FaCD Online Syndrome Fact Sheet

Last updated: 19 Mar 2008

Name: Familial Multiple Symmetric Lipomatosis

Synonym: Benign Symmetric Lipomatosis, Benign Cervical Lipomatosis, Madelung's disease, Launois-Bensaude Adenolipomatosis

Mode of Inheritance: AD?

Tumor features

lipoma

Tumor features (possible)

laryngeal cancer
oropharyngeal cancer

Non-tumor features

neuropathy

Comment

This disorder is characterized by the development of diffuse lipomas, in particular of the shoulders, neck, head and the upper arms. The lipomas may invade deep tissues. Age of onset is middle age, with more males than females affected (4:1). Some familial cases have been reported. [1]. The disorder may occur in association with polyneuropathy[2]. Interestingly, Di Gennaro et al.[3] reported multiple symmetric lipomatosis in 2 families with mitochondrial encephalopathy and mutations in the mitochondrial DNA. Respiratory chain defects were detected in the lymphocytes of a group of multiple symmetric lipomatosis patients studied by Becker-Wegerich et al.[4]. Use of alcohol is associated with symmetric lipomatosis. A few cases of head-and-neck cancer have been repported in patients with multiple symmetric lipomatosis[7,8].

References

[1] Leffell DJ, Braverman IM. Familial multiple lipomatosis. Report of a case and a review of the literature. J Am Acad Dermatol 1986; 15(2 Pt 1):275-279.
[2] Chalk CH, Mills KR, Jacobs JM, Donaghy M. Familial multiple symmetric lipomatosis with peripheral neuropathy. Neurology 1990; 40(8):1246-1250.
[3] Di Gennaro G, Santorelli FM, Tolli VS, Scoppetta C, Pierelli C, Spadaro M, Casali C. Multiple symmetrical lipomatosis in mitochondrial disorders. Am J Hum Genet 1997; 61(4):A307.
[4] BeckerWegerich P, Steuber M, Olbrisch R, Ruzicka T, Auburger G, Hofhaus G. Defects of mitochondrial respiratory chain in multiple symmetric lipomatosis. Arch Dermatol Res 290[12], 652-655. 1998.
[5] Meningaud JP, Pitak-Arnnop P, Bertrand JC. Multiple symmetric lipomatosis: case report and review of the literature. Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons 2007; 65(7):1365-9.
[6] González-García R, Rodríguez-Campo FJ, Sastre-Pérez J, Muñoz-Guerra MF. Benign symmetric lipomatosis (Madelung's disease): case reports and current management. Aesthetic plastic surgery 2004 Mar-Apr; 28(2):108-12; discussion 113.
[7] Guastella C, Borsi C, Gibelli S, Della Berta LG. Madelung's lipomatosis associated with head and neck malignant neoplasia: a study of 2 cases. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery 2002; 126(2):191-2.
[8] Chan ES, Ahuja AT, King AD, Lau WY. Head and neck cancers associated with Madelung's disease. Annals of surgical oncology 1999; 6(4):395-7.