Launois-Bensaude Syndrome: An Unusual Localization of Obesity Disease

Verna, Giovanni; Kefalas, Nicola; Boriani, Filippo; Carlucci, Salvatore; Choc, Ingrid; Bocchiotti, Maria Alessandra

doi:10.1007/s11695-008-9502-2

Cited by 23 publications

(30 citation statements)

References 34 publications

(38 reference statements)

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“…Localization and size of fatty deposits and existing complications will determine the choice of surgical technique. Results are often dissatisfying because of the number of lipomas, the diffi culty of determining the extent of resection and a high recurrence rate [4,10]. Because our patient did not present with any symptoms of functional impairment at the time of presentation, we recommended strict alcohol abstinence.…”

Section: Discussionmentioning

confidence: 96%

“…Type I is characterized by fat masses around the neck, the upper back, the shoulders and upper arms, giving a pseudo-athletic appearance. In Madelung's type II, the fatty deposits re-diff usely distributed over the entire body surface, resembling simple obesity [10]. Our patient presented with the characteristic physical features of Madelung's disease type I. Madelung's disease is associated with metabolic and endocrine disturbances including impaired glucose tolerance, excessive secretion of insulin, insulin resistance, hyperuricaemia, renal tubular acidosis, alteration in liver enzyme levels, hypercholesterolemia, and abnormal function of thyroid, adrenal glands, hypophysis and testicles [11].…”

Section: Discussionmentioning

confidence: 99%

“…Mutations or deletions of mitochondrial DNA are also discussed as possible causes [14]. Th ere is a strong association of Madelung's disease and alcohol consumption; in up to 90% of cases mild-to-excessive alcohol abuse was involved but patients without any history of alcoholism have been described [7,10]. Alcohol could serve as a cofactor in any of the above-mentioned hypotheses, because it reduces not only the number but also the activity of ß-adrenergic receptors, promoting lipogenesis [14].…”

Section: Discussionmentioning

confidence: 99%

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Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Friedl

Sampl

Prandl

et al. 2011

Wien Klin Wochenschr

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Madelung's disease, or multiple symmetric lipomatosis, is an extremely rare disease and very likely to be under-diagnosed. It is characterized by multiple symmetrical non-encapsulated fat accumulations mainly located around the neck, shoulders, upper extremities and upper parts of the trunk. The disorder predominantly affects middle-aged men of Mediterranean origin with a history of ethanol intake. Reports of this uncommon disorder in women are very rare. The aim of this article is to report the endocrine and metabolic workup leading to the diagnosis of this uncommon disorder. A 55-year-old woman with osteoporosis was referred to our outpatient clinic with a suspected diagnosis of Cushing's disease. The patient complained of undesired weight gain with atypical fat accumulations predominantly in the upper trunk region during the previous 10 weeks. She presented with the characteristic physical features of Madelung's disease and underwent a thorough examination with endocrine and metabolic evaluation of this rare condition and was finally diagnosed with Madelung's disease. This report demonstrates how a diagnosis of this rare disorder can be reached efficiently. A history of osteoporosis in combination with weight gain and atypical fat accumulations ultimately led to the diagnosis of Madelung's diseases, a rather unknown disorder likely to be under-diagnosed. Although treatment options are limited, a diagnosis is still important for the affected individual.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Friedl

Sampl

Prandl

et al. 2011

Wien Klin Wochenschr

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“…44 It is characterized by accumulation of multiple, symmetric, nonencapsulated lipomatous masses mainly in the superior part of the body. 5,45 Due to the peculiar localization of fat bulges, it causes functional more than aesthetic concerns to the patients. Its etiology remains unknown and could be attributable to mutations or deletions of mitochondrial DNA with reduced activity of the cytochrome c oxidase with a decreased activity of mitochondrial respiratory chain; alcohol is a possible cofactor.…”

Section: Multiple Symmetric Lipomatosis (Madelung Disease or Launois-mentioning

confidence: 99%

“…Its etiology remains unknown and could be attributable to mutations or deletions of mitochondrial DNA with reduced activity of the cytochrome c oxidase with a decreased activity of mitochondrial respiratory chain; alcohol is a possible cofactor. 5,45 It seems that the adipose masses are due to hypertrophy of embryonic residues of brown fat caused by an abnormal catecholamine-related synthesis of cAMP. 45 Various metabolic disturbances can be associated with this condition such as insulin resistance, impaired glucose tolerance, elevated uric acid, renal tubular acidosis, alteration in the levels of liver enzymes, abnormal function of thyroid, adrenal glands, hypophysis, and testicles.…”

Section: Multiple Symmetric Lipomatosis (Madelung Disease or Launois-mentioning

confidence: 99%

Functional and Therapeutic Indications of Liposuction

Atiyeh¹,

Costagliola²,

Illouz³

et al. 2015

Annals of Plastic Surgery

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Liposuction is the most common cosmetic surgical procedure worldwide. It has evolved from being designed primarily for body contouring to becoming essential adjunct to various other aesthetic procedures, greatly enhancing their outcome. Despite its hard clear differentiation between an aesthetic and therapeutic indication for some pathologic conditions, liposuction has been increasingly applied to a gamut of disorders as a therapeutic tool or to improve function. In fact, liposuction has ceased to define a specific procedure and became synonymous to a surgical technique or tool same as the surgical knife, laser, electrocautery, suture material, or even wound-dressing products. At present, there seems to be an enormous potential for the application of the basic liposuction technique in ablative and reconstructive surgery outside the realm of purely aesthetic procedures. The present review contemplates the various nonaesthetic applications of liposuction, displaying the enormous potentials of what should be considered a basic surgical technique rather than a specific aesthetic procedure. Implications of this new definition of liposuction should induce third-party public payers and insurance companies to reconsider their remuneration and reimbursement policies.

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Disorders of Fat Tissue

Lacour¹

2011

Harper's Textbook of Pediatric Dermatology

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Launois-Bensaude Syndrome: An Unusual Localization of Obesity Disease

Abstract: Launois-Bensaude syndrome causes a functional rather than esthetic concern due to the peculiar localization of fat bulges. Currently, the only effective therapy is surgery, through lipectomy or liposuction of adipose bulges.

Cited by 23 publications

References 34 publications

Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Functional and Therapeutic Indications of Liposuction

Disorders of Fat Tissue

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