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familial benign cervical lipomatosis
MIM.151800
Thursday 16 October 2008
Launois-Bensaude disease, cervicothoracic lipodystrophy, multiple symetric lipodystrophy, Madelung disease
Definition: Launois-Bensaude syndrome is a rare pathology consisting of adipose masses symmetrically distributed mainly in the superior part of the body. Men are especially affected between age of 30 and 60 as well as chronic alcohol abusers. Etiopathogenesis is attributable to mutations or deletions of mitochondrial DNA, and alcohol is a possible cofactor.
The presence of multiple, symmetric, nonencapsulated lipomatous masses in the face, neck, upper arms and upper trunk is typical. Until now many causes have been discussed among which liver dysfunctions are described frequently. In up to 90% of patients, alcoholism is observed.
Images
MADELUNG’S DISEASE: ariability in size & shape of adipocytes; bland nuclei; interspersed brown-fat
Synopsis
variability in size & shape of adipocytes
bland nuclei
interspersed brown-fat
Association
alcohol consomption
corticosteroids
liver transplantation (18268407)
References
Klopstock, T.; Naumann, M.; Schalke, B.; Bischof, F.; Seibel, P.; Kottlors, M.; Eckert, P.; Reiners, K.; Toyka, K. V.; Reichmann, H. : Multiple symmetric lipomatosis: abnormalities in complex IV and multiple deletions in mitochondrial DNA. Neurology 44: 862-866, 1994. PubMed ID : 8190288
