Multiple Symmetric Lipomatosis: Korean Experience

Lee, Hae Wan; Kim, Tae Hwa; Cho, Ji Woong; Ryu, Byoung Y.; Kim, Hong Ki; Choi, Choongik

doi:10.1046/j.1524-4725.2003.29056.x

Cited by 30 publications

(27 citation statements)

References 18 publications

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“…Male-to-female ratio was 4:1 in a previous review of the literature, 8 but it ranged to 30:1 in a recently published series of 32 Korean patients with the disease. 9 In our most recent study on the disease, 4 we confirmed a male-to-female ratio of 30:1 in a series of 66 Mediterranean subjects. Since then, however, we had the opportunity to observe several female cases of symmetrical lipomatosis who presented some of the usual aspects of MSL, but demonstrated some peculiar clinical features.…”

Section: Introductionsupporting

confidence: 66%

Differential clinical expression of multiple symmetric lipomatosis in men and women

et al. 2003

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BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterised by the growth of uncapsulated masses of adipose tissue. MSL is associated with high ethanol intake and complicated by somatic and autonomic neuropathy and the infiltration of adipose tissue at the mediastinal level. To date, the disease is considered as largely more prevalent in men. OBJECTIVE: To provide a detailed description of the clinical aspects of MSL in women. PATIENTS: A total of 11 women and 58 men with MSL. MEASUREMENTS: Morphological aspect of patient, location of the lipomatous masses, alcohol intake, extension of lipomatous tissue to the mediastinum, association with somatic and autonomic neuropathy, and metabolic profile. RESULTS: All female patients had the obesity-like appearance of type II MSL and the most frequent locations of lipomatous masses in women were at the proximal arms (90.9%) and legs (54.5%). Very few female patients (27.3%) presented with the submental deposition of lipomatous tissue typical of both type I (97.3%; Po0.001) and type II (66.7%; Po0.05) male subjects. An extension of the lipomatus tissue around the upper airways, associated with compression or dislocation of deeply located mediastinal structures, was observed less frequently in women than in men. The presence of a high ethanol intake, the association with somatic and autonomic neuropathy and the occurrence of a characteristic metabolic pattern (high HDLcholesterol, low LDL-cholesterol, high uric acid) were similar in men and in women. CONCLUSIONS: Female patients with MSL had a sex-specific morphological aspect, characterised by a low occurrence of the typical 'Madelung collar' and a usual obesity-like appearance.

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Section: Introductionsupporting

confidence: 66%

Differential clinical expression of multiple symmetric lipomatosis in men and women

et al. 2003

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“…Primary recommendations 1) Alcohol abstinence: Abstinence from alcohol may arrest further progression of the MSL SAT but does not cause regression of the SAT deformities [92] .…”

Section: Msl Treatment Recommendationsmentioning

confidence: 99%

“…In a majority of cases of MSL Type II and III, resection or liposuction of the lipomatosis is considered cosmetic and insurance companies are reluctant to cover this procedure [98] . Unfortunately, the fat usually penetrates and surrounds deeper structures such as muscle and bone, making total excision of the abnormal tissue difficult [92] ; the lipomatosis can, therefore, recur after liposuction or excision [87,99,100] ; in three of eleven patients in one series [101] .…”

Section: Msl Treatment Recommendationsmentioning

confidence: 99%

Rare adipose disorders (RADs) masquerading as obesity

2012

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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidencebased data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.

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“…Asimismo, la macroglosia puede estar presente en una gran variedad de condiciones, como síndromes congénitos, enfermedades metabólicas, endocrinas, o pueden aparecer en otras como amiloidosis, hialinois o tumores linguales. 15 El curso clínico de la enfermedad comienza con un primer período de crecimiento rápido, seguido de una progresión lenta. No existe ningún caso publicado de regresión espontánea de los lipomas, a pesar de abandonar el hábito alcohólico.…”

Section: Discussionunclassified

Lipomatosis simétrica benigna de la lengua en la enfermedad de Madelung

Ceres¹,

Lizarralde

García³

et al. 2006

Rev Esp Cirug Oral y Maxilofac

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Resumen: La enfermedad de Madelung, o lipomatosis simétrica benigna es una enfermedad rara caracterizada por acúmulos grasos no encapsulados localizados de forma simétrica alrededor de cuello y hombros. Esta enfermedad, afecta predominantemente a hombres en edades comprendidas entre los 30 y 60 años, con una relación hombre:mujer de 15:1. Existe una gran relación con el abuso del alcohol. Las personas no alcohólicas y las mujeres también pueden verse afectadas, aunque de forma más rara. Presentamos el caso de una mujer diagnosticada de Enfermedad de Madelung refiriendo engrosamiento progresivo de la lengua. Presentaba dificultad para tragar, disartria y disnea con el decúbito. Esp Cir Oral y Maxilofac 2006;28,2 (marzo-abril):114-118 © 2006 ergon Rev Esp Cir Oral y Maxilofac 2006;28,2 (marzo-abril):114-118 © 2006 ergon 115 A. López Ceres y cols. IntroducciónLa enfermedad de Madelung, o lipomatosis simétrica benigna es una enfermedad rara caracterizada por acúmulos grasos no encapsulados localizados de forma simétrica alrededor de cuello y hombros, y que rara vez afecta a caderas y miembros inferiores. Fue descrita por primera vez por Brodie en 1846 y Otto Madelung, en 1988 aportó una serie de 35 pacientes con lipomatosis cervical. A finales del siglo XIX, Launois y Bensaude (1898) documentaron más series y definieron el síndrome como la presencia de múltiples acúmu-los grasos de forma simétrica que afectan cabeza, cuello y mitad superior del cuerpo. 1 Esta enfermedad, afecta predominantemente a hombres en edades comprendidas entre los 30 y 60 años, con una relación hombre:mujer de 15:1. La mayor incidencia se encuentra en el área mediterránea, presentando en Italia una incidencia de 1/25000 hombres, 2 y en casi todos los pacientes existe una gran relación con el abuso del alcohol. Las personas no alcohólicas y las mujeres también pueden verse afectadas, aunque de forma más rara. 3 La enfermedad se define como la presencia de múltiples acú-mulos grasos de forma simétrica, afectando generalmente al cuello, pared abdominal, espalda, axilas, torso y brazos.La etiología es todavía desconocida. Generalmente se acompaña de una disfunción hepática debida al alcohol, 4 y un un efecto lipogénico y antilipolítico. 1 Asimismo, se ha observado una lipogé-nesis anormal inducida por las catecolaminas, 5 y una posible asociación con lesiones hipofisarias e hipotalámicas tales como adenomas endocrinos, hipotiroidismo, acidosis tubular renal e hipertrigliceridemia. 4 El diagnóstico debe establecerse fundamentalmente por los datos clínicos y por estudios de imagen como la TC, la Ultrasonografía o la RM. 6 La lipomatosis de forma simétrica que afecte a la lengua como parte de la enfermedad de Madelung es muy rara, y ha sido previamente descrita en sólo tres casos. [7][8][9] Caso clínico Una paciente de 53 años de edad es derivada a nuestro servicio desde el departamento de Medicina Interna, diagnosticada de Enfermedad de Madelung, refiriendo engrosamiento progresivo de la lengua presentando cierta dificultad para tragar, disartria y...

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Multiple Symmetric Lipomatosis: Korean Experience

Cited by 30 publications

References 18 publications

Differential clinical expression of multiple symmetric lipomatosis in men and women

Differential clinical expression of multiple symmetric lipomatosis in men and women

Rare adipose disorders (RADs) masquerading as obesity

Lipomatosis simétrica benigna de la lengua en la enfermedad de Madelung

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