005; 24: 367-374
- Multiple symmetric lipomatosis: a defect in adrenergic stimulated lipolysis.J Clin Invest. 1977; 60: 1221-1229
- The emergence of cold-induced brown adipocytes in mouse withe fat depots is determined predominantly by withe to brown adipocite transdifferentiation.Am J Physiol Endocrinol Metab. 2010; 298: E1244-E1253
- Multiple symmetric lipomatosis: ultrastructural investigation of the tissue and preadipocytes in primary colture.Lab Invest. 1990; 63: 253-258
- Ultrastructural features of cultured mature adipocytes precursors from adipose tissue in multiple symmetric lipomatosis.Ultrastruct Pathol. 1984; 5: 145-151
- Transdifferentiating properties of adipocytes in the adipose organ.Am J Physiol Endocrinol Metab. 2009; 297: E977-E986
- PRDM16 controls a brown fat/skeletal muscle switch.Nature. 2008; 454: 961-967
- Initiation of myoblast to fat switch by a PRMD16- C/EBP-β transcriptional complex.Nature. 2009; 460: 1154-1158
- Muscle injury activates resident fibro/adipogenic progenitors that facilitate myogenesis.Nat Cell Biol. 2010; 12: 153-163
- Basic FGF-dependent hyperplastic growth of preadipocytes in multiple symmetric lipomatosis.Int J Obes. 1994; 18: S125
- Basic fibroblast growth factor (b-FGF) contributes to the enlargement of brown adipose tissue during cold acclimation.Plüfger Arch. 1994; 428: 552-556
- Cold-activated brown adipose tissue in healthy men.N Engl J Med. 2009; 360: 1500-1508
- Identification and importance of brown adipose tissue in adult humans.N Engl J Med. 2009; 360: 1509-1517
- Functional brown adipose tissue in healthy adults.N Engl J Med. 2009; 360: 1518-1525
- The presence of UCP1 demonstrates that metabolically active adipose tissue in the neck of adult humans truly represents brown adipose tissue.FASEB J. 2009; 23: 1-7
- Benign symmetric lipomatosis: functional sympathetic denervation of adipose tissue and possible hypertrophy of brown fat.Metab Clin Exp. 1974; 23: 937-945
- Multiple symmetric lipomatosis may be the consequence of detective noradrenergic modulation of proliferation and differentiation of brown fat cells.J Pathol. 2002; 198: 378-387
- Pten positively regulates brown adipose tissue function, energy expenditure, and longevity.Cell Metab. 2012; 15: 382-394
- PTEN loss in the Myf5 lineage redistributes body fat and reveals subsets of with adipocytes that arise from Myf5 precursors.Cell Metab. 2012; 16: 348-362
- Resveratrol increases brown adipose tissue thermogenesis markers by increasing SIRT 1 and energy expenditure and decreasing fat accumulation in adipose tissue of mice fed a standard diet.Eur J Nutr. 2014; 53: 1503-1510
- Long term results following surgical treatment of benign symmetrical lipomatosis.Int J Oral Maxillofac Surg. 2001; 30: 479-483
- Liposuction in benign symmetric lipomatosis: sense or senseless?.Aesthetic Plast Surg. 2003; 27: 319-321
- Combined surgical lipectomy and liposuction in the treatment of benign symmetrical lipomatosis of the head and neck.Scand J Plast Reconstr Surg Hand Surg. 2003; 37: 90-96
- Surgical treatment of multiple symmetric lipomatosis with ultrasound-assisted liposuction.Ann Plast Surg. 2014; 73 ([Epub ahead of print]): 559-562
- Multiple benign symmetrical lipomatosis-a differential diagnosis of obesity: is there a rationale for fibrate treatment?.Obes Surg. 2008; 18: 1354-1356
- Mesotherapy for benign symmetric lipomatosis.Aesthetic Plast Surg. 2010; 34: 153-156
- Mesotherapy should not replace the surgical approach in the treatment of benign symmetric lipomatosis.Aesthetic Plast Surg. 2011; 35: 278-280
- A randomized controlled trial assessing the effect of oral diazepam on 18F-FDG uptake in the neck and upper chest region.Mol Imaging Biol. 2009; 11: 364-368
- Reduction of FDG uptake in brown adipose tissue by propranolol.Eur J Nucl Med Mol Imaging. 2005; 32: 1130
- Intense (18) F-FDG uptake in brown fat can be reduced pharmacologically.J Nucl Med. 2004; 45: 1189-1193
Article info
Publication history
Published online: February 05, 2015
Accepted:
January 28,
2015
Received in revised form:
January 28,
2015
Received:
September 22,
2014
Identification
Copyright
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
