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ACTUALIZACION SOBRE LA LIPOMATOSIS SIMETRICA MULTIPLE

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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

ACTUALIZACION SOBRE LA LIPOMATOSIS SIMETRICA MULTIPLE

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
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Multiple Symmetrical Lipomatosis

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


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



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Enviar correspondencia a:
Igor A. Harsch, Medical Department 1, Division of Endocrinology and Metabolism, Friedrich-Alexander University Erlangen-Nuremberg, 91054, Ulmenweg 18, Erlangen, Alemania
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