Launois- Bensaude Syndrome (Madelung' s Disease)
A case of Launois-Bensaude Syndrome (Madelung's disease) in a chronic alsoholic is presented and the typical features are discussed. This entity should be considered in patients who present with doughy, symmetrically distributed, adipose masses.
Launois- Bensaude Syndrome or Madelung' s Disease, is rare disorder of unknown etiology. It was first described in 1898 by Launois and Bensaude. Since this report, about 200 cases have been published in the literature. The disease is characterized by prominent, symmetrical masses on the neck, shoulders, arms and uppers parts of the trunk, composed of symmetrically arranged adipose tissue, which gives a pseudoathletic appearance. The characteristic masses are doughy on palpation which distinguishes them from truly hypertrophied muscles. When well developed, these masses render neck movement and breathing more difficult. The disease begins between 35 and 50 years of age and develops over a period of months to years. Males are more often affected than females. The disease can be associated with hyperlipoproteinemia, hyperuricemia, diabetes mellitus and chronic alcoholism. The only effective treatment is surgical removal or liposuction, but recurrences are common.[2,4]
A middle aged, white, male patient presented prominent, soft masses on his neck, shoulders, arms and upper trunk. He exhibited a pseudoathletic appearance, characteristic of the Launois- Bensaude Syndrome. On palpation, the masses were doughty and completely non-tender. Laboratory analyses were obtained (CBC with differential, sholesterol, triglycerides, uric acid, glucose) and were all within normal limits. Chronic alcoholism, a common finding in reported cases of Madelung's disease, was also documented in this patient. Except for cosmetic concerns, he had no other complaints relating to the excess adipose tissue. He was not interested in liposuction or surgical removal of the tumorous masses.
We presented a patient with the completely developed picture of Launois- Bensaude Syndrome. Although rare (about 200 cases have been published), this syndrome may be seen in clinical practice and dermatologists should be aware of its existence.
References1. Launois PE, Bensaude R. De l adenolipomatose symetrique. Soc Med Hop Paris Bull Mem 1898: 298.
2. Basse P, Lohman M, Hovgaard C, Alsbjorn B. Multiple symmetric lipomatosis. Acta Chir Plast 1991, 33: 204- 9.
3. Topalo VM. Benign symmetrical lipomatosis with predominant localization in the neck area [Madelung' s Disease]. Khirurgiia [Mosk] 1992; 3: 26- 32.
4. Darsonal V, Duly T, Munin O, Hoete JF. Surgical treatment of Launois Bensaude disease. The value of liposuction. Ann Chir Plast Esthet 1990; 35: 128- 33.
© 2001 Dermatology Online Journal