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25 February 2003

Launois-Bensaude syndrome in a female with type 2 diabetes.

Igor Alexander Harsch, Patrick Michaeli, Eckhart Georg Hahn, Joachim Hans Ficker, Peter Christopher Konturek

Med Sci Monit 2003; 9(2): CS5-8 :: ID: 4751

Abstract

BACKGROUND: The Launois-Bensaude syndrome (LBS) is a rare disease characterized by an accumulation of multiple lipomata with a preponderance at the thorax, abdomen, upper arms and the thighs. Frequently, the condition is associated with past or present alcohol abuse with no clear temporal coincidence between the onset and termination of lipomata growth and onset or termination of alcohol consumption. Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia. CASE REPORT: A 79-yr-old female observed an increase of fat mass especially at the upper arms, the thighs and the thorax in combination with a weight gain of 19 kg within 2 years without any changes in the nutrition habits. The unique features confirmed the diagnosis of LBS. Interestingly, she had diabetes mellitus, hypertension and hyperlipidemia before the manifestation of LBS and without any history of heavy alcohol consumption. Furthermore, the condition predominantly affects males. In her very case, treatment with sultanol, as successfully performed in some cases, could not be recommended due to her cardiac insufficiency. A surgical approach is of limited value due to the frequent relapses of the lipomata. CONCLUSIONS: Establishing the diagnosis of LBS is an important step for patients confronted with an inexplicable physical disfigurement that is not related to excessive nutrition. The disease is often not diagnosed because of its rareness, but its features are unique and easily to be distinguished from 'simple' truncal obesity.

Keywords: Diabetes Mellitus, Type 2 - complications, Lipomatosis, Multiple Symmetrical - complications, Lipomatosis, Multiple Symmetrical - diagnosis

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