Conceptos Categóricos

ACTUALIZACION SOBRE LA LIPOMATOSIS SIMETRICA MULTIPLE

ACTUALIZACION SOBRE LA LIPOMATOSIS SIMETRICA MULTIPLE

(especial para SIIC © Derechos reservados)
La lipomatosis simétrica múltiple es una enfermedad poco frecuente caracterizada por el crecimiento de lipomas indoloros no encapsulados. Los lipomas están localizados preferentemente en el cuello, cintura escapular, la parte proximal de brazos y piernas y excluyen el rostro, antebrazos y muslos. La enfermedad generalmente comienza en la madurez y predomina en el sexo masculino. La mayoría de los pacientes presenta o tuvo antecedentes de un elevado consumo de alcohol.
bergmann.jpg Autor:
Tanja Bergmann
Columnista Experto de SIIC

Institución:
Medical Department 1, Friedrich-Alexander University of Erlangen-Nuremberg


Artículos publicados por Tanja Bergmann
Coautor
Igor Alexander Harsch* 
MD, Assistant Professor, Medical Department 1, Friedrich-Alexander University of Erlangen-Nuremberg - Division of Endocrinology and Metabolism, Erlangen, Alemania*
Recepción del artículo
5 de Mayo, 2006
Aprobación
17 de Mayo, 2006
Primera edición
13 de Octubre, 2006
Segunda edición, ampliada y corregida
7 de Junio, 2021

Resumen
La lipomatosis simétrica multiple (LSM) es una enfermedad poco frecuente caracterizada por el crecimiento de lipomas indoloros no encapsulados. Los lipomas están localizados preferentemente en el cuello, cintura escapular, la parte proximal de brazos y piernas y excluyen el rostro, antebrazos y muslos. La enfermedad generalmente comienza en la madurez y predomina en el sexo masculino. La mayoría de los pacientes presenta o tuvo antecedentes de un elevado consumo de alcohol. Existe un número pequeño de casos familiares. El análisis ultraestructural de los adipocitos de los lipomas muestra características de tejido adiposo pardo más que blanco. En relación con la etiología existen distintas teorías. La más reciente es la hipótesis de una alteración de la señalización simpática, lo que induce una proliferación y diferenciación defectuosas de los adipocitos. La enfermedad puede asociarse con neuropatía, hepatopatía, hiperuricemia y tumores malignos del tracto orofaríngeo. El crecimiento del tejido adiposo puede causar el síndrome de apnea obstructiva del sueño, disnea, disfagia y compresión del los grandes vasos mediastinales y cervicales. La dieta carece de impacto sobre el crecimiento del tejido lipomatoso y el tratamiento quirúrgico es el único que ha probado ser efectivo, no obstante, la remoción completa es dificultosa y las recaídas son frecuentes. Puede haber una mortalidad significativamente elevada en los pacientes con LSM.

Palabras clave
Lipomatosis simétrica múltiple, lipomas, tejido adiposo, obesidad, adipocitos


Artículo completo

(castellano)
Extensión:  +/-11.01 páginas impresas en papel A4
Exclusivo para suscriptores/assinantes

Abstract
Multiple symmetrical lipomatosis (MSL) is a rare disorder characterized by symmetrical growth of painless non encapsulated lipomata. The lipomata are predominantly located around the neck, shoulder girdle and proximal upper arms and legs with regularly excluding the face, the forearms and thighs. The onset of the disease is usually the middle-age with a predominance of the male sex. The majority of the patients have or have had a history of high ethanol intake. There is a small group of familial cases. In ultrastructural analysis the adipocytes within the lipomata show characteristics of brown adipose tissue rather than white adipose tissue. Concerning the etiology different theories exist. Most recently a defective sympathetic signalling resulting in altered proliferation and differentiation of adipocytes has been proposed. The disease can be associated with neuropathy, hepatopathy, hyperuricemia and malignant tumours of the oropharyngeal tract. Adipose tissue growth can cause obstructive sleep apnea syndrome, dyspnea, dysphagia and compression of the great cervical and mediastinal vessels. Dietary treatment does not have any impact on lipomatous tissue growth. Surgical removal is the only proven means of therapy, but complete removal is difficult and relapses are seen. There can be seen a significant higher mortality in MSL patients.

Key words
Multiple symmetrical lipomatosis, lipomata, adipose tissue, adipocytes, obesity


Full text
(english)
para suscriptores/ assinantes

Clasificación en siicsalud
Artículos originales > Expertos del Mundo >
página   www.siicsalud.com/des/expertocompleto.php/

Especialidades
Principal: Medicina Interna
Relacionadas: Anatomía Patológica, Diagnóstico por Laboratorio, Endocrinología y Metabolismo, Medicina Familiar



Comprar este artículo
Extensión: 11.01 páginas impresas en papel A4

file05.gif (1491 bytes) Artículos seleccionados para su compra



Enviar correspondencia a:
Igor A. Harsch, Medical Department 1, Division of Endocrinology and Metabolism, Friedrich-Alexander University Erlangen-Nuremberg, 91054, Ulmenweg 18, Erlangen, Alemania
Bibliografía del artículo
1. Brodie BC. Lectures illustrative of various subjects in pathology and surgery. Longman: London; 1846:275-276.
2. Farag TI, Sabry MA, Iskandar A. Queen of punt syndrome? http://www.faseb.org/genetics/ashg99/f803.htm.
3. Enzi G. Multiple symmetric lipomatosis: an updated clinical report. Medicine (Baltimore) 1984; 63(1):56-64.
4. Lacotte B, Themelin D, Ledoux A. Launois-Bensaude disease. Apropos of 2 new cases. J Chir (Paris) 1987; 124(12):684-689.
5. Harsch IA, Schahin SP, Wiedmann R. Multiple symmetric lipomatosis: a paradigm of metabolically innocent obesity? Response to Haap et al. Diabetes Care 2004; 27(7):1849-1850.
6. Busetto L, Strater D, Enzi G, Coin A, Sergi G, Inelmen EM, Pigozzo S. Differential clinical expression of multiple symmetric lipomatosis in men and women. Int J Obes Relat Metab Disord 2003; 27(11):1419-1422.
7. Enzi G, Angelini C, Negrin P, Armani M, Pierobon S, Fedele D. Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis. Medicine (Baltimore) 1985; 64(6):388-393.
8. Rudenskaia GE. A case of multiple symmetric lipomatosis with polyneuropathy. Zh Nevrol Psikhiatr Im S S Korsakova 2001; 101(8):56-58.
9. Loke TK, Yung CK, Chow TL, Lo SS, Chan CS. Multiple symmetric lipomatosis in the chinese: ultrasound, CT and MR imaging. Clin Radiol 1998; 53(12):903-906.
10. Chan ES, Ahuja AT, King AD, Lau WY. Head and neck cancers associated with Madelung's disease. Ann Surg Oncol 1999; 6(4):395-397.
11. Lee HW, Kim TH, Cho JW, Ryu BY, Kim HK, Choi CS. Multiple symmetric lipomatosis: Korean experience. Dermatol Surg 2003; 29(3):235-240.
12. Kitano H, Nakanishi Y, Takeuchi E, Nagahara K. Multiple symmetrical lipomatosis: no longer just a Mediterranean disease? ORL J Otorhinolaryngol Relat Spec 1994; 56(3):177-180.
13. Morinaka S, Sato T, Miyoshi H, Iwashita K. A case of multiple symmetrical lipomatosis (Madelung's disease). Auris Nasus Larynx 1999; 26(3):349-353.
14. Hacker SM, Ramos-Caro FA. An uncommon presentation of multiple symmetric lipomatosis. Int J Dermatol 1993; 32(8):594-597.
15. Leung NW, Gaer J, Beggs D, Kark AE, Holloway B, Peters TJ. Multiple symmetric lipomatosis (Launois-Bensaude syndrome): effect of oral salbutamol. Clin Endocrinol (Oxf) 1987; 27(5):601-606.
16. Pollock M, Nicholson GI, Nukada H, Cameron S, Frankish P. Neuropathy in multiple symmetric lipomatosis. Madelung's disease. Brain 1988; 111(Pt 5):1157-1171.
17. Klopstock T, Naumann M, Seibel P, Shalke B, Reiners K, Reichmann H. Mitochondrial DNA mutations in multiple symmetric lipomatosis. Mol Cell Biochem 1997; 174(1-2):271-275.
18. Chalk CH, Mills KR, Jacobs JM, Donaghy M. Familial multiple symmetric lipomatosis with peripheral neuropathy. Neurology 1990; 40(8):1246-1250.
19. Muñoz Fernández C, Aladro Y, Conde, Campos Y, Arenas J. Multiple symmetrical lipomatosis with familial polyneuropathy. Rev Neurol 2001; 32(12):1107-1111.
20. Gamez J, Playan A, Andreu AL, Bruno C, Navarro C, Cervera C, Arbos MA, Schwartz S, Enriquez JA, Montoya J. Familial multiple symmetric lipomatosis associated with the A8344G mutation of mitochondrial DNA. Neurology 1998; 51(1):258-260.
21. Maher B, Alfirevic A, Vilar FJ, Wilkins EG, Park BK, Pirmohamed M. TNF-alpha promoter region gene polymorphisms in HIV-positive patients with lipodystrophy. AIDS 2002; 16(15):2013-2018.
22. Enzi G, Busetto L, Ceschin E, Coin A, Digito M, Pigozzo S. Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study. Int J Obes Relat Metab Disord 2002; 26(2):253-261.
23. Payne CE. Hereditary Madelung's disease. J R Soc Med 2000; 93(4):194-195.
24. Kratz C, Lenard HG, Ruzicka T, Gartner J. Multiple symmetric lipomatosis: an unusual cause of childhood obesity and mental retardation. Eur J Paediatr Neurol 2000; 4(2):63-67.
25. Castro-Gago M, Alonso A, Pintos Martínez E, Novo Rodríguez MI, Blanco Barca MO, Campos Y, Arenas J, Eiris Punal J. Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathy. Rev Neurol 2003; 36(11):1026-1029.
26. Hengel RL, Watts NB, Lennox JL. Benign symmetric lipomatosis associated with protease inhibitors. Lancet 1997; 350(9091):1596.
27. Donhauser G, Vieluf D, Ruzicka T, Braun-Falco O. Benign symmetric Launois-Bensaude type III lipomatosis and Bureau-Barrière syndrome. Hautarzt 1991; 42(5):311-314.
28. Soler R, Requejo I, Fontan FJ, Lopez-Suso ME. MR of laryngeal and scrotal involvement in multiple symmetrical lipomatosis. Eur Radiol 1997; 7(6):946-948.
29. Fessel WJ, Follansbee SB, Barker B. Ultrastructural findings consistent with brown adipocytes in buffalo humps of HIV-positive patients with fat redistribution syndrome. Abstract P1. Antivir Ther. 2000;5:25.
30. Ruzicka T, Vieluf D, Landthaler M, Braun-Falco O. Benign symmetric lipomatosis Launois-Bensaude. Report of ten cases and review of the literature. J Am Acad Dermatol 1987; 17(4):663-674.
31. Stavropoulos PG, Zouboulis CC, Trautmann C, Orfanos CE. Symmetric lipomatosis in female patients. Dermatology 1997; 194(1):26-31.
32. Harsch IA, Schahin SP, Fuchs FS, Hahn EG, Lohmann T, Konturek PC, Ficker JH. Insulin resistance, hyperleptinemia, and obstructive sleep apnea in Launois-Bensaude syndrome. Obes Res 2002; 10(7):625-632.
33. Wechsler JG. Obesity and lipid metabolism disorders. Internist (Berl) 1997; 38(3):231-236.
34. Enzi G, Favaretto L, Martini S, Fellin R, Baritussio A, Baggio G, Crepaldi G. Metabolic abnormalities in multiple symmetric lipomatosis: elevated lipoprotein lipase activity in adipose tissue with hyperlaphalipoproteinemia. J Lip Res 1983; 24(5):566-574.
35. Hufnagel C. Ontogenetische Veränderung des Plasmaleptinspiegels und seine Regulation bei 10 Tage alten Ratten [ontogenetic change of the plasma leptin level and its regulation in 10 days old rats]. Inaugural-Dissertation. Giessen 2001.
36. Beltowski J. Adiponectin and resistin - new hormones of white adipose tissue. Med Sci Monit 2003; 9(2):RA55-61.
37. Hotamisligil GS. The irresistible biology of resistin. J Clin Invest 2003; 111(2): 173-174.
38. Harsch IA, Michaeli P, Hahn EG, Ficker JH, Konturek PC. Launois-Bensaude syndrome in a female with type 2 diabetes. Med Sci Monit 2003; 9(2):CS5-8.
39. Haap M, Siewecke C, Thamer C, Machann J, Schick F, Haring HU, Szeimies RM, Stumvoll M. Multiple symmetric lipomatosis: a paradigm of metabolically innocent obesity? Diabetes Care 2004; 27(3):794-795.
40. Nisoli E, Regianini L, Briscini L, Bulbarelli A, Busetto L, Coin A, Enzi G, Carruba MO. Multiple symmetric lipomatosis may be the consequence of defective noradrenergic modulation of proliferation and differentiation of brown fat cells. J Pathol 2002; 198(3):378-387.
41. Kodish ME, Alsever RN, Block MB. Benign symmetric lipomatosis: functional sympathetic denervation of adipose tissue and possible hypertrophy of brown fat. Metabolism 1974; 23(10):937-945.
42. Constantinidis J, Steinhart H, Zenk J, Bohlender J, Iro H. Surgical therapy of Madelung's disease in the head and neck area. HNO 2003; 51(3):216-220.
43. Fedele D, Bellavere F, Bosello G, Cardone C, Girardello L, Ferri M, Enzi G. Impairment of cardiovascular autonomic reflexes in multiple symmetric lipomatosis. J Auton Nerv Syst 1984; 11(2):181-188.
44. Neundorfer B. Alcohol polyneuropathy. Fortschr Neurol Psychiatr 2001; 69(8): 341-345.
45. Saiz Hervas E, Martin Llorens M, Lopez Alvarez J. Peripheral neuropathy as the first manifestation of Madelung's disease. Br J Dermatol 2000; 143(3):684-686.
46. Zancanaro C, Sbarbati A, Morroni M, Carraro R, Cigolini M, Enzi G, Cinti S. Multiple symmetric lipomatosis. Ultrastructural investigation of the tissue and preadipocytes in primary culture. Lab Invest 1990; 63(2):253-258.
47. Cinti S, Enzi G, Cigolini M, Bosello O. Ultrastructural features of cultured mature adipocyte precursors from adipose tissue in multiple symmetric lipomatosis. Ultrastruct Pathol 1983; 5(2-3):145-152.
48. Ujpal M, Nemeth ZS, Reichwein A, Szabo GY. Long-term results following surgical treatment of benign symmetric lipomatosis (BSL). Int J Oral Maxillofac Surg 2001; 30(6):479-483.
49. Tizian C, Berger A, Vykoupil KF. Malignant degeneration in Madelung's disease (benign lipomatosis of the neck): case report. Br J Plast Surg 1983; 36(2):187-189.
50. Ricquier D. Neonatal brown adipose tissue, UCP1 and the novel uncoupling proteins. Biochem Soc Trans 1998; 26(2):120-123.
51. Del Mar González Barroso M, Ricquier D, Cassard-Doulcier AM. The human uncoupling protein-1 gene (UCP1): present status and perspectives in obesity research. Obes Rev 2000; 1(2):61-72.
52. Garruti G, Ricquier D. Analysis of uncoupling protein and its mRNA in adipose tissue deposits of adult humans. Int J Obes Relat Metab Disord 1992; 16(5):383-390.
53. Krief S, Lonnqvist F, Raimbault S, Baude B, Van Spronsen A, Arner P, Strosberg AD, Ricquier D, Emorine LJ. Tissue distribution of ß3-adrenergic receptor mRNA in man. J Clin Invest 1993; 91(1):344-349.
54. Nielsen S, Levine J, Clay R, Jensen MD. Adipose tissue metabolism in benign symmetric lipomatosis. J Clin Endocrinol Metab 2001; 86(6):2717-2720.
55. Enzi G, Inelmen E, Baritussio A, Dorigo P, Prosdocimi M, Mazzoleni F. Multiple symmetric lipomatosis: a defect in adrenergic-stimulated lipolysis. J Clin Invest 1977; 60(6):1221-1229.
56. Kather H, Schroder F. Adrenergic regulation of fat-cell lipolysis in multiple symmetric lipomatosis. Eur J Clin Invest 1982; 12(6):471-474.
57. Nisoli E, Clementi E, Tonello C, Sciorati C, Briscini L, Carruba MO. Effects of nitric oxide on proliferation and differentiation of rat brown adipocytes in primary cultures. Br J Pharmacol 1998; 125(4):888-894.
58. Berkovic SF, Andermann F, Shoubridge EA, Carpenter S, Robitaille Y, Andermann E, Melmed C, Karpati G. Mitochondrial dysfunction in multiple symmetrical lipomatosis. Ann Neurol 1991; 29(5):566-569.
59. Silvestri G, Ciafaloni E, Santorelli FM, Shanske S, Servidei S, Graf WD, Sumi M, DiMauro S. Clinical features associated with the A'G transition at nucleotide 8344 of mtDNA ("MERRF mutation"). Neurology 1993; 43(6):1200-1206.
60. Schon EA, Manfredi G. Neuronal degeneration and mitochondrial dysfunction. J Clin Invest. 2003; 111(3):303-312.
61. Matthews PM, Squier MV, Chalk C, Donaghy M. Mitochondrial abnormalities are not invariably present in neurologic syndromes associated with multiple symmetric lipomatosis. Neurology 1995; 45(1):197-198.
62. Cederbaum AI. Effects of alcohol on hepatic mitochondrial function and DNA. Gastroenterology 1999; 117(1):265-269.
63. Mansouri A, Demeilliers C, Amsellem S, Pessayre D, Fromenty B. Acute ethanol administration oxidatively damages and depletes mitochondrial DNA in mouse liver, brain, heart, and skeletal muscles: protective effects of antioxidants. J Pharmacol Exp Ther 2001; 298(2):737-743.
64. Huttunen P, Kortelainen ML. Long-term alcohol consumption and brown adipose tissue in man. Eur J Appl Physiol Occup Physiol 1990; 60(6):418-424.
65. Fischer T, Schworer H, Ramadori G. Benign symmetrical lipomatosis ("peripheral lipodystrophy") during antiretroviral therapy of HIV infection. Dtsch Med Wochenschr 1998; 123(50):1512-1516.
66. Brinkman K, Smeitink JA, Romijn JA, Reiss P. Mitochondrial toxicity induced by nucleoside-analogue reverse-transcriptase inhibitors is a key factor in the pathogenesis of antiretroviral-therapy-related lipodystrophy. Lancet 1999; 354(9184):1112-1115.
67. Carr A, Samaras K, Chisholm DJ, Cooper DA. Pathogenesis of HIV-1-protease inhibitor-associated peripheral lipodystrophy, hyperlipidaemia, and insulin resistance. Lancet 1998; 351(9119):1881-1883.
68. Walker UA. Mitochondriale Toxizität von Nukleosidanaloga [mitochondrial toxicity of nucleoside analogues]. HIV.NET. Steinhäuser 2004. Chapter 8:285-292.
69. Lyon IP. Adiposis and Lipomatosis: Considered in reference to their constitutional relations and symptomatology. Arch Intern Med 1910; 6:28-120.
70. Madelung OW. Ueber den Fetthals (diffuses Lipom des Halses) [About the fatty neck - diffuse lipoma of the neck]. Archiv für klinische Chirurgie 1888; 37:106-130.
71. Ciambelli F, Limbiati S, Mattaini R, Mozzana R. Symmetric multiple lipomatosis. A case report with an unusual onset. Minerva Med 2000; 91(5-6):123-126.
72. Smola MG, Scharnagl E, Deutschmann W, Beham A. Multiple symmetrical lipomatosis. A retrospective study of 14 cases and review of the literature. Handchir Mikrochir Plast Chir 1988; 20(5):234-238.
73. Fischer M, Wohlrab J, Taube KM, Marsch WC. Intralesional injection of enoxaparin in benign symmetrical lipomatosis: an alternative to surgery? Br J Dermatol 2001; 144(3):629-630.
74. Horl C, Biemer E. Benign symmetrical lipomatosis. Lipectomy and liposuction in the treatment of Madelung's disease. Handchir Mikrochir Plast Chir 1992; 24(2):93-96.
75. Kuipers T, Greulich M, Gubisch W. Clinical aspects and therapy of benign symmetrical lipomatosis - Madelung's disease. Handchir Mikrochir Plast Chir 1999; 31(6):393-400.
76. Martinez-Escribano JA, Gonzalez R, Quecedo E, Febrer I. Efficacy of lipectomy and liposuction in the treatment of multiple symmetric lipomatosis. Int J Dermatol 1999; 38(7):551-554.

 
 
 
 
 
 
 
 
 
 
 
 
Está expresamente prohibida la redistribución y la redifusión de todo o parte de los contenidos de la Sociedad Iberoamericana de Información Científica (SIIC) S.A. sin previo y expreso consentimiento de SIIC.
ua31618
-->